Lupus - Your first 100 Questions

[Guest guest]

GENERAL ASPECTS OF LUPUS

Q How can I explain lupus to my friends?

A Lupus is a disease in which the

immune system of the body becomes too active. This over-activity affects

the blood, and in turn, almost any organ in the body. The commonest

clinical problems are tiredness, skin rashes, allergies, joint and muscle

pains, and, if the disease spreads, of kidney, lung and other organ disease.

Q Is lupus still considered a rare disease in the UK? As none of my friends have heard of it I

suppose it must be?

A At long last, lupus is being

recognized as the important disease it is in this country. Figures for its

prevalence vary between 1 in 800 and 1 in 1000 women. It is commoner than many

well-known diseases, such as multiple sclerosis. In the 30 years in which I

have been running a lupus clinic in this country, the picture has changed out

of all recognition. When I first started, lupus was considered to be extremely

rare and there was very little research undertaken - happily this has changed

and many physicians now have experience in lupus.

Q Are there different types of lupus?

A There are two main forms of lupus.

Discoid lupus is a milder form of the disease, which involves only the skin,

usually the face, neck and sometimes the upper chest. It may cause raised scaly

skin areas and/or irregular bald spots on the scalp. The second type, which is

usually more severe, is called Systemic Lupus. It involves the internal organs

and systems of the body. Although systemic lupus may be mild, if it is not

controlled it can result in damage to vital organs such as the kidneys, brain,

heart and lungs, and therefore may be life-threatening.

Q Are there more cases of lupus now than say

10 years ago, or is it just better known?

A There are certainly many more cases of

lupus throughout the world. It is conventional for we

doctors to say that the reason for this is “better recognition” of

mild cases, but in my heart of hearts, I think the disease is genuinely on the

increase.

1

Q At what age does lupus usually occur?

A Lupus occurs at any age. It occurs

in the newborn, in rare instances. Some cases appear in childhood

.. The main age groups are the 15-45 year olds and there is a huge female

predominance - women outnumbering men by 9 to 1.

Q When telling somebody I have lupus, they

said that’s just like ME isn’t it? Is there any relationship

as I was lost for words?

A Many of our lupus patients are first

diagnosed with having ME. It must be remembered that “ME” is

a rather diffuse syndrome with widespread aches and pains and stiffness, and in

fact is a mimic for many diseases. Lupus is NOT related to ME in any

direct sense.

Q Is lupus contagious or infectious?

A There is no evidence that it is

either contagious or infectious.

Q What “triggers” lupus?

A During its course, there may be

“flare-ups” when the disease is active and remissions when the

disease is controlled. Perhaps the best-known triggering factor is sunlight.

Infections, injury, surgery, overexertion and exhaustion, nervous tension and

emotional upsets have all been identified as possible precipitating factors.

Certain drugs, such as sulfa compounds, may produce lupus symptoms and cause

flare-ups.

Q Why are lupus patients not being asked to partake in trials

for medication etc. as they are in many other prevalent diseases?

A Lupus patients are taking part in trials throughout the

world - certainly our research work at St ’ is totally dependent on the help of

our patients. I agree that drug trials may be less frequent in lupus than say

in osteoarthritis, rheumatoid arthritis and osteoporosis, where big drug

companies are competing with each other for new therapies in these huge

markets. Lupus, in this respect, has always been a “poor relation”.

However, in terms of scientific and clinical research, collaboration between

doctors and lupus patients is producing results. Take a look at any issue of

the international research journal “LUPUS” for example.

2

Q I have read of a connection between

glandular fever and lupus. Is there any truth in this?

A The virus that causes glandular fever has not

been shown to cause lupus. Having said this there are many patients with lupus

who, especially in their teens and twenties, are given the label of

“recurrent glandular fever”. This is probably because they have had

swollen glands and general malaise and aches and pains - features common to

both diseases.

Q I am a male sufferer, will lupus affect my ability to father

a child?

A No, there is no specific hormone or

reproductive problems in the majority of males with lupus.

Q Why is there a 9 to 1 female to male ratio in lupus?

A We still do not know why there is

such a strong female preponderance, though a lot of research work is focusing

on the effect of various sex hormones on the immune responses. It is also a

mystery because men who develop lupus do not have any particular hormone

abnormalities.

Q My lupus seems to get worse before my periods. Is this

unusual?

A This is very common and a very prominent

feature of lupus. In some patients the joint pains and general tiredness are far

worse in the days before the menstrual period. Perhaps this is another example

of the importance of hormones in the clinical pattern of lupus.

Q Everyone tells me how great I look, but I feel rotten. How

do I tell them about myself?

A You are absolutely right. One of the

biggest problems in lupus is that most patients LOOK completely well. There is

no easy answer to this question. I find that my patients each handle this

problem differently. The help of close friends or family is often vital here, being

better able to explain this disease to the unsympathetic neighbor or colleague.

Q Has anything been found to link lupus with

areas of the country?

A There is no good evidence for this

at the present time. In general local “epidemics” have been associated

more with the training of the doctor and his or her ability to diagnose lupus

cases at an early stage.

