Re: Salt and heart size in PA

November 14, 2011

I had a MRI of the heart. It showed LVH, cardiomyapathy and that I

should rule out for sarcodiosis.

I wonder if the MRI was seeing fibrosis. He wanted to do a biopsy of

my heart. I refused- biopsies of the heart dont have good outcomes.

I had a CT of the lung and it didnt show adenapathy or nodules in

the lung.

Phyllis

4.

J Clin

Endocrinol Metab. 2011 Sep;96(9):2813-20. Epub

2011 Jun 1.

Cardiac dimensions are largely determined

by dietary salt in patients with primary

aldosteronism: results of a case-control

study.

Pimenta E, Gordon RD, Ahmed AH, Cowley D, Leano R, Marwick TH, Stowasser M.

Source

Endocrine Hypertension Research Centre,

University of Queensland School of Medicine,

Princess andra Hospital, Brisbane, Queensland

4102, Australia. e.pimenta@...

Abstract

CONTEXT:

Animal studies have demonstrated that dietary

sodium intake is a major influence in the

pathogenesis of aldosterone-induced effects in the

heart such as left ventricular (LV) hypertrophy

and fibrosis. LV hypertrophy is an important

predictor for cardiovascular morbidity and

mortality.

OBJECTIVE:

We aimed to investigate the relationships

between aldosterone and dietary salt and LV

dimensions in patients withprimary

aldosteronism (PA).

DESIGN

AND PARTICIPANTS:

This case-control study included 21 patients

with confirmed PA and 21 control patients with

essential hypertension matched for age, gender,

duration of hypertension, and 24-h systolic and

diastolic blood pressure.

MAIN

OUTCOME MEASURES:

Patients were evaluated by echocardiography and

24-h urinary sodium (UNa) excretion while

consuming their usual diets.

RESULTS:

Patients with PA had significantly greater mean

LV end-diastolic diameter, interventricular septum

and posterior wall thicknesses, LV mass (LVM) and

LV mass index, and end systolic and diastolic

volumes than control patients. UNa significantly

positively correlated with interventricular

septum, posterior wall thicknesses, and LVM in the

patients with PA but not in control patients. In a

multivariate analysis, UNa was an independent

predictor for LV wall thickness and LV mass among

the patients with PA but not in patients with

essential hypertension.

CONCLUSIONS:

These findings emphasize the importance of

dietary sodium in determining the degree of

cardiac damage in those patients with PA, and we

suggest that aldosterone excess may play a

permissive role. In patients with PA, because a

high-salt diet is associated with greater

LVM, dietary salt restriction might reduce

cardiovascular risk.

November 14, 2011

Most of is with untreated PA have LVH. I know mine improved greatly after PA was found and treatedSent from my Palm Pre on the Now Network from Sprint

I had a MRI of the heart. It showed LVH, cardiomyapathy and that I

should rule out for sarcodiosis.

I wonder if the MRI was seeing fibrosis. He wanted to do a biopsy of

my heart. I refused- biopsies of the heart dont have good outcomes.

I had a CT of the lung and it didnt show adenapathy or nodules in

the lung.

Phyllis

4.

J Clin

Endocrinol Metab. 2011 Sep;96(9):2813-20. Epub

2011 Jun 1.

Cardiac dimensions are largely determined

by dietary salt in patients with primary

aldosteronism: results of a case-control

study.

Pimenta E, Gordon RD, Ahmed AH, Cowley D, Leano R, Marwick TH, Stowasser M.

Source

Endocrine Hypertension Research Centre,

University of Queensland School of Medicine,

Princess andra Hospital, Brisbane, Queensland

4102, Australia. e.pimenta@...

Abstract

CONTEXT:

Animal studies have demonstrated that dietary

sodium intake is a major influence in the

pathogenesis of aldosterone-induced effects in the

heart such as left ventricular (LV) hypertrophy

and fibrosis. LV hypertrophy is an important

predictor for cardiovascular morbidity and

mortality.

OBJECTIVE:

We aimed to investigate the relationships

between aldosterone and dietary salt and LV

dimensions in patients withprimary

aldosteronism (PA).

DESIGN

AND PARTICIPANTS:

This case-control study included 21 patients

with confirmed PA and 21 control patients with

essential hypertension matched for age, gender,

duration of hypertension, and 24-h systolic and

diastolic blood pressure.

MAIN

OUTCOME MEASURES:

Patients were evaluated by echocardiography and

24-h urinary sodium (UNa) excretion while

consuming their usual diets.

RESULTS:

Patients with PA had significantly greater mean

LV end-diastolic diameter, interventricular septum

and posterior wall thicknesses, LV mass (LVM) and

LV mass index, and end systolic and diastolic

volumes than control patients. UNa significantly

positively correlated with interventricular

septum, posterior wall thicknesses, and LVM in the

patients with PA but not in control patients. In a

multivariate analysis, UNa was an independent

predictor for LV wall thickness and LV mass among

the patients with PA but not in patients with

essential hypertension.

CONCLUSIONS:

These findings emphasize the importance of

dietary sodium in determining the degree of

cardiac damage in those patients with PA, and we

suggest that aldosterone excess may play a

permissive role. In patients with PA, because a

high-salt diet is associated with greater

LVM, dietary salt restriction might reduce

cardiovascular risk.

November 14, 2011

I need you complete story and a thumbnail to better answer this.Most likely LVH is from HTN and high salt intake. Why would they think of sarcoid?What is your Hx of low K?CE Grim MD I had a MRI of the heart. It showed LVH, cardiomyapathy and that I should rule out for sarcodiosis. I wonder if the MRI was seeing fibrosis. He wanted to do a biopsy of my heart. I refused- biopsies of the heart dont have good outcomes. I had a CT of the lung and it didnt show adenapathy or nodules in the lung. Phyllis 4. J Clin Endocrinol Metab. 2011 Sep;96(9):2813-20. Epub 2011 Jun 1. Cardiac dimensions are largely determined by dietary salt in patients with primary aldosteronism: results of a case-control study. Pimenta E, Gordon RD, Ahmed AH, Cowley D, Leano R, Marwick TH, Stowasser M. Source Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Princess andra Hospital, Brisbane, Queensland 4102, Australia. e.pimenta@... Abstract CONTEXT: Animal studies have demonstrated that dietary sodium intake is a major influence in the pathogenesis of aldosterone-induced effects in the heart such as left ventricular (LV) hypertrophy and fibrosis. LV hypertrophy is an important predictor for cardiovascular morbidity and mortality. OBJECTIVE: We aimed to investigate the relationships between aldosterone and dietary salt and LV dimensions in patients withprimary aldosteronism (PA). DESIGN AND PARTICIPANTS: This case-control study included 21 patients with confirmed PA and 21 control patients with essential hypertension matched for age, gender, duration of hypertension, and 24-h systolic and diastolic blood pressure. MAIN OUTCOME MEASURES: Patients were evaluated by echocardiography and 24-h urinary sodium (UNa) excretion while consuming their usual diets. RESULTS: Patients with PA had significantly greater mean LV end-diastolic diameter, interventricular septum and posterior wall thicknesses, LV mass (LVM) and LV mass index, and end systolic and diastolic volumes than control patients. UNa significantly positively correlated with interventricular septum, posterior wall thicknesses, and LVM in the patients with PA but not in control patients. In a multivariate analysis, UNa was an independent predictor for LV wall thickness and LV mass among the patients with PA but not in patients with essential hypertension. CONCLUSIONS: These findings emphasize the importance of dietary sodium in determining the degree of cardiac damage in those patients with PA, and we suggest that aldosterone excess may play a permissive role. In patients with PA, because a high-salt diet is associated with greater LVM, dietary salt restriction might reduce cardiovascular risk.

November 14, 2011

I had a MRI of my heart because on the echo my heart was very big

but the EKG was normal. My doc didnt understand this.

The MRI report said LVH, cardiomyapthy, r/o sarcoid granulomas. I

also had calcium and vitamin d dysregulation. I couldnt eat anything

with calcium and vitamin d

even though my vitamin d levels are low. I also had bad joint pain

and I couldnt tolerate the heat or sun. The doc said these were

signs of sarcoid.

I first had a CT scan of the lung and it was very clear. As I

understand it, sarcoid first appears in the lung. My CT scan was

perfect. Then he wanted to do a biopsy of my heart.

I refused. Plus the only treatment for sarcoid is steroids and I am

definitely not going there.

ON to search for another diagnosis. I went to another doc who put

me on Inspra and Benicar and most of the 'sarcoid' symptoms went

away.

I can now eat foods with vitamin d and calcium. I had sun and heat

intolerance. Although it is winter time now, I can go out in the sun

without it hurting my eyes most days.

The muscle pain I would get with eating vitamin d and calcium rich

foods has gone away. I still have random joint pain and muscle pain

, fatigue and palpitations but not like before.

The shortness of breath is also better. I can go up a flight of

stairs without collapsing.