3

Q If you have lupus, can you not get AIDS?

A One does not protect from the other.

There have been a few reports from America of both AIDS and lupus in the one patient.

Q How often should my consultant see me for a check-up if my

lupus is still active, or even when it’s calm?

A In our unit we see patients

frequently when the disease is active, or when changes are being made in

treatment, but for those with quiet disease or disease in remission we tend to

see every 6 months. This is not ideal as 3 monthly appointment

would be the norm in many countries, but sadly, our waiting list dictates our

position.

Q What are your views on breast silicone implants? I was

considering this step when my lupus is calm.

A Although the “official”

line is that these do not cause autoimmune diseases, I think there is still a

suspicion hanging over them, especially in people with an autoimmune disease

background such as lupus. Unfortunately the whole issue has been muddied by the

legal side and good research is now hard to come by. My own advice on this, for

what it is worth, is to avoid silicone implants if at all possible.

Q When applying for a job, I have to fill in

a medical form. As soon as this is seen I magically don’t get the job. Do

you feel a lupus sufferer should be discriminated in this way if their lupus is

calm and they are able to do the job that is required?

A Unfortunately the situation you

describe is all too common and in my clinic I spend a lot of time writing

letters in support of my patients who have been discriminated against by the

label “lupus”. I suppose in one way it is understandable if an

administrator or clerk looks up lupus in one of the older books and reads a

saga of doom and gloom; the odds are “stacked against you”. I think

that with general education of the public this is changing for the better but

certainly if your disease itself is not severe you should try to get your

doctor to write a strongly supportive letter. You may know that some of the

members of my extremely busy team at St ’ themselves have lupus.

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Q What does ‘being in remission’

really mean for the lupus patient?

A ‘Being in remission’ means being well as

far as clinical symptoms are concerned. We find many patients who are

clinically very well but in whom the blood tests are still not back to normal.

Definitions vary but, as far as I’m concerned, the clinical features are

far more important than any immunological test results. Although most of us

practicing in the world of lupus feel that the chances of prolonged and

life-long remission are good - certainly after the age of 40 - only time will

tell, and complacency is wrong as far as doctors are concerned.

Q Is it common to progress from mild lupus to

Sjogren’s Syndrome after the menopause, and would this mean that the

debilitating fatigue, myalgia and arthritis of mild lupus, instead of hopefully

disappearing with the menopause, might continue for the rest of my life?

A Yes it is. Many of our lupus

patients around the time of the menopause suffer less

“serious” disease but are left with Sjogren’s Syndrome

(dry eyes, dry mouth and aches and pains). Even these do not necessarily

continue for more than a few years.

Q Is rheumatoid arthritis the same as the

arthritis my lupus gives me?

A No. Rheumatoid arthritis can and does damage

joints. In lupus the joints are not primarily damaged (prolonged high-dose

steroids can in fact, cause joint damage, but this is

a separate issue). Some lupus patients get deformities from tightening of their

tendons, but this is rather different from the erosive or

“corrosive” disease caused by rheumatoid.

5 THE

GENETICS OF LUPUS

Q I have read about a lupus chromosome/genetic link - is this true?

A Yes, almost every department in the world

studying lupus is looking at genetic aspects of the disease. All of us, both

patients and doctors, know that there are families with lupus and genetic

aspects are clearly involved - these are weak but definite. Those who study

genetics (map readers of the gene) are finding clues in lupus, not yet as strong as in other diseases - but major

advances are being made.

Q I am a father with lupus whose son has just

been diagnosed with lupus as well. Is this extremely rare?

A Yes, it is. I have, in fact, one

other family with a father and son involved, but this is within the context of

a clinic seeing 2,500 patients! There are certainly genetic factors in lupus,

although these are not as strong as in many other diseases. Despite the fact

that this association is rare, I still think it underlines the need for lupus

patients who are worried about their offspring having symptoms to have them

tested for lupus.

Q Do men have worse lupus than women?

A No, there have been many studies of

this question and the answer has always been that, in general, there are no

major differences.

Q My daughter of 10 has been diagnosed as having lupus, is the

prognosis any different for a child compared to an adult? Will she always

be chronically ill?

A The prognosis for children has been shown to

be the same as for adults, and NO she will not always be chronically ill. Many

of my young patients are now leading normal lives and are off all treatment.

Q Is lupus exactly the same in white and

black sufferers?

A In general, yes. There aren’t

major differences. Older studies in America have suggested in particular that black

patients had worse disease than white, but these studies were flawed in that

they did not take into account treatment regimes, socio-economic status and so

on. More modern studies show that although there are some differences (possibly

more kidney disease in black patients), these differences are not as

exaggerated as had been previously thought.

6

CLINICAL FEATURES OF LUPUS

THE SKIN

Q Do lupus patients have fluorescent tube

lighting or VDU allergy?

A In general no. Although ultra-violet

wavelength light can make lupus worse, it is only the very, very exceptional

patient who is exquisitely sensitive to UV light and develops rashes in intense

fluorescent lighting. Normal office, shop and home fluorescent lighting is not

a threat to the vast majority of lupus patients.

Q I lost my hair, will it come back?

A Yes. Hair loss is very common during active

lupus and it almost invariably comes back, though there are some patients, with

very severe discoid lupus, in whom patches of hair loss persist.

Q My finger nails are always blue, should I worry about this?

A There are a number of causes of

nail discoloration and it is difficult to give an easy answer to this question.

Certainly lupus (especially discoid lupus) can cause nail changes. So, too, can

drugs - occasionally antimalarials cause a darkening of the nails, but there

are many other conditions and it is worth seeing a doctor, perhaps a

dermatologist.

Q Lupus panniculitis or profundus - what is

it? What triggers it? I have been on plaquenil, steroid creams and injections.

A Lupus panniculitis is a rare

inflammation of the fat beneath the skin, leading to a lumpy, sometimes painful

dimpling of the skin and the tissue beneath it. It is an extension of skin

lupus. It is rare and sometimes slow to respond to treatment (normally

treatment is with plaquenil and occasionally with steroids). The cause is not

known. It is usually not associated with more severe internal lupus.

Q I have a severe reaction to insect bites.

How do I deal with this? Is it common to lupus sufferers?

A Yes, it is very common for

lupus patients to suffer severe adverse reactions to insect bites. My

experience has been that this does, in fact, fluctuate and sometimes, as the

lupus becomes more quiet, the problems are less severe.

7 THE ORGANS

Q I suffered liver damage due to a severe

lupus flare, will it clear up or will I feel tired and unwell always?

A Surprising though it may seem, the

liver is rarely seriously involved in lupus - one of the organs which seems

curiously to escape. When we see a lupus patient with liver abnormalities we

always consider all other options, such as virus infection etc. I’m

afraid in this case it would need your own doctor to answer this specific

question.

Q Does lupus lead to abnormal cervical smears?

A Yes. Abnormal cervical smears are very common

in lupus - though it is very rare that these abnormalities are serious or

pre-malignant.

The reasons for these abnormal

smears are unknown, and certainly my own team at St ’ are studying this observation further.

Q What are the dangers of protein in my urine?

A Protein in the urine is the first

sign, in many cases, of inflammation in the kidney. It is very important and

may or may not require an increase in treatment. It is our practice here in the

Lupus Unit at St ’ to teach patients to test their own

urine for protein. A negative test at least provides a lot of reassurance. A

positive test may require further investigation, especially if the protein in

the urine persists.

Q I suffer from lupus and in particular

trigeminal neuralgia. Is this common? I am on carbamazepine - is this

appropriate?

A Trigeminal neuralgia - pain and pins

and needles along one side of the face, often across the cheekbone, is a common

medical condition. Normally, the cause is unknown. Yes, it is seen in lupus and

perhaps even more frequently in patients who have Sjogren’s

Syndrome (dry eyes and dry mouth). You say that you are on

carbamazepine, which is the most widely used medicine for this troublesome

complaint. It has no adverse effects in lupus patients.

8

Q What is the link between inflammatory bowel

disease and lupus? I have ulcerative colitis and lupus. Are there many

suffering like me?

A There are a small number of lupus patients

who do suffer from inflammatory bowel disease. There is a far greater number

who have “irritable bowel”. The distinction is often very difficult

to make and requires expert gastro-intestinal consultant opinion.

Q How can the hips be affected in lupus

arthritis and what is the treatment?

A The hips can be affected in lupus - usually

mildly. A potentially more serious problem is in patients who have been on

steroids for a long time, where softening of the hips occurs (so-called

avascular necrosis). Sometimes this is sufficiently severe to warrant hip

replacement.

Q I am so moody and depressed, is it the

lupus or the drugs? Is there anything I can do?

A Both the disease and the drugs

(especially steroids) can cause these symptoms. Lupus in particular can cause

lethargy, moodiness and depression. In some patients the depression can become

severe. It is important to recognize this, as medical treatment of the lupus

can help this problem.

Q Do many lupus sufferers have to undergo

spleen removal or are there any other satisfactory means of platelet control?

What are the benefits of spleen removal?

A A small number of lupus patients

undergo spleen removal. In some patients the first manifestation of lupus has

been bruising and a low platelet count, and the original diagnosis is

“thrombocytopenia” (low platelets).

In some, the disease does not respond to conventional medicines, including

steroids, and ultimately spleen removal is required. The results of spleen

removal are largely (though not always) successful and the results in lupus are

as good as those in patients who do not have the disease. There is one negative

as far as spleen removal is concerned and that is an increased risk of certain

infections such as pneumococcal infection. Vaccination for this is vital.

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Q I have a pleural effusion from lupus. Can

it be reversed and what is the treatment now?

A Pleurisy (inflammation of the

lining of the lungs) and fluid in the pleura (so called pleural effusions) are a feature of lupus flares. Yes, they can be reversed,

usually with steroid treatment, and the good news is that they rarely leave any

permanent scarring or side-effects.

Q I bruise easily, is this lupus or steroid

medication?