I am still on a search ,I think I have found it with PA after 15

years of suffering. Thanks God I have not had a massive stroke and

on dialysis. It is only God that has saved me. i went for 15 years

with bp 170/100- 235/120 despite 5-7 bp meds. I also knew something

else was wrong. I had nailed it down to endocrine system. I always

felt like i was operating on the flight-or fight system. I used to

refer myself to endocrinologist but they wouldnt see me without

referral. I would then make an appt with an endocrinogist and lie

and say my doc referred me. They would call my doc and cancel my

appt. I would get 'surges' of something in my body that would

immediately increase my heart rate and bp. Now I know it was probaly

a surge of aldosterone. In fact I still get these 'surges' now

especially when I get scared or excited.

Phyllis

44yo AA Female, LVH, Cardiomyapathy, TIA, Insulin Resistance, Obese,

GERD, 6 bp meds previously with bp 190/110, 15 years of htn

170/100-235/120 despite medicine

Now: Inspra 50mg daily, Benicar 10mg daily for 3 months, BP

averaging 150/85. Trying to dash ,

I need you complete story and a thumbnail to better

answer this.

Most likely LVH is from HTN and high salt intake. Why

would they think of sarcoid?

What is your Hx of low K?

CE Grim MD

On Nov 14, 2011, at 9:00 AM, Phyllis

wrote:

I had a MRI of the heart. It showed LVH,

cardiomyapathy and that I should rule out for

sarcodiosis.

I wonder if the MRI was seeing fibrosis. He

wanted to do a biopsy of my heart. I refused-

biopsies of the heart dont have good outcomes.

I had a CT of the lung and it didnt show

adenapathy or nodules in the lung.

Phyllis

4.

J

Clin Endocrinol Metab. 2011

Sep;96(9):2813-20. Epub 2011 Jun 1.

Cardiac

dimensions are largely determined by

dietary salt in patients with primary

aldosteronism: results of a

case-control study.

Pimenta

E, Gordon

RD, Ahmed

AH, Cowley

D, Leano

R, Marwick

TH, Stowasser

M.

Source

Endocrine Hypertension Research

Centre, University of Queensland

School of Medicine, Princess

andra Hospital, Brisbane,

Queensland 4102, Australia. e.pimenta@...

Abstract

CONTEXT:

Animal studies have demonstrated

that dietary sodium intake is a

major influence in the pathogenesis

of aldosterone-induced effects in

the heart such as left ventricular

(LV) hypertrophy and fibrosis. LV

hypertrophy is an important

predictor for cardiovascular

morbidity and mortality.

OBJECTIVE:

We aimed to investigate the

relationships between aldosterone

and dietary salt and LV dimensions

in patients withprimary

aldosteronism (PA).

DESIGN

AND PARTICIPANTS:

This case-control study included

21 patients with confirmed PA and 21

control patients with essential

hypertension matched for age,

gender, duration of hypertension,

and 24-h systolic and diastolic

blood pressure.

MAIN

OUTCOME MEASURES:

Patients were evaluated by

echocardiography and 24-h urinary

sodium (UNa) excretion while

consuming their usual diets.

RESULTS:

Patients with PA had

significantly greater mean LV

end-diastolic diameter,

interventricular septum and

posterior wall thicknesses, LV mass

(LVM) and LV mass index, and end

systolic and diastolic volumes than

control patients. UNa significantly

positively correlated with

interventricular septum, posterior

wall thicknesses, and LVM in the

patients with PA but not in control

patients. In a multivariate

analysis, UNa was an independent

predictor for LV wall thickness and

LV mass among the patients with PA

but not in patients with essential

hypertension.

CONCLUSIONS:

These findings emphasize the

importance of dietary sodium in

determining the degree of cardiac

damage in those patients with PA,

and we suggest that aldosterone

excess may play a permissive role.

In patients with PA, because a

high-salt diet is associated

with greater LVM, dietary salt

restriction might reduce

cardiovascular risk.

November 14, 2011

Phyllis, I'm curious to know what your symptoms were with calcium and vitamin d

dysregulation.

Before my PA diagnosis, my ex-doctor ( " the one that I fired " for those of you

who know my whole story) diagnosed me with severely low Vit D and put me on 2000

IU daily. Within a few days I had chest pain and palpitations - I absolutely

could not tolerate the stuff!

-msmith1928

Nulliparous female, 46, 5'3 " , 115 lbs, CT showed 1cm left adrenal nodule, AVS

determined disease is unilateral, had left laparoscopic adrenalectomy on

10/13/2011. Low sodium, fructose- and grain-free diet due to hereditary fructose

intolerance, lactose intolerance, gluten intolerance (probable celiac).

I also

> had calcium and vitamin d dysregulation. I couldnt eat anything with

> calcium and vitamin d

> even though my vitamin d levels are low.

November 14, 2011

Here is some information about Sarcoidosis

U .S . D E PA RT M E N T O F H E A LT H A N D H U M A N S E RV I C E S

N AT I O N A L I N S T I T U T E S O F H E A LT H

N AT I O N A L H E A RT, L U N G , A N D B L O O D I N S T I T U T E

U .S . D E PA RT M E N T O F H E A LT H A N D H U M A N S E RV I C E S

N A TI O N A L I N S T I T U T E S O F H E A LT H

N A TI O N A L H E A RT, LU N G, A N D B L O O D I N S T I T U T E

I NTRODUCTION

Sarcoidosis is a disease that causes inflamma-tion

of the body's tissues. Inflammation is a

basic response of the body to injury and usu-ally

causes reddened skin, warmth, swelling,

and pain. Inflammation from sarcoidosis is

different. In sarcoidosis, the inflammation

produces small lumps (also called nodules or

granulomas) in the tissues.

The inflammation of sarcoidosis can occur in

almost any organ and always affects more

than one. Most often, the inflammation starts

in either the lungs or the lymph nodes (small

bean-shaped organs of the immune system).

Once in a while, the inflammation occurs

suddenly and symptoms appear quickly, but

usually it develops gradually and only later

produces symptoms.

Sarcoidosis usually is a mild condition and

does not result in lasting harm to tissues. In

most patients, the inflammation that causes

the granulomas gets better with or without

treatment and the lumps go away. In others,

however, the lumps do not heal or disappear,

and the tissues remain inflamed. If untreated,

these tissues can become scarred. The tissue

is then called " fibrotic. " But even those who

need treatment can usually lead a normal life.

The cause of sarcoidosis is not yet known—

there may be several. For instance, an abnor-mal

response from the immune system may

be involved. (The immune system normally

Sarcoidosis

attacks and eliminates foreign substances,

such as bacteria, that enter the body.)

Once thought rare, sarcoidosis is now known

to be common and affects persons worldwide.

In fact, sarcoidosis is the most common

chronic fibrotic interstitial lung disorder.

(Chronic illnesses are those that last for some

time or recur often; interstitial lung diseases

affect the tissue that surrounds the air sacs,

blood vessels, and air passageways.)

This fact sheet gives an overview of sarcoido-sis.

It tells who gets sarcoidosis, the disease's

symptoms, diagnosis, and treatment, and

reviews some of the studies underway to

learn more about the illness. In addition,

it provides references to articles and support

groups to contact for more information.

The fact sheet also has special sections on

diagnostic tests, the disease's effects on vari-ous

organs, and commonly asked questions.

The fact sheet closes with a glossary.

What Is the History of Sarcoidosis?

Sarcoidosis was first identified more than a

century ago. In 1869, Hutchinson,

an English doctor, saw a 58-year-old man

who had " multiple, raised, dusty-red patches

on his feet, fingers, and arm. " Hutchinson

later reported on more patients with patches

or lumps on the skin, eyes, or other organs.

These patients had only one affected organ

and only later was the disease known to

involve the whole body.

2

The name " sarcoidosis " was coined by

Dr. Caesar Boeck of Norway, who

thought the skin lesions looked like

benign (not life-threatening) sarcomas

(tumors). The words " sark " and " oid "

come from Greek and refer to the

disease's flesh-like tumors.

Today, it is known that sarcoidosis can

affect almost any part of the body—

lungs, eyes, skin, bones, lymph nodes,

spleen, liver, heart, and so on. It also

is now known that sarcoidosis can

cause hypercalcemia (in which there is

too much calcium in the blood) and

hypercalciuria (in which there is too

much calcium in the urine), both of

which can lead to kidney stones.

Knowledge has brought better tests

to diagnose the disease and improved

treatment. For example, in the 1970s,

the use of a flexible bronchoscopic

biopsy was initiated to help diagnose

sarcoidosis in the lungs. In this

procedure, doctors take a sample

of lung tissue with a bronchoscope,

a long, thin, flexible tube, about the

thickness of a pencil (see Box 1).

The bronchoscope also lets a doctor

look inside the lungs and, besides

improving diagnosis, has added to

scientists' understanding of how the

immune system may be involved in

the development of sarcoidosis.

Treatment too has steadily advanced:

Cortisone (a steroid drug) was first

used to treat the disease in 1951. In

1958, an x ray " staging " method

that describes the lung x ray pattern

was devised to aid diagnosis and

treatment. In 1975, researchers found

that the levels in the blood of a sub-stance

called angiotensin converting

enzyme (ACE) could be used as a bio-chemical

marker to help identify and

treat those with sarcoidosis. ACE is

made by cells in the granulomas.