A Bruising is most commonly a

feature of steroid medication. It is almost universal in people who have been

on steroids for a long time. Lupus patients who are not on steroids, in fact,

have no major problems with bruising, though there is a rare condition in lupus

known as thrombocytopenia (low platelets), and, if bruising is a major feature

and you are not on steroids, then the platelet count should be checked.

10 BLOOD

TESTS IN LUPUS

Q What tests can be done to find out if I have lupus?

A The screening test for lupus is the

anti-nuclear antibody (ANA) test. This is very helpful, some 90 to 95% of

patients being positive. Obviously, from this figure, it is clear that a

negative ANA does not EXCLUDE the diagnosis, but in any patient or family where

lupus is suspected, this is the test the GP should be ordering. If this test is

positive then more specific tests for lupus such as anti-DNA testing can be

arranged.

Q Is a positive ANA always serious?

A No, many people have positive

ANA’s without any symptoms whatsoever (interestingly we find an increased

prevalence of positive ANA’s in relatives of lupus patients).

Q Is it possible I have lupus or a connective

tissue disorder with negative blood result tests? I have a multitude of medical

problems and believe there is some systemic factor involved.

A Yes, there are some lupus patients in

whom the conventional antinuclear antibody tests are negative. In the main,

these are patients with skin rashes and little in the way of generalized

internal problems. The true number of such cases is obviously unknown. A lot

depends on definition, but the agreed figure is probably quite small, 1% to 5%

of lupus patients.

Q Should my young son and daughter

have blood tests to check that they don’t have lupus?

A In general no. If, however, your

teenage son or daughter develop symptoms which worry

you, then I think a screening antinuclear antibody (ANA) test is worth while.

The commonest teenage feature to look out for are

joint pains (“growing pains”), recurrent “glandular

fever” or recurrent migraine headaches.

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Q Should I sit in the sun and find out if

I’m sensitive or can a test be done?

A I wish there were tests which could

forecast who is sun sensitive and who is not. Matters are more difficult as the

tendency to photosensitivity may fluctuate. Some patients whose disease has

gone into remission appear to be no longer sun-sensitive. In general, the

patient knows best and treats the sun with caution.

Q Why is my ESR always important when blood tests are taken?

A The ESR is a useful barometer of disease

activity. It is not specific for lupus. For example, it can go up in anything

from influenza to malaria, but it does provide a general idea of whether there

is inflammation in the body or not. It is cheap and easy to measure and

therefore one of the first tests which is carried out in someone who is unwell.

Q My white count is decreasing slowly, now at

2000. All my other tests are normal and my kidney function is good. I have had

lupus nephritis and was on cyclophosphamide and went into remission. I am on

10mg steroid, alternate days, Istin 5mgs and Innovace 5mgs at night.

A It is common for lupus patients to

run a low white cell count, often around 2,000, and many such patients manage

very well with this surprisingly low blood count. Obviously it is the concern

of both doctors and patients that cyclophosphamide may have been responsible

(you do not say how long the cyclosphosphamide was given). From what you say, I

would not worry unduly at this stage about the white cell count, unless it

continues to fall.

12 THE

TREATMENT OF LUPUS

Q Do lupus consultants disagree about the

treatment of lupus in specific cases or in general? I have often been given opposite treatment

regimes when my particular consultant is away.

A In general there is surprising

unanimity amongst consultants dealing with lupus. The annual international

conferences such as that of the American College of Rheumatology and the international

lupus meeting generally show huge agreement concerning progress in management.

Yes, there are differences, and it is very sad to hear that you have been given

different opinions by different doctors. Are these all lupus experts? I must

say, as a rheumatologist, I find that our work in lupus involves more agreement

and co-operation between specialists and across nations than any other aspect

of my job.

Q I have been told that having lupus I should

not take sulphonamides - what are they and are there any other drugs I should

avoid?

A It is certainly true that lupus

patients have a very high percentage of adverse reactions to sulphonamides.

Luckily these drugs are rarely given nowadays. A related drug, however -

septrin - is still used for urinary infections and I advise my lupus (and

Sjogren’s) patients to avoid this drug if possible. Adverse reactions to

septrin, notably rashes, are almost universal in our lupus patients. Tattoo it

on your forearm “Don’t give me septrin”.

Q Can I have a flu

jab? Should I anyway?

A Patients with lupus do not appear to

be at risk from flu, though it can be argued that sick patients are at extra

risk for the more serious complications of flu and would therefore be wise to

obtain a flu jab. Some lupus patients in my experience certainly do suffer

uncomfortable reactions to flu jabs, though whether more frequently than

non-lupus individuals is uncertain.

Q I am now 70 years of age and have had

active lupus for 20 years. My doctors had told me after diagnosis that 5 years

was my limit. Am I unusual or just very lucky?

A No, you are not unusual. In the old

days doctors used to think that lupus invariably had a poor prognosis, numbered

in months or a few years at most. There are many like you and it is a

reflection of how tremendously improved the prognosis is now recognized to be.

13

Q What do you think of new “wonder

drugs” to treat osteoporosis? Will it help my steroid-induced

osteoporosis?