However, ACE levels are not always

elevated in those with sarcoidosis and

a high level alone does not mean

someone has the disease. A high level

also does not mean that treatment

must be given. Still, the discovery

has handed doctors another tool

to help them make diagnosis and

treatment decisions.

Who Gets Sarcoidosis?

Sarcoidosis occurs worldwide. It

affects men and women of all ages

and races. However, it occurs most

commonly in adults between the

ages of 20 and 40, and in those of

African (especially women), Asian,

German, Irish, Puerto Rican, or

Scandinavian origin. In the United

States, the disease occurs slightly

more often and more severely among

African Americans than whites.

Studies also have shown that the

disease is more likely to affect certain

organs in certain populations. For

example, sarcoidosis of the heart

and eye appears to be more common

in Japan. Painful skin lumps on

the legs (erythema nodosum) occur

more often in people from

Northern Europe.

Sarcoidosis may occur in families.

In the United States, this happens

more often among African Americans

than whites.

Environmental factors also may affect

the occurrence of sarcoidosis. For

example, sarcoidosis occurs more

often in nonsmokers than smokers.

Several studies have noted higher

rates of sarcoidosis among health care

workers. Other environmental factors,

such as beryllium metal (used in air-craft

and weapons manufacture) and

organic dust from birds or hay, may

cause sarcoidosis-like reactions in the

lungs. Thus, doctors need to know

a person's history of occupational

and environmental exposure in trying

to diagnose sarcoidosis. Infectious

agents have been suspected of causing

sarcoidosis, but there is no proof

of an infectious cause. More research

is needed to better understand the

effect of environmental factors on a

person's risk of developing sarcoidosis.

What Are the Pathology and

Course of the Disease?

A normal organ is made of an orderly

arrangement of cells. Sarcoidosis

upsets this arrangement, eventually

causing lumps to form in organs.

These lumps get larger and are called

" granulomas " because they look like

grains of sugar or sand. These " grains "

are very small and can only be seen

with a microscope.

Various other diseases can cause the

formation of granulomas. For exam-ple,

tuberculosis can cause granulo-mas.

However, in other diseases, the

granuloma forms around a particle,

germ, or other foreign substance. In

the case of tuberculosis, for instance,

the granuloma forms around the

invading organism, which is a

mycobacterium. The immune system

causes granulomas to form so that the

Biopsy sample taken from a sarcoidosis

skin lesion, multiplied 200 times.

The granuloma is round and contains

large cells.

3

particles, germs, or other foreign sub-stances

can be isolated or eliminated.

In sarcoidosis, there is no such visible

enclosed particle or germ. No cause

for the granuloma can be seen under

the microscope. The immune system

appears to be responding to an

unknown substance.

When thousands of these microscopic

granulomas clump together, they

result in a variety of small and large

lumps. These lumps can appear on

the lungs, skin, or other organs, such

as the eyes, mouth, salivary glands,

liver, spleen, or lymph nodes in the

neck, armpits, and groin. Lymph

nodes are small organs of the body's

immune system.

The lumps can show up as shadows

on x rays. If many large groups of

granulomas form, they can affect the

organ's function. This can cause

symptoms that need to be treated.

The disease has active and nonactive

stages. In the active stage, the im-mune

system is fighting the disease

and granulomas form or enlarge.

In this stage, symptoms can develop

and scar tissue can form. In the non-active

stage, the disease is easing, and

the granulomas are stable, shrinking,

or have become scars.

The course of the disease varies: In

most persons, the sarcoidosis goes

away over time. In others, the sar-coidosis

does not get worse, but the

disease remains and a person can feel

well or continue to have symptoms.

When treatment is given, it usually

shrinks the granulomas, and they may

even disappear. Such treatment may

last for many months. In still other

persons, scars can form in the granu-lomas.

The scars often remain, even

with treatment, and symptoms may

never go away, and an affected organ

may continue to function poorly.

What Are the Symptoms?

Most people with sarcoidosis have

no symptoms. Some have only one

symptom, while still others have

many. Symptoms typically depend

on which organs the disease affects.

General symptoms caused by the

disease include weight loss, fatigue,

night sweats, fever, and an overall

feeling of ill health.

Most often, the disease will affect the

lungs. Thus, the most common

symptoms of the disease are a cough

that does not go away and shortness

of breath, particularly with exertion.

Symptoms common in sarcoidosis

include the following—for more on

each organ and for some not listed

below, see Box 2:

General Symptoms

Uneasiness, feeling sick ( " malaise " )

Tiredness, fatigue, weakness

Loss of appetite or weight

Fever

Sweating at night during sleep

Lymph Node Symptoms

Enlarged lymph nodes—most

often those of the neck, but also

may be those under the chin,

in the arm pits, or in the groin

Skin Symptoms

Skin rash—painful or hot red

bumps on the legs or arms,

or small brownish and painless

bumps on the arms, legs,

and/or back

Eye Symptoms

Burning, itching, tearing, pain

Red eye

Sensitivity to light (photophobia)

Dryness

Seeing black spots (called floaters)

Blurred vision

Lungs and Heart Symptoms

Shortness of breath

Wheeze

Cough

Chest pain

Irregular heartbeat (palpitations)

Joint Symptoms

Joint stiffness, swelling—most

commonly of the ankles, feet,

and hands

How Is Sarcoidosis Diagnosed?

The symptoms of sarcoidosis are like

those of other diseases, some more

harmful and even life-threatening.

So it is important to properly diag-nose

the condition.

Someone who is thought to have

the disease should see a doctor who

specializes in sarcoidosis, usually

a lung physician (pulmonologist).

The specialist will work with patients

and their regular physician to help

diagnose the disease and to develop

a schedule of treatment and follow-up

care.

To make a diagnosis, a doctor will

ask for a medical history and do a

physical examination. The doctor

also may need to take laboratory

tests of the blood, a chest x ray,

and breathing tests. Some of the

tests and procedures used to help

diagnose sarcoidosis are described

in Box 1.

BOX 1: KEY TESTS FOR DIAGNOSIS AND TREATMENT

Various tests and procedures are

used to help diagnose sarcoidosis.

Some of these also help monitor the

disease during and after treatment.

Here are some of the key tests:

Physical Examination

The doctor will look for symptoms

of the disease, such as red bumps

on the skin, swollen lymph nodes,

or redness in the eyes. The doctor

also will check for other possible

causes of any symptoms.

Chest X Ray

A chest x ray, which poses little risk

to health, can detect sarcoidosis.

About 90 percent of all persons

with sarcoidosis will have an abnor-mal

chest x ray.

X-ray beams cannot pass as easily

through granulomatous or scarred

tissue as through normal tissue.

The x ray may show granulomas,

which appear as a shadow, or

enlarged lymph glands in the chest.

Frequently, sarcoidosis is diagnosed

because a chest x ray, taken routine-ly

or for some other reason, shows

an abnormality.

Chest x rays also may be taken to

follow the course of the disease.

However, the x rays typically are

not done as often for this purpose

as are the pulmonary function tests.

Blood Tests

Blood analyses evaluate the number

and types of blood cells in the body.

The tests also measure the blood

levels of various proteins, such as

ACE (see page 2), which are known

to be involved in immunological

activities, as well as increases in

calcium levels. Additionally, they

can show liver, kidney, and bone

marrow abnormalities that can

occur with sarcoidosis.

Pulmonary Function Tests

Pulmonary function tests are used

to monitor the course of the disease

in the lungs. These tests are safe

and easy to do. The results are

compared over time.

One pulmonary function test uses

a " spirometer, " a device that

measures how much and how fast

a person can blow air out of the

lungs after taking a deep breath.

This amount will be less than

normal if there is significant inflam-mation

and/or scarring in the lung.

Another test measures lung volume,

which indicates how much air

the lungs can hold. In some

patients, the lungs may shrink or

contract due to sarcoidosis, and

the lung volumes will be smaller

than normal.

Other tests check for diffusing

capacity, or how well a gas moves

into the bloodstream from the

lungs. Sarcoidosis makes it harder

for oxygen to move from the lungs

into the bloodstream. In one test,

a device called a pulse oximeter

is placed on the finger to give the

doctor a rough idea of the level (or

saturation) of oxygen in the patient's

blood. An arterial blood gas test is

a more accurate way to check the

level of oxygen in the bloodstream.

Blood from an artery (usually in the

wrist) is used because it has passed

through the lungs and taken up

oxygen. The blood is then analyzed

for its oxygen and carbon dioxide

4

Only a biopsy gives a reliable diagno-sis

of sarcoidosis. In a biopsy, a sample

of tissue is taken from an affected

organ. The biopsy tissue can be taken

from any affected organ. So the sim-plest

and least uncomfortable tissue

to biopsy is usually chosen. For exam-ple,

if the skin and lungs are affected,

the biopsy will be done on the skin.

In many cases, a simple skin or a con-junctival

(membrane lining the eye-lid's

inner surface) biopsy is done in

a doctor's office under local anesthe-sia,

and no hospital stay is needed.

The tissue is examined for the pres-ence

of granulomas. As noted, these

granulomas will have no germs or

particles within them.

A biopsy may not be needed in every

case. For instance, erythema nodosum

(painful red bumps, usually on the

legs–see Box 2), may be diagnostic of

sarcoidosis when accompanied by an

abnormal chest x ray.

In 1941, a skin test was developed to

help diagnose sarcoidosis, but it is

not readily available in the United

States. Called the Kveim-Siltzbach

test, it involves injecting a standard-ized

preparation of " sarcoidosis " tissue

into the skin. The test is considered

positive if a lump forms at the injec-tion

site and a biopsy of the lump

shows granulomas. The result is not

always positive, even if the person

has sarcoidosis. It is rarely used in

the United States because the U.S.

Food and Drug Administration has

not approved a test preparation for

sale. However, some hospitals and

clinics may have privately prepared

a standardized test preparation.

levels. The better the lungs are

working, the more oxygen there

will be in the arterial blood.

Fiberoptic Bronchoscopy

In this procedure, a long, narrow,

flexible tube with a light at the end

is inserted into an airway of the lung.

This makes it possible for the doctor

to look at the tissue lining the air

passageways of the lungs. It is also

possible to use the bronchoscope to

obtain small samples of lung tissue

and to obtain lung washings (that

contain lung cells) from various parts

of the lungs.

Fiberoptic Bronchoscopy

Biopsy

In this procedure, a sample of lung

tissue is removed. The procedure

is usually done to make the diagnosis

when pulmonary function tests

or chest x rays are abnormal and

characteristic of sarcoidosis. If per-formed,

it is done at the time of

a fiberoptic bronchoscopy. The test

is done while the patient is awake

but slightly sedated. The test is

usually very safe and done on an

outpatient basis.

Bronchoalveolar Lavage

Often, a procedure called a bron-choalveolar

lavage (BAL) is done

as part of a fiberoptic bronchoscopy.

BAL involves injecting saline

(salt water) into a region of the lung.

The fiberoptic bronchoscope then

uses suction to remove the fluid,

which has washed out cells and other

materials from the tiny air sacs

(alveoli) of the lung. The pulmonary

inflammation associated with

sarcoidosis begins in the lung in

these air sacs. The removed sample

is then examined for signs of inflam-mation

that reflect the disease's

active stage.

CT Scan

A computed tomographic (CT)

scan is a complicated kind of x

ray that gives a better picture of

the lungs than the ordinary chest

x ray. A CT scan may be done

to better assess how much of the

lung is affected by sarcoidosis.

CT scans are not done routinely

because they expose a person to

more radiation than an ordinary

chest x ray and are costly. Instead,

they are done when specific factors

call for their need. For example,

a CT scan might be done to

diagnose sarcoidosis in the brain,

spinal cord, nerves—all of which

are dangerous to biopsy. CT scan

of the lungs is important if the

patient is coughing up blood.

MR Scan

Magnetic resonance (also called

nuclear magnetic resonance,

NMR scanning, or magnetic

resonance imaging, MRI) uses

powerful magnets and radio

waves to see inside the body.

A computer generates images of

the heart, brain, and other organs.

The test is not invasive and has

no known hazards. It can show

if features typical of sarcoidosis

are present in organs.

Thallium and Gallium Scans

These scans are used to help diag-nose

sarcoidosis and are often done

to see if it is in the heart. Thallium

and gallium are radioactive elements.

The doctor injects one of these into

a vein and the element collects at

places in the body that have been

affected by sarcoidosis or another

inflammatory condition. At a speci-fied

time after the injection, the

body is scanned for radioactivity.

An increase in the activity at any site

might indicate that inflammatory

activity has developed there. The

test gives an idea of which tissues in

the body have been affected by the

disease and by how much. Since any

inflammation will cause an uptake

of the radioactive element, the test

does not give a definitive diagnosis

of sarcoidosis.

Eye Test

All persons diagnosed with sarcoido-sis

should have an eye test done

by an ophthalmologist (eye doctor).

Even if there are no symptoms of

the disease in the eyes, the results

of the test can be used to help

monitor the disease. If eye symptoms

appear, the test will be repeated

during treatment. It also should

be repeated periodically for those

treated for their sarcoidosis with a

particular drug called chloroquine

or hydroxycholoroquine (Plaquenil)

that can sometimes cause side effects

related to vision. Also, patients

receiving corticosteroids need to

be seen by an ophthalmologist to

check for signs of cataract develop-ment.

For the eye examination, the

doctor looks into the eye for abnor-malities

and does tests to check for

color blindness.

5

6

How Is Sarcoidosis Treated?

The treatment of sarcoidosis depends

on a person's symptoms. Often,

no treatment is needed—up to 60

percent of those with sarcoidosis

receive no therapy. But, for some,

intense treatment is required, especial-ly

if there is critical organ involvement,

such as of the lungs, eyes, heart, or

central nervous system.

Here are some key points about the

use of treatment:

Treatment is done to control symptoms

or to improve the function of organs

affected by the disease.

Treatment may or may not affect the

long-term outcome of the disease.

One study found that 5-10 years

after diagnosis, there was no

difference in recovery between

those who had received a short

course of treatment and those

who had not.

Sarcoidosis granulomas result from

a response of the immune system.

Thus, most medications used to treat

sarcoidosis suppress the immune system.

This can leave a person more likely

to get sick from an infection,

and this risk must be considered

in making treatment decisions.

Treatment for sarcoidosis involves

the use of medications. A wide

variety is available, but most are

strong and can cause bad side

effects. Different ones will work

better for different persons, and

sometimes more than one is used.

Living with the symptoms of the

disease must thus be weighed

against the side effects produced

by the drugs.

Drugs are either taken by mouth

for " systemic " effects throughout

the body or are applied locally to

an affected area. Local therapy is

the safest way to treat the disease,

since only the affected area is

exposed to the drug. Drugs can be

applied locally by drop, inhaler,

or cream. Drugs used in this way

include corticosteroids.

However, to use drugs locally, the

affected area must be easily

reached. For instance, drops and

creams help with some eye or

skin problems, while inhalers are

used to apply steroids to affected

lung tissue, especially to ease

coughing and wheezing. However,

it does not appear that an inhaled

drug can relieve such symptoms

when the affected lung tissue is

deep within the chest.

Here is a list of the main drugs used

to treat sarcoidosis:

Prednisone. Prednisone belongs to

a group of medicines called corticos-teroids

or steroids. It is the most

commonly used drug for sarcoidosis.

Sometimes it is used in combination

with one of the other drugs listed in

this section. Sometimes other steroids

are used.

Prednisone almost always relieves

symptoms due to inflammation.

If a symptom does not get better

after a couple of months of treatment

with prednisone, then there are two

possibilities: either the symptom is

not due to sarcoidosis, or it will

not improve because sarcoidosis has

already caused scarring. In the first

case, the doctor may look for another

cause of the symptom; in the second

case, the symptom will not improve

with further prednisone treatment—

the drug may even cause more symp-toms

due to its side effects.

Prednisone treatment usually lasts for

many months, but can go on for many

years. If prednisone treatment is

stopped after 3 months, the chance

that symptoms will return is 80

percent. If treatment is stopped after

6 months, the chance that symptoms

will return is 50 percent. But, if

treatment is stopped after 1 year,

the chance of a return is only 30

percent and, if treatment is stopped

after 2 years, the chance of a return

is only 25 percent.

However, prednisone can have bad

side effects. These include weight

gain, diabetes, high blood pressure,

mood swings, difficulty sleeping at

night, heartburn, acne, and, when

prednisone is taken for long periods,

thinning of the bones (osteoporosis)

and skin, cataracts, and occasionally

glaucoma. Side effects usually can

be managed by the patient working

with his or her doctor. Also, low

doses of prednisone can frequently

relieve symptoms without causing

significant side effects.

Hydroxychloroquine. The brand name

of this drug is Plaquenil. Hydroxy-chloroquine

is used to treat various

diseases. It has long been used for

malaria and is given for such other

diseases as rheumatoid arthritis and

lupus erythematosis (a disorder that

causes inflammation of the skin

and other parts of the body). With

sarcoidosis, the drug is effective in

about a third of persons. It is more

likely to be effective if sarcoidosis

has affected the skin and if there is

a high level of calcium in the blood.

Hydroxychloroquine has few side

effects, but it can irritate the

stomach and cause eye problems.

7

Anyone taking the drug should have his

or her eyes examined every 6 months.

Methotrexate. This drug too has long

been used to treat other diseases. With

sarcoidosis, the drug works in 60 to 80

percent of persons. However, it takes

up to 6 months to relieve symptoms.

The drug can have various side effects,

including nausea and mouth sores.

Methotrexate also can kill white blood

cells, a type of blood cell used by the

immune system to fight off infection.