A Yes, there are major advances in the

management of osteoporosis. Many patients with lupus have been on long-term

steroids and osteoporosis is a real problem here. The careful use of hormone

replacement treatment and the new vitamin D-like agents have been very useful and

definitely advantageous in the management of these problems.

Q Do you believe diet plays a part in lupus

control?

A Yes I do. A number of patients have

allergies to certain foods, usually shown by an increase in joint pains. There

is no rule about this and I always advise patients to keep a mental diary of

days on which their joints and other symptoms seem to be worse in order to try

and pin-point a pattern.

Q What is Raynauds disease and how can I help myself in

the winter?

A This is a condition where the fingers become

extremely cold and white. It is worse in cold weather and can be severely

disabling. Great care is required in winter and many patients need to wear

gloves at all times outdoors. Very occasionally some patients have required electrically-heated

gloves.

Q What should I do when I have active lupus

and get a bad cold or flu?

A There are no particular extra requirements

in lupus as far as colds are concerned. Flu is a much more aggressive virus

infection and patients on steroids may need a slight increase in steroid dosage

under the guidance of their doctor.

Q I would very much like to cheer myself up

and go blond. Is it safe to have bleach put on my

hair, could I also have a perm?

A Yes, by all means do so. When lupus

is moderately under control, then most patients get away with treating their

hair in a variety of ways! When lupus is active and hair loss is a feature, the

hair is much more delicate and at this stage it is certainly clinical

experience that hair dyes, bleaches and so on, can be a problem.

14

Q Due to antibodies in my blood I cannot be

found a donor to match me for a blood transfusion. Is this peculiar to me, or to other lupus sufferers as well?

A This is unfortunately a problem sometimes seen

in lupus patients. Lupus patients have a whole variety of antibodies and in

some cases these do cause miss-matching problems in blood transfusions. It is

not peculiar to you and needs very careful checking with blood transfusion

experts.

Q I have developed osteoarthritis in both

knees and I have been told by my surgeon that replacement is necessary. He is

reluctant because I have lupus. Is his concern justified or can I undertake it

without a considerable risk of my lupus flaring up? I am 69, have had lupus for

50 years and am able to lead an acceptable existence.

A There is certainly nothing against having

surgery in systemic lupus, and many of our patients, for various reasons,

undergo surgery. If the lupus itself is active, it is the normal practice for

the anaesthetist to increase the steroid dose, but when the disease remains

quiet there may be no need to take any special medical precautions.

Q I have kidney-affected lupus, will I recover from this when

my lupus is calm?

A Yes. The kidney is an organ which scars and the

aim of modern treatment is to try to catch inflammation in the kidney early on

in order to “put out the fire” as quickly as possible and to avoid

permanent scarring.

Q Are kidney transplants required for those

patients with lupus-induced kidney disease?

A For those patients who have

developed end-stage kidney failure, transplantation has been very effective

indeed. It perhaps came as something of a surprise in the early days that the

lupus didn’t recur and damage the kidney. Oddly enough, it is unusual

following renal transplantation for the underlying lupus itself to remain

active.

Q Is plasma exchange still a good option?

A In very occasional cases plasma

exchange is helpful but it has not proved as universally effective as the early

reports had hoped - the usual story!

15

Q I have started swimming regularly and am

concerned that it will aggravate my joints.

A Swimming is one of the best sports for

people with lupus, especially those with joint problems. It often surprises

both patients and doctors how much improving muscle tone helps the joints.

Q What do you think about alternative

therapies?

A Perhaps it is not surprising that quite a

number - perhaps the majority - of patients attending our Lupus clinic also

undergo alternative therapy and I have nothing against it. My only caution here

would be not to fall into the hands of people who are charging large sums of

money for what is, after all, unproven therapy.

DRUGS

Q I sometimes think the side-effects of the

drugs helping my lupus cause greater problems than the

illness - am I right?

A Many of the treatments used in lupus

have side-effects. Some of the problems of lupus, such as kidney disease, may

not be painful and may go unnoticed and it is not uncommon for a patient when

first started on treatment to feel that they are going backwards rather than

forwards. On the positive side, during the past 20 to 30 years the management

of lupus has become much more precise, and “fine-tuning” has helped

make the old vision of patients on long-term high-dose steroids less common.

Q What are the dangers of stopping

one’s dosage of prednisone?

A Stopping steroids suddenly can be dangerous. The adrenal

glands may be suppressed and fail to work properly and the patient may end up

in the hospital in a matter of days. So, it isn’t a good idea to stop prednisone without being under a

doctor’s supervision. Generally, it is best to taper the dose slowly over

a period of months.

Q Is pulse therapy preferable to taking

steroids orally?

A Most physicians use injections (a

drip) of steroids in the early stages of management, especially when it is

urgent to get things under control. Repeated pulses are rarely given, and once

the patient’s disease is brought under control by the initial treatment,

most physicians use steroid tablets.

16

Q How does a consultant know which

antimalarial drug to prescribe when there are so many?

A Different antimalarials have different effects and

different side-effects. Hydroxychloroquine (plaquenil) is by far the safest and

most widely used. It is not as strong as mepacrine, but mepacrine is limited by

the major side-effect of causing skin pigmentation (a yellowish color when used

in higher does). Chloroquine (the older-used antimalarial) is probably more

toxic and almost certainly is the drug which had the problem of eye toxicity.