Thus, blood tests must be taken

regularly to check the level of these

cells. Rarely (less than 1 percent of the

time) methotrexate causes an allergic

reaction in the lungs. But this reaction

goes away when treatment with the

drug ends. The most serious possible

side effect with methotrexate is liver

damage. If methotrexate must be

taken for more than 2 years, a liver

biopsy may be done first to see if the

organ has been damaged or if the drug

can continue to be used. The drug also

can harm an unborn baby and should

not be taken if a woman is pregnant.

Side effects from methotrexate usually

occur when the drug is taken at higher

doses than those needed to treat sar-coidosis.

The chance of having a bad

side effect also can often be decreased

by taking the vitamin folic acid.

Azathioprine. The brand name of

this drug is Imuran. It also has long

been used to treat various diseases

and in organ transplantation. It works

in about 50 percent of those with

sarcoidosis. Treatment lasts for more

than 6 months. It can lower the num-ber

of white blood cells and may cause

nausea. The biggest concern is that it

may increase the risk of developing

cancer after treatment. However, this

risk has been found only in transplant

patients–and not in those taking the

drug for other diseases. Azathioprine

can harm an unborn baby and should

not be taken by a pregnant woman.

Cyclophosphamide. The brand name

of this drug is Cytoxan. Cyclophos-phamide

is a very strong drug. It

is more likely to lower white blood

cells and cause nausea than either

methotrexate or azathioprine. Thus

the level of white blood cells in the

blood must be closely monitored

during treatment. The drug also

can irritate the bladder. Some of

those on the drug for more than 2

years have developed bladder cancer.

Because of these side effects, the

drug is given only to those with

severe forms of the disease, such as

neurosarcoidosis. The drug can

harm an unborn baby and should

not be taken by a pregnant woman.

Cyclophosphamide can be given intra-venously,

which lessens some of its

side effects but does not reduce the risk

of cancer.

What Tests Are Done To Follow

the Disease?

Those with sarcoidosis need to have

their condition checked during and

after treatment. Those who receive no

treatment also need regular checkups,

since symptoms can develop later.

The patient will work with his or her

sarcoidosis specialist and regular physi-cian

to develop a schedule of periodic

examinations and laboratory tests. The

followup examination usually includes

a review of symptoms, a physical

examination, a chest x ray, breathing

tests, and laboratory blood tests. How

often these examinations and tests are

done depends on the severity of the

symptoms and the organs affected

at diagnosis, the therapy used, and

any complications that may develop

during treatment.

Routine followup care usually lasts for

2-3 years. Whether the specialist or

primary doctor oversees this care

depends on symptoms during the

first year of followup (see the first

bullet below). Patients should tell

their doctor about any new symptom

that lasts for more than a week.

They also should see the doctor if

symptoms appear and do not go

away before the next regularly

scheduled followup visit. Changes in

sarcoidosis occur slowly—usually

over months. Except for disturbed

heart rhythms, sarcoidosis does not

cause sudden illness.

Box 1 gives an overview of follow-up

care. Here are some recommen-dations

for followup care, based

on the condition at diagnosis or the

treatment used:

If at diagnosis, there are no symptoms,

a normal breathing test, and an abnor-mal

chest x ray, then the following is

recommended: A followup examina-tion

should be done every 6-12

months until the illness is stable

or improving. The breathing

test may be repeated, depending

on a patient's symptoms and

exercise capacity.

If new eye symptoms have appeared,

then the eye test should be repeated.

Eye symptoms are often severe.

If no new symptoms have developed,

and the chest x ray is normal,

then the patient can see his or her

regular family physician for future

followup care.

(Continued on page 12)

BOX 2: EFFECTS BY ORGAN

Sarcoidosis is a " multiorgan "

disease—it always involves more

than one organ. An organ is affected

when granulomas cause an abnor-mality

that can be found during

diagnosis or about which a patient

complains. For example, a biopsy

may show that bumps on the skin

are made of granulomas. If no other

cause for the granulomas is found

and there is evidence of sarcoidosis

in another organ, such as the

lungs, the problem is diagnosed as

sarcoidosis. Only one organ biopsy

is necessary.

Some organs are affected more often

than others. Sarcoidosis occurs most

often in the lungs. It also commonly

affects the skin, eyes, lymph nodes,

and liver. Less commonly, it affects

the spleen, brain, nerves, heart, tear

glands, salivary glands, and bones

and joints. Rarely, it affects other

organs, such as the thyroid gland,

breasts, kidneys, and male and fe-male

reproductive organs.

A doctor may not detect sarcoidosis

in every organ affected by the dis-ease.

Often, the effects of sarcoidosis

in an organ are so mild that there

are no symptoms and the organ

continues to function well. In such

cases, identifying the disease in that

organ is not necessary and would

not change the treatment given. For

more on diagnosis, see pages 4 and 5.

Here is a rundown of how sarcoi-dosis

affects different organs,

starting with the organ most

frequently affected and going to

the least affected.

Lungs

Sarcoidosis most commonly affects

the lungs. It may affect the lung

tissue itself and lymph nodes in the

chest. Its effects can range from very

mild (without symptoms) to severe.

Symptoms caused by the disease

occurring in the lungs include short-ness

of breath, coughing, wheezing,

and, rarely, chest pain. If chest pain

occurs, it often is felt in the middle

of the chest and worsens with deep

breathing or coughing.

The disease is usually seen on an x

ray. A staging system is used to

classify chest x rays taken to detect

sarcoidosis. Stage 0 is a normal chest

x ray. Stage 1 is a chest x ray with

enlarged lymph nodes but otherwise

clear lungs. Stage 2 is characterized

by a chest x ray with enlarged lymph

nodes plus infiltrates (shadows) in

the lungs. In Stage 3, the infiltrates

are present but the lymph nodes are

no longer seen. In Stage 4, the chest

x ray shows scars in the lung tissue.

The x-ray stages do not tell the

severity of the disease. However, in

general the higher the stage of the x

ray, the worse the person's symptoms

and lung function (as measured by

breathing tests). Persons with Stage

4 x rays usually have some perma-nent

lung damage. But there is a lot

of individual variation, and persons

at Stages 0 through 3 may or may

not have symptoms.

When no symptoms appear, treat-ment

usually is not given, and

persons recover with time. With

or without treatment, persons

with symptoms often improve,

and their x-ray and breathing tests

become normal.

Eyes

All those diagnosed with sarcoidosis

should see an eye doctor. The doctor

looks in the eyes and does simple

tests. In a third of all those diagnosed

with sarcoidosis, the eyes will be

affected by the disease.

The disease can cause eyes to become

red and painful, especially in bright

light, and blur vision. Other common

symptoms are burning and itching,

discomfort, and, if tear ducts and

glands are affected, dryness. Very

rarely, permanent damage results,

and blindness may occur.

Sarcoidosis of the eyes almost always

responds well to treatment. Most

often, the only treatment needed is

eye drops.

Skin

Sarcoidosis of the skin can result in

rashes or various types of skin

lesions. About 10-35 percent of

persons with sarcoidosis develop

skin lesions. Other illnesses also

can result in skin lesions, so a biopsy

is frequently done to aid diagnosis.

One type of lesion is called erythema

nodosum. It produces painful bumps

that can be warm, tender, and red

or painful, purple-to-red, and

slightly raised bumps. The bumps

appear on the skin, commonly on

the ankles and shins. The lesions

may occur along with fever and

swollen ankles.

These lesions do not contain granu-lomas

(therefore are not biopsied)

and they may occur in other diseases

too. However, the lesions are usually

an early sign of sarcoidosis.

8

9

Typically, erythema nodosum lesions

go away in weeks to months with

or without treatment. Because of

this, treatment does not involve

drugs ordinarily given for sarcoidosis.

Persons receive either no drugs

or, for those who are very uncom-fortable,

aspirin or ibuprofen (an

over-the-counter anti-inflammatory

medication).

Specific skin lesions are another type

of problem, and these show granulo-mas

when a biopsy is done. These

lesions may appear as bumps, ulcers,

or, rarely, flat areas of discolored

skin. They occur most commonly

near the nose, eyes, back, arms,

legs, and scalp and last a long time.

Typically, they are not painful but

sometimes itch.

Another lesion is named lupus

pernio. This type causes disfiguring

lesions on the nose. Treatment is

frequently needed. Also, the lesions

tend to be chronic and often return

after treatment is over.

Lymph Nodes

The body has lymph nodes (or

glands) in various areas, including

the neck, armpits, and groin.

The nodes are part of the body's

immune system. The nodes in

these areas may be affected by

sarcoidosis and appear as swollen

lumps. Treatment may be given

to reduce swelling.

Liver

Granulomas often form in the liver.

However, the disease rarely causes

significant liver damage.

Symptoms of the disease in the liver

include fever, fatigue, itching, and

pain in the upper right part of the

abdomen (area of the body under

the right ribs). The disease can cause

the liver to enlarge.

Blood tests, a CT scan of the

abdomen, or a biopsy may be

done to detect sarcoidosis in the

liver. The CT scan will show if

the liver is enlarged and if there

is a pattern suggesting granulomas

in the organ. The biopsy is done less

often and also shows the formation

of granulomas.

Sarcoidosis of the liver almost

never causes permanent damage

to the organ and, therefore, is

usually not treated unless it is

causing significant symptoms.

Followup care includes regular

blood tests to monitor how well

the liver is working.

If needed, drug treatment reduces

granulomas. Rare cases in which

the disease worsens have been treated

successfully by liver transplantation.

PAROTID/SALIVARY—4.0%

EYES—12.0%

LYMPH NODES—15.0%

SINUSES—3.0%

LUNGS—95.0%

LIVER—11.5%

HEART—2.3%

SPLEEN—6.7%

MUSCLE—0.4%

BONE—0.5%

SKIN—24.0%

(INCLUDING ERYTHEMA NODOSUM) HIGH CALCIUM—3.7%

NEUROLOGIC—4.0%

BONE MARROW—4.0%

P E R C E N T O F C A S E S W I T H S A R C O I D O S I S I N S P E C I F I C O

R G A N S *

*ACCORDING TO DATA FROM " A CASE CONTROL ETIOLOGIC STUDY OF SARCOIDOSIS " (ACCESS)

10

Salivary Glands

These include the two parotid

glands, which are below and in

front of the ears. When sarcoidosis

affects the parotid glands, it causes

them to swell—making the cheeks

look enlarged. Sarcoidosis in the

salivary glands can cause the mouth

and throat to be excessively dry.

Treatment can be given to ease

symptoms.

Blood, Urinary Tract,

and Kidneys

Sarcoidosis can cause too much

calcium in the blood and urine.

This results from an enzyme made

by the granulomas. Excess calcium

in the urine can lead to painful kidney

stones. A blood test for calcium

should be done. If the calcium level

is high, it probably will need treat-ment.

Sarcoidosis patients with

high calcium levels should not take

vitamin and mineral supplements

containing calcium or vitamin D.

Nervous System

The nervous system includes the

brain and all the body's nerves,

and it may be affected by sarcoidosis.

The disease can cause a mass of

granulomas in the brain or meninges,

which are the membranes that cover

the brain. The disease also can affect

one or more nerves anywhere in the

body. Most often, it affects the

nerves of the face.

Symptoms of the disease in the

nervous system vary. If there is a

mass in the brain, symptoms can

include headaches, visual problems,

and weakness or numbness of an

arm or leg. Coma also can occur,

but does so rarely. Sarcoidosis

can also can cause headaches.

When sarcoidosis affects a facial

nerve, it can cause one side of

the face to droop. This may be

the first symptom that someone

has sarcoidosis. The droop often

goes away or improves without

therapy. When sarcoidosis affects

the spinal cord, it can cause

weakness or even paralysis of the

arms or legs. When multiple

nerves in more than one place are

affected, the disease can cause

weakness, pain, or a " stinging

needles " sensation in those areas.

Sarcoidosis of the nervous system

is often hard to diagnose. To be sure

of the diagnosis, a biopsy may have

to be done, but this is hard to do

on the brain, nerves, or spinal cord.

For the brain, if symptoms are typical

of sarcoidosis and, especially, if the

disease has affected other organs,

a CT or MRI scan will be taken to

check for any abnormalities

(see Box 1).

Neurologic sarcoidosis usually needs

treatment. Nerve tissue heals slowly,

so treatment lasts a long time and

may consist of multiple drugs at

high doses.

Heart

Sarcoidosis sometimes affects the

heart. This happens most often in

Japanese persons living in Japan.

The reason is unknown.

Sarcoidosis can cause the heart to

pump weakly. This results in such

symptoms as shortness of breath,

swelling of the legs, wheezing,

Chest x ray of Stage 1 sarcoidosis shows

normal lungs with enlarged lymph

nodes (see arrows) in the middle of

the chest.

Chest x ray of Stage 2 sarcoidosis shows

enlarged lymph nodes (see arrows) and

streaks in the lungs.

Chest x ray of Stage 3 sarcoidosis shows

streaks in lungs, but no enlarged

lymph nodes.

Chest x ray of Stage 4 sarcoidosis shows

small lungs with streaks and spots.

Dark areas at the top of both lungs are

caused by air cysts. The diaphram

(see arrow) has peaks, which are evidence

of scars in the lungs.

11

and coughing. Sarcoidosis also

can affect the heart's electrical

pacing and transmission system,

which tells it when to beat. This

can make the heart beat too fast

or very slowly, or skip beats.

Symptoms of an electrical-system

problem include palpitations

(a fluttering sensation of rapid

heartbeats), skipped beats, and,

rarely, fluid buildup in the lungs

or sudden loss of consciousness.

Sarcoidosis of the heart is often

hard to diagnose. A biopsy can

be taken and a diagnosis made

if granulomas are seen. However,

granulomas are often not seen

because the tissue sample is small.

Thallium or gallium scans also are

used to detect inflammation in the

heart (see Box 1). If a scan shows a

particular abnormal pattern, then

the diagnosis of sarcoidosis is made.

The diagnosis is more likely if a

biopsy has already proven that

the disease exists in another organ.

Sarcoidosis in the heart is usually

treated with steroids (see page 6).

Additionally, heart drugs are given

to improve the heart's pumping

ability or to correct a disturbed

heart rhythm. If a rhythm distur-bance

is severe, it may be restored

to normal by use of a cardiac pace-maker

(a small battery-operated

device, often put under the skin,

that regulates the heartbeat) or

defibrillator (an implanted device

that shocks a heart into a normal

heartbeat or, if it has stopped,

into beating). If the heart is severely

affected and does not respond to

therapy, a transplant may be done.

But this is rarely needed.

Musculoskeletal

Sarcoidosis may affect the muscu-loskeletal

system. This includes

the muscles ( " musculo " ), joints,

and bones ( " skeletal " ).

In muscles—Sarcoidosis of the

muscles may cause severe muscle

pain, a mass in the muscle, or

muscle weakness.

In joints—Persons with the skin

lesions of erythema nodosum also

may develop arthritis in the ankles.

This form of arthritis usually clears

up in several weeks. Sarcoidosis

also can cause a granulomatous

form of arthritis. Although less

common, this condition is chronic

and can last for months or even

years. Granulomatous arthritis

requires treatment.

In bones—Sarcoidosis can cause

painless holes in bones and painless

swelling, most commonly in the

fingers. Sarcoidosis also can affect

the bone marrow (soft, organic

material that fills bone cavities),

which produces blood cells. This

can result in anemia, in which

there are too few red blood cells,

or a lowered number of white

blood cells. Red blood cells are

needed to deliver oxygen to the

body; white blood cells help fight

infections. Treatment is usually

given to counter these effects.

Other

Sarcoidosis can affect almost any

organ, but rarely strikes the

thyroid gland, the breasts, female

and male reproductive systems,

or the intestines. Other areas

affected more commonly by the

disease include the following:

Sinuses—These are cavities in the

skull, and they can be affected by

sarcoidosis and result in frequent

bouts of sinusitis (inflammation of

the sinus cavities). Treatment is

given to reduce inflammation.

Spleen—When sarcoidosis affects

the spleen, it can reduce the num-bers

of red or white blood cells, or

platelets (important in helping

blood to clot). The spleen also may

enlarge. The person may feel pain

in the upper left part of the

abdomen. Treatment is usually

given to increase cell counts and

ease pain. Rarely, the spleen may

need to be removed.

12

(Continued from page 7)

If at diagnosis, there are symp-toms

and an abnormal chest x

ray, but no treatment is needed,

then the following is recommend-ed:

A followup examination

should be done in 3-6 months.

If the sarcoidosis has wors-ened

by then–causing symp-toms,

or another abnormal

x-ray and abnormal laborato-ry

tests–treatment may be

needed. If treatment is start-ed,

further followup tests may

need to be done more often.

If after diagnosis, treatment is

begun with prednisone, then

the following is recommended:

Followup tests will be done

to monitor for the side effects

of elevated blood pressure,

too much weight gain,

diabetes, and arthritis of

one or both hips.

If after diagnosis, treatment is

begun with hydroxychloroquine,

then the following is recommend-ed:

The patient needs to have

an eye examination every

6 months while the drug is

being taken.

If after diagnosis, treatment is

begun with methotrexate, then

the following is recommended:

Monthly blood tests will

be done to avoid anemia

(in which the blood lacks

enough red blood cells),

low white blood cell and

platelet levels, and inflam-mation

of the liver.

What Is the Prognosis?

Sarcoidosis affects the body in

many ways and the outcome

can vary from person to person.

But the chance of recovering from

the disease is good. Most often,

the disease goes away within a

few years. About 75 percent of

all patients have only the acute

form of sarcoidosis and, for about

half of them, the disease leaves

no significant problems.

However, sarcoidosis sometimes

stays for years and can cause

organ damage and significantly

reduce physical activity. About

25 percent of all patients have

the chronic form of the disease.

In these patients, the disease usu-ally

leaves scar tissue in the lungs,

skin, eyes, or other organ.

However, chronic cases can be

improved with treatment.

Sarcoidosis–whether acute or

chronic–rarely results in death.

What Does the Future Hold?

Scientists in the United States and

around the world are trying to

learn more about sarcoidosis and

improve its diagnosis and treat-ment.