It is rarely used in this country. Plaquenil in very low dose (eg one 200mg tablet

daily) is a very safe drug indeed

Q Is it dangerous to take plaquenil long

term? Can it damage my kidneys or liver? I take 200mg a day. Can your body

become used to the drug so that it is no longer as effective?

A Plaquenil is one of the safest medications

that we know and many of our lupus patients around the world have been on this

drug for years on end. It is very rare for it to cause internal organ problems

such as damage to the kidneys or liver and blood tests are not required. The

major limiting factor is eye testing but a small dose of one per day (200mg per

day) is now believed to be very safe indeed.

Q Can my sperm count be

reduced by the drugs I take for lupus?

A Certain drugs (particularly

immunosuppressive drugs) do reduce sperm count. Fortunately the majority of

drugs used in moderation in lupus (including steroids) do not have any adverse

effect on the sperm count.

Q Are there any new medications for lupus

discovered during the last few years?

A A number are being tried, but

few can justifiably be said to be routine as yet. The major advances in the

last 10 years or so have come from different ways of using old drugs, for

instance more conservative steroid regimes, injections of stronger drugs such

as cyclophosphamide rather than tablets, the more widespread use of

antimalarials etc.

17

Q I have read recently in the newspaper about

“stem cell transplant” and bone marrow transplant for lupus

sufferers. Is this true?

A Yes, over the years a number of patients

with diseases such as lupus, scleroderma and rheumatoid arthritis have been

seen to improve following marrow transplant (or stem cell transplant). This

aggressive therapy has been used for certain malignancies such as leukemia and

the improvement in the arthritis etc. has been coincidental. Now, however, with

improvements in stem cell techniques, this treatment is being seriously considered

for certain patients with severe connective tissue diseases such as lupus. The

data is still very anecdotal at this stage.

Q I read of a claim that the hormone DHEA has

helped to diminish symptoms of lupus in a small group of women. Do you agree?

A There are many people who have been

advocates of the value of DHEA in lupus, but, until recently, there was little

in the way of scientific trial. In the United States there is now an on-going scientific trial.

Watch this space........

Q What are your views on methotrexate in

lupus? My most common complaint is generalized joint pain which is unresponsive

to non-steroidal anti-inflammatories.

A Methotrexate is an extremely

useful drug. It has revolutionized the rheumatology world and is perhaps the

best-ever drug in the management of rheumatoid arthritis. Its use in lupus to

date has been largely confined to those patients with moderate to severe joint

problems, and it has not been as widely assessed in lupus as it has in

rheumatoid. Anecdotally, a number of patients have done very well on

methotrexate.

Q Is it true that thalidomide is being used

for lupus patients and why?

A Yes, thalidomide is now being

used in very severe cases of skin lupus, particularly some forms of discoid

lupus. Obviously, in view of its toxicity, it can only be used in patients who

will not become pregnant. It is being used under strict monitoring conditions

at the present time, but it certainly does seem to have a place in the management of lupus in some patients.

18

Q I get recurrent infections from

lupus, can I continually take a mild antibiotic to prevent this occurring?

A In general doctors are against this.

Long term antibiotics, except for specific reasons, bring about their own

problems such as increased chance of fungus infections (thrush etc.) The major

cause of infections in lupus is steroids and this more than anything else is

the aspect which needs looking at in patients with lupus who have recurrent

infections.

Q I am so depressed, if it’s the steroids

should I counteract them with anti-depressants?

A Depression is a major feature in

some lupus patients. Clearly, steroids themselves can contribute to depression

but, more often than not, it is the disease which is associated. Certainly it

is important, if depression does become severe, to obtain the correct

treatment. For example, in our clinic we have a strong link with a

neuro-psychiatrist who has a particular expertise in lupus and tells us whether

or not medication is required. A number of our patients do benefit, not only by

treatment of the lupus itself, but by direct treatment of the depression.

Q Can my drugs given for lupus make me feel worse than the lupus itself? I am told they have worked

in calming the lupus, but I feel awful.

A Very definitely. High dose steroids over a long period have

enormous side-effects and it is one of the major jobs of lupus doctors to try

and taper down the treatment. I often tell patients that it is far harder to

always strive to reduce the medication - it is very easy to prescribe a high

dose of steroids knowing that this has a temporary benefit. Too much treatment

means that the side-effects are sometimes worse than the disease itself.

19 PREGNANCY AND

LUPUS

Q Having suffered severe lupus, it is now

calm and we are thinking of starting a family. Should I tell my GP? Will my

lupus react to my pregnancy and should I increase my steroids?

A From what you say, there seems every chance of

a successful pregnancy. Clearly, it is important to know that the lupus is

relatively calm, both from the clinical point of view and from the tests, that

the blood pressure and kidney function are reasonable and that the

anticardiolipin antibody levels are not high (patients with high

anticardiolipin antibodies have a higher risk of miscarriage and this can now

be largely prevented). There is no specific need to routinely increase the

steroids just because of the pregnancy.