Much of this research is

being supported by the National

Institutes of Health (NIH), locat-ed

in Bethesda, MD.

One NIH study is called ACCESS,

which stands for " A Case Control

Etiologic Study of Sarcoidosis. "

It followed the varied course of the

disease in a large group of persons

and resulted in new standards for

the diagnosis and management

of sarcoidosis.

Other studies now underway are

trying to find the agent or

substances that cause sarcoidosis.

That knowledge would lead to

much-improved diagnostic tests

for the disease, along with treat-ments

able to target its cause and,

perhaps, a prevention.

Research also is aimed at finding

out why sarcoidosis appears to

behave differently in different

races and why it clusters in some

families. For instance, scientists

are comparing sarcoidosis symp-toms

in African Americans with

those in whites. Other researchers

are seeking clues about how genes,

passed from one generation to

another, may make some members

of a family more likely than others

to develop sarcoidosis.

Still other researchers are examin-ing

how sarcoidosis progresses at

the cellular and biochemical levels.

They want to know what happens

after the agent or substance caus-ing

the disease has invaded the

body—how cells behave and com-municate

with each other to

result in sarcoidosis. Some of these

studies already have led to possible

new treatments, which in turn are

under investigation.

Scientists also are testing new

drug treatments for sarcoidosis.

These drugs include medicines

used for other diseases, such

as thalidomide, pentoxifylline,

and infliximab.

13

1. Is sarcoidosis a form

of cancer?

Sarcoidosis is not a form of cancer.

Also, having sarcoidosis does not

appear to increase a person's risk

of developing cancer. In cancer,

cells multiply out of control and

lack order; in sarcoidosis, cells

act as if they are growing around

an unseen " invader, " forming

granulomas or lumps.

However, persons who have sar-coidosis

may be at a slightly ele-vated

risk for developing some

types of cancer–for example,

lymphomas, and liver, lung, and

skin cancers. The increased risk

may be related to the chronic

inflammation in the organ. The

overall risk is similar to what is

seen in other chronic conditions,

such as diabetes, inflammatory bowl

disease, and rheumatoid arthritis.

Some drugs used to treat sarcoido-sis

have been shown to increase the

risk of cancer, but only when used

in high doses for other conditions.

2. Is sarcoidosis contagious?

Sarcoidosis is not considered

contagious. One person cannot

" catch " it from another person.

3. Is sarcoidosis a genetic

disease?

A genetic disease is one passed

from parent to child. Sarcoidosis

has not been found to be passed

from parents to children. How-ever,

for reasons as yet unknown,

it can occur in families. If one

family member has the disease,

others may be at an increased risk

of developing it. But that risk is

still relatively low.

BOX 3: COMMONLY ASKED QUESTIONS

4. How did I get sarcoidosis?

The cause of sarcoidosis is not

known. Thus, it is impossible to

say how anyone got the illness.

Once the cause is found, it will

lead to improved ways to diagnose

and treat the disease. To help

find the answer, the NIH supports

research in the United States

and worldwide.

5. Why is it so difficult

to make the diagnosis

of sarcoidosis?

Since its cause is unknown,

sarcoidosis cannot be diagnosed

directly. Instead, it must be

diagnosed by a number of factors,

including symptoms, results of

a medical examination, laboratory

tests, and a biopsy. The process

is like putting together the pieces

of a jigsaw puzzle. The pieces allow

the doctor to say, " The diagnosis

is sarcoidosis. " The process gives

a correct diagnosis more than 95

percent of the time.

6. What can I do to avoid

sarcoidosis or to make it

go away?

Since the cause of sarcoidosis is still

a mystery, there is no known way

to prevent the disease. Most experts

do not believe that making envi-ronmental

or lifestyle changes will

affect the course of sarcoidosis.

However, those with sarcoidosis can

help protect themselves by staying

healthy: Do not smoke. Keep away

from substances, such as dusts and

chemicals, which can harm the

lungs. Talk with the doctor about

whether or not to use drugs to help

stop the inflammation caused by

sarcoidosis. Most patients will get

better without treatment.

7. Can doctors tell if the

disease will get worse or

go away?

No. But the types and severity of

symptoms are clues about how the

disease will progress. For instance,

those who have shortness of breath

that grows worse usually develop

a chronic and more severe case of

sarcoidosis. Also, those who have

sarcoidosis of the skin sometimes

develop a chronic and more severe

case of the disease, while those with

erythema nodosum almost always

get better.

8. Can I become pregnant

and have children?

Severe sarcoidosis can reduce the

chance of becoming pregnant,

particularly for older women.

Nevertheless, many women have

given birth to healthy babies

while being treated for sarcoidosis.

In turn, a pregnancy has little

effect on the course of sarcoidosis,

and treatment usually continues

without interruption. Occasionally,

the disease worsens in women

after delivery of a new baby.

Women planning to have a baby

should discuss the matter with

their doctor.

It is especially important for

women with sarcoidosis to have

medical checkups throughout

and after pregnancy.

9. Does sarcoidosis affect

African Americans more

than others?

African Americans have a higher

risk for sarcoidosis than do other

Americans. However, the illness

occurs in every race in the United

States—and throughout the world.

> >>

> >>>

> >>> 4.

> >>> J Clin Endocrinol Metab.

> >>>

<http://www.ncbi.nlm.nih.gov/pubmed?term=diet%20AND%20Primary%20Aldosteronism#>

2011

> >>> Sep;96(9):2813-20. Epub 2011 Jun 1.

> >>>

> >>> Cardiac dimensions are largely determined by dietary salt in

> >>> patients with *primary aldosteronism*: results of a case-control

> >>> study. <http://www.ncbi.nlm.nih.gov/pubmed/21632817>

> >>>

> >>> Pimenta E

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Pimenta%20E%22%5BAuthor%5D>,

> >>> Gordon RD

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Gordon%20RD%22%5BAuthor%5D>,

> >>> Ahmed AH

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Ahmed%20AH%22%5BAuthor%5D>,

> >>> Cowley D

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Cowley%20D%22%5BAuthor%5D>,

> >>> Leano R

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Leano%20R%22%5BAuthor%5D>,

> >>> Marwick TH

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Marwick%20TH%22%5BAuthor%5D>,

> >>> Stowasser M

> >>> <http://www.ncbi.nlm.nih.gov/pubmed?term=%22Stowasser%20M%22%5BAuthor%5D>.

> >>>

> >>> Source

> >>>

> >>> Endocrine Hypertension Research Centre, University of Queensland

> >>> School of Medicine, Princess andra Hospital, Brisbane,

> >>> Queensland 4102, Australia. e.pimenta@...

> >>>

> >>> Abstract

> >>>

> >>> CONTEXT:

> >>>

> >>> Animal studies have demonstrated that dietary sodium intake is a

> >>> major influence in the pathogenesis of aldosterone-induced effects

> >>> in the heart such as left ventricular (LV) hypertrophy and fibrosis.

> >>> LV hypertrophy is an important predictor for cardiovascular

> >>> morbidity and mortality.

> >>>

> >>> OBJECTIVE:

> >>>

> >>> We aimed to investigate the relationships between aldosterone and

> >>> dietary salt and LV dimensions in patients with*primary

> >>> aldosteronism* (PA).

> >>>

> >>> DESIGN AND PARTICIPANTS:

> >>>

> >>> This case-control study included 21 patients with confirmed PA and

> >>> 21 control patients with essential hypertension matched for age,

> >>> gender, duration of hypertension, and 24-h systolic and diastolic

> >>> blood pressure.

> >>>

> >>> MAIN OUTCOME MEASURES:

> >>>

> >>> Patients were evaluated by echocardiography and 24-h urinary sodium

> >>> (UNa) excretion while consuming their usual diets.

> >>>

> >>> RESULTS:

> >>>

> >>> Patients with PA had significantly greater mean LV end-diastolic

> >>> diameter, interventricular septum and posterior wall thicknesses, LV

> >>> mass (LVM) and LV mass index, and end systolic and diastolic volumes

> >>> than control patients. UNa significantly positively correlated with

> >>> interventricular septum, posterior wall thicknesses, and LVM in the

> >>> patients with PA but not in control patients. In a multivariate

> >>> analysis, UNa was an independent predictor for LV wall thickness and

> >>> LV mass among the patients with PA but not in patients with

> >>> essential hypertension.

> >>>

> >>> CONCLUSIONS:

> >>>

> >>> These findings emphasize the importance of dietary sodium in

> >>> determining the degree of cardiac damage in those patients with PA,

> >>> and we suggest that aldosterone excess may play a permissive role.

> >>> In patients with PA, because a high-salt *diet* is associated with

> >>> greater LVM, dietary salt restriction might reduce cardiovascular risk.

> >>

> >

>

November 14, 2011

Sounds very similar to what happened to me when I was put on Vit D, elevated

calcium and all. My former doctor said something similar, that he had people on

20,000 IU so there should be no reason 2000 IU could cause problems for me.

Maybe this is something else that primary care doctors need to learn about us PA

folks - that some of us can't tolerate Vit D supplementation...

> >

> > I also

> > > had calcium and vitamin d dysregulation. I couldnt eat anything with

> > > calcium and vitamin d

> > > even though my vitamin d levels are low.

> >

>

November 14, 2011

I take 100,00UI per week. I dont think i notice any symptoms from it, but I had low calcium and low vit D, and high PTH. All straightened out when I cut back on the salty foods. ============================================================================45-Male-Caucasian, 5'9"- 242lbs, PA Diagnosed 2007 Suspected Hyperplasia-No tumors on CT - No AVS.Meds: 50mg Inspra, 40meq Potassium, 2400mg Calcium, 1000mg Magnesium, 100,000UI Vit D (weekly), 20mg OmeprazoleSide effects: Gynecomastia, stomach inflammation (from potassium citrate)Other Diags: GERD, Hiatal Hernia, Metabolic Syndrome - PreDiabetic, Secondary Hyperparathyroidism caused by Renal calcium leak, Bone Cyct in left Femoral Head and Pelvis. Benign Lung Nodules,

Fibromyalgia, Scarring on Right Kidney Lower Pole, Right Flank PainDASH: Started "sort of" DASHing 5/3/2011Status: Last Urine K/Na ratio was 1.1. But total of Na high alsoInitial Presenting Symptom: Muscle twitching all over body with low normal K, Mg, Ca, Low Ionized Ca, High PTH, low Vitamin DTo: hyperaldosteronism Sent: Monday, November 14, 2011 8:14 PMSubject: Re: Salt and heart size in PA

Sounds very similar to what happened to me when I was put on Vit D, elevated calcium and all. My former doctor said something similar, that he had people on 20,000 IU so there should be no reason 2000 IU could cause problems for me.

Maybe this is something else that primary care doctors need to learn about us PA folks - that some of us can't tolerate Vit D supplementation...

> >

> > I also

> > > had calcium and vitamin d dysregulation. I couldnt eat anything with

> > > calcium and vitamin d

> > > even though my vitamin d levels are low.

> >

>

November 14, 2011

So the high salt diet may have been messing with the Vit D etc as well as the PA?I take 100,00UI per week. I dont think i notice any symptoms from it, but I had low calcium and low vit D, and high PTH. All straightened out when I cut back on the salty foods.

November 14, 2011

Not likely surges of aldo. But high salt diet, low K diet and aldo sets you up for quick reactions to a salt load.Should be getting better as you DASH + MCB. Benicar does nothing in PA if you read my Evolution Article again. CE Grim MD I had a MRI of my heart because on the echo my heart was very big but the EKG was normal. My doc didnt understand this. The MRI report said LVH, cardiomyapthy, r/o sarcoid granulomas. I also had calcium and vitamin d dysregulation. I couldnt eat anything with calcium and vitamin d even though my vitamin d levels are low. I also had bad joint pain and I couldnt tolerate the heat or sun. The doc said these were signs of sarcoid. I first had a CT scan of the lung and it was very clear. As I understand it, sarcoid first appears in the lung. My CT scan was perfect. Then he wanted to do a biopsy of my heart. I refused. Plus the only treatment for sarcoid is steroids and I am definitely not going there. ON to search for another diagnosis. I went to another doc who put me on Inspra and Benicar and most of the 'sarcoid' symptoms went away. I can now eat foods with vitamin d and calcium. I had sun and heat intolerance. Although it is winter time now, I can go out in the sun without it hurting my eyes most days. The muscle pain I would get with eating vitamin d and calcium rich foods has gone away. I still have random joint pain and muscle pain , fatigue and palpitations but not like before. The shortness of breath is also better. I can go up a flight of stairs without collapsing. I am still on a search ,I think I have found it with PA after 15 years of suffering. Thanks God I have not had a massive stroke and on dialysis. It is only God that has saved me. i went for 15 years with bp 170/100- 235/120 despite 5-7 bp meds. I also knew something else was wrong. I had nailed it down to endocrine system. I always felt like i was operating on the flight-or fight system. I used to refer myself to endocrinologist but they wouldnt see me without referral. I would then make an appt with an endocrinogist and lie and say my doc referred me. They would call my doc and cancel my appt. I would get 'surges' of something in my body that would immediately increase my heart rate and bp. Now I know it was probaly a surge of aldosterone. In fact I still get these 'surges' now especially when I get scared or excited. Phyllis 44yo AA Female, LVH, Cardiomyapathy, TIA, Insulin Resistance, Obese, GERD, 6 bp meds previously with bp 190/110, 15 years of htn 170/100-235/120 despite medicine Now: Inspra 50mg daily, Benicar 10mg daily for 3 months, BP averaging 150/85. Trying to dash , I need you complete story and a thumbnail to better answer this. Most likely LVH is from HTN and high salt intake. Why would they think of sarcoid? What is your Hx of low K? CE Grim MD I had a MRI of the heart. It showed LVH, cardiomyapathy and that I should rule out for sarcodiosis. I wonder if the MRI was seeing fibrosis. He wanted to do a biopsy of my heart. I refused- biopsies of the heart dont have good outcomes. I had a CT of the lung and it didnt show adenapathy or nodules in the lung. Phyllis 4. J Clin Endocrinol Metab. 2011 Sep;96(9):2813-20. Epub 2011 Jun 1. Cardiac dimensions are largely determined by dietary salt in patients with primary aldosteronism: results of a case-control study. Pimenta E, Gordon RD, Ahmed AH, Cowley D, Leano R, Marwick TH, Stowasser M. Source Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Princess andra Hospital, Brisbane, Queensland 4102, Australia. e.pimenta@... Abstract CONTEXT: Animal studies have demonstrated that dietary sodium intake is a major influence in the pathogenesis of aldosterone-induced effects in the heart such as left ventricular (LV) hypertrophy and fibrosis. LV hypertrophy is an important predictor for cardiovascular morbidity and mortality. OBJECTIVE: We aimed to investigate the relationships between aldosterone and dietary salt and LV dimensions in patients withprimary aldosteronism (PA). DESIGN AND PARTICIPANTS: This case-control study included 21 patients with confirmed PA and 21 control patients with essential hypertension matched for age, gender, duration of hypertension, and 24-h systolic and diastolic blood pressure. MAIN OUTCOME MEASURES: Patients were evaluated by echocardiography and 24-h urinary sodium (UNa) excretion while consuming their usual diets. RESULTS: Patients with PA had significantly greater mean LV end-diastolic diameter, interventricular septum and posterior wall thicknesses, LV mass (LVM) and LV mass index, and end systolic and diastolic volumes than control patients. UNa significantly positively correlated with interventricular septum, posterior wall thicknesses, and LVM in the patients with PA but not in control patients. In a multivariate analysis, UNa was an independent predictor for LV wall thickness and LV mass among the patients with PA but not in patients with essential hypertension. CONCLUSIONS: These findings emphasize the importance of dietary sodium in determining the degree of cardiac damage in those patients with PA, and we suggest that aldosterone excess may play a permissive role. In patients with PA, because a high-salt diet is associated with greater LVM, dietary salt restriction might reduce cardiovascular risk.

November 14, 2011

I think it is better to just give the link. This is more info than most need to know even me.CE Grim MDOn Nov 14, 2011, at 1:10 PM, Francis Bill SUSPECTED PA wrote:

November 15, 2011

Not sure If I still have the link. Got this back about 2003 when my brother was

DX. Sarcoidosis seems to come up many times here. With about 670 here some may

even have it. Like PA it is missed by many PCP. My brother's was missed by many.

He had many tests done was loosing is his eye site. Eye Dr Dx him. Treatment

restored site.

If you think you have Sarcoidosis they may be just the information you need.

>

November 15, 2011

but we cannot be the medical resource for every possiblitiy that folks may have here. Some are complaining about too much info so think we should try to limit ourselves to HTN and CVD for long attachments. On the other hand we have unlimited space for attachments but limited space for files we upload. I am told. On Nov 15, 2011, at 5:55 AM, Francis Bill SUSPECTED PA wrote: Not sure If I still have the link. Got this back about 2003 when my brother was DX. Sarcoidosis seems to come up many times here. With about 670 here some may even have it. Like PA it is missed by many PCP. My brother's was missed by many. He had many tests done was loosing is his eye site. Eye Dr Dx him. Treatment restored site. If you think you have Sarcoidosis they may be just the information you need. >

November 15, 2011

Phyllis, I stumbled upon this tonight:

Macro- and micronutrients in African-Americans with heart failure

Source: http://www.ncbi.nlm.nih.gov/pubmed/16819577

I wonder if this might provide you some answers or at least something to discuss

with your doctor. I could only see the abstract but it sounded like some of

what you were talking about.

- 65 yo super ob. male - 12mm X 13mm rt. a.adnoma with previous rt. flank

pain. Treating with Meds. And DASH. . Current BP(last week ave): 131/76 HR 60

Other Issues/Opportunities: OSA w Bi-Pap settings 13/19, DM2, and PTSD.

Meds: Duloxetine hcl 80 MG, Metoprolol Tartrate 200 MG, 81mg asprin, Metformin

2000MG and Spironolactone 50 MG.