Q I am scared of having a “flare-up” of lupus

after a natural birth. What precautions can be

taken?

A Statistically, the chances of having

a flare in lupus are higher after delivery. Having said this, the chances are

still small - only a small minority of our lupus patients

have flares in the few months after delivery of the baby. Nevertheless,

we like to watch our patients more closely at this time and monitor the urine

and blood tests more frequently. If the test results become more abnormal, then

we can at least step in earlier with more active treatment.

Q I have been found to have antibodies in my blood common to

lupus. I am now pregnant, having suffered two miscarriages. Do I have lupus

when pregnant but the disease is dormant when not?

A This is a very important question and I will

try to answer it clearly. If you have had two miscarriages and have antibodies

in the blood it is quite possible that there is a connection. The most

important antibodies are anticardiolipin antibodies which has been associated

with a tendency to recurrent miscarriage. In answer to the second part of your

question however, it does not mean that you have lupus when pregnant, the

disease being dormant when not. If you do have anticardiolipin antibodies and

are planning a further pregnancy you really should keep in close touch with

your obstetrics doctor regarding the possibility of using low-dose aspirin as

an anti-clotting agent. You may wish to find more about pregnancy and lupus

from our two web sites listed on page 26.

20

Q My baby is to be delivered by caesarian

section at 36 weeks to prevent my lupus flaring. Is this a good idea or not? I

am worried about having a premature baby.

A Obviously, there are many

individual reasons for planning an early caesarian section, but do rest assured

that this is common and generally beneficial for the baby. You really should

not worry about having a premature baby at 36 weeks.

Q Can azathioprine be used in pregnancy?

A The answer, perhaps surprisingly, is yes,

although obviously common sense and good practice dictates that the fewer drugs

used in pregnancy the better.

Q Should I not consider the pill as a

contraceptive means? I

have a problem in that other methods don’t suit me.

A Many years ago (and more

recently) we have looked at the pill in our lupus patients

and have found that in general there are no major problems,

especially with the mini pill. A small group of patients with antiphospholipid

antibody (or anticardiolipin antibody) have more of a clotting tendency and

obviously these patients present a totally different problem.

Q Is HRT (hormone replacement therapy)

advisable?

A We have analyzed the effects of HRT

in our lupus patients and it may come as something of a surprise that the

majority of patients with lupus who go on HRT do not appear to flare in any

way. In America HRT is known as estrogen replacement

therapy - ERT.

21 ASSOCIATED

CONDITIONS

HUGHES SYNDROME

Q I have been diagnosed as having

“sticky blood” and receive treatment for it. I have so many

problems with my health and have been prescribed everything possible to help,

but to no avail. Everything I have read about lupus sounds “just like

me”, but is it possible that I may have this condition and for it to have

remained unaffected by all the various drugs I’ve tried?

A The “sticky blood” syndrome is

extremely important and is increasingly recognized as a major part of the

symptoms in some lupus patients. Symptoms vary from headache, migraine, memory

loss, fatigue, through to clots in the vein and more serious problems. It has

become a major issue for doctors who now realize that not all lupus

manifestations require steroids - some require measures such as aspirin to thin

blood. For those who want to read about this syndrome (sometimes called the

antiphospholipid or ’ Syndrome), there is a booklet from LUPUS UK. Also see our web sites on listed on page

26.

Q Due to having Syndrome, I was

prescribed warfarin. This has helped my lupus, is that possible?

A Very definitely. One of the major advances in our

understanding of this syndrome has been that not all patients require steroids.

In those patients who have Syndrome (or, as the media call it,

‘sticky blood’), the treatment is not steroids but ways of thinning

the blood (either aspirin in milder cases, or warfarin where the clotting has

been a major problem).

We have many, many

patients in whom the whole illness has improved once they started warfarin.

This is particularly true in patients who have had brain or other cerebral

features. There is undoubted improvement in some of these patients in speed of

thought process etc. when blood thinning treatment is started. Your observation

is a very important one.

22

Q I have Syndrome, why do I have to be on warfarin for

life?

A As the syndrome has only been

known for just over 15 years, it is still difficult to know what the true

answer to this is. For those patients who

have had serious clots or strokes, then it is our own policy to advise anticoagulation

for life. For less life-threatening clots, then probably compromise is

necessary. We are just about to embark on a major study of different

anticoagulant regimes and I hope to keep you posted on the progress of this.

Q Is it wise to have an angioplasty for cold

toes if I have antiphospholipid antibodies?

A Patients with antiphospholipid

() syndrome have a tendency to clot. Therefore any procedure, including

injections, angioplasties (procedures involving blood vessels) are at an increased

risk from clotting. The physicians involved are normally well aware of this and

watch the anticoagulant cover during such procedures very carefully indeed.

Providing this is done, there is no reason against having this procedure

performed.

Q Are aspirins contra-indicated in lupus? If

not, what is the correct dose, as my pharmacist and GP disagree on this matter?

A Definitely not. In fact, with patients having the

antiphospholipid () syndrome (sticky blood), aspirin is the first line of

treatment. “Junior” aspirin 75mgs daily is very helpful in stopping

the platelets of the blood sticking to each other and has helped many patients

with this syndrome, particularly those who have had clotting of the placenta

and recurrent miscarriages.

Q Are there many people who have APS?

A It has always been my belief

that Antiphospholipid Syndrome (APS) will one day come be recognized as being

more common than lupus. Different specialties recognize that their patients

with migraine, with multiple sclerosis, with early strokes, with memory loss,

with vein thrombosis, with recurrent miscarriage, could in fact be cases of APS

and therefore potentially treatable.

23

Q Are lupus patients more susceptible to

strokes and therefore should take aspirin daily?

A The discovery of “sticky blood” -

the antiphospholipid syndrome - in 1983 has been important in defining a

sub-group of lupus patients who are more prone to clotting, including strokes.

All lupus patients should be tested for anticardiolipin antibodies, an

inexpensive and vital test, and if positive there is a strong argument for

taking one junior aspirin every day as a preventative measure.

Q Dr , now you have discovered and

developed understanding of Syndrome, what is your next aim?

A Our work concentrates on the mechanisms of why

abnormal clotting occurs in some lupus patients ( Syndrome) and on the

best means of stopping this. The implications of this syndrome are enormous.

Patients who are attending neurology clinics for strokes, for example, vascular

surgery clinics for DVT’s and a variety of other seemingly unrelated

problems are coming to be recognized as having this syndrome. Most important of

all is that between 5% and 25% of all women with recurrent miscarriages have

this syndrome and it is an important and potentially very treatable cause of

miscarriage. Even more exciting is the possibility that some patients wrongly

diagnosed as “multiple sclerosis” or even Alzheimers, could in fact

have Syndrome and be effectively treated.

MIXED CONNECTIVE TISSUE

DISEASE

Q MCTD, how common is this and is it directly

related to lupus?

A Mixed connective tissue disease

(MCTD), despite its rather cumbersome name, is an important condition closely

related to lupus. The name “mixed” was given to it because it has

features suggestive of more than one connective tissue disease, often with

joint pains, muscle inflammation and especially with cold fingers (Raynaud’s). It differs from lupus in

that kidney disease is distinctly rare.

24

Q Is auto-immune disturbance one disease with

many and variable symptoms, or many diseases of symptoms clusters? I have been

told over the years that I have lupus, scleroderma, Raynauds, MCTD, Sjogrens and am completely

puzzled.

A The symptoms and signs of lupus and related

diseases are very varied, and you have obviously been through the mill as far

as diagnostic labels are concerned. Mostly diseases do fit into one or other

category, but there are some examples such as mixed connective tissue disease

where the overlap is considerable, and obviously doctors as well as patients

can get confused. Hopefully this is slowly improving as education in lupus and

connective tissue diseases is more widespread.

SJOGRENS SYNDROME

Q Why do I have sore eyes?

A A Swedish eye doctor named Hendrick

Sjogren noticed that a number of his patients, in addition to having dry eyes

and mouth, also had rheumatism. The term “Sjogren’s syndrome”

has now been given to this condition - dry eyes, dry mouth, rheumatic

disease. The rheumatic disease in question can be lupus, rheumatoid or a

variety of other connective tissue diseases. The severity of the eye and mouth

(and sometimes vaginal) dryness varies enormously. Many lupus patients in their

50s, when the more life-threatening aspects of the disease have abated, are

left with the less serious but still troublesome features of the dryness. The

eyes are treated with artificial tears (methyl-cellulose), but at present there

aren’t any really satisfactory treatments for the mouth dryness.

DISCOID LUPUS

Q How serious is discoid lupus?

A Generally, discoid lupus tends to

remain confined to the skin. In those that have typical discoid lupus about

five percent disseminate, but in the majority of cases it tends to remain

localized. Despite its better prognosis, it nevertheless can cause widespread

skin and scalp problems and required continuous and careful treatment.

25 OVERLAP SYMPTOMS

Q I have lupus, Sjogrens Syndrome and Raynauds. I take Plaquenil. I am controlled,

although I still have weight loss and most worryingly, memory lapses, learning

difficulties and disturbing dreams. Should I accept this situation? Please

enlighten me.

A I though it appropriate to keep this

question until last as it is so important.

The fact is that in many

patients the clinical picture does not seem to fit into one particular disease

pattern. Although blood tests, especially sophisticated ANA testing, has helped

to define different connective tissue diseases far more precisely, there are

some patients where the unsatisfactory diagnosis “overlap syndrome”

is made. In this situation, it is the clinical features more than

the diagnosis which are important.

In this question, for

example, the memory and other problems may be due to medication. However, it

may be due to “sticky blood”. Have you been tested for

antiphospholipid antibodies?

If not, why not?

The antiphospholipid syndrome (“sticky blood”) is a feature of a number

of patients with lupus and Sjogrens. One of its most frequent and prominent

features is memory loss. And it is treatable - often by simply adding in junior

aspirin, once daily.

Back to your doctor!

Straight away!

Hugs,

Deanna