Re: Staging? What is it?

[Guest guest]

Hi Deb;

There are basically IV stages of PSC, diagnosed from the

histology/pathology observed in the liver biopsy.

The following is copied from a message posted earlier this year

(message # 59434) about staging of PSC, derived from the following

article:

Angulo P, Lindor KD 1999 Primary biliary cirrhosis and primary

sclerosing cholangitis. Clin. Liver Dis. 3: 529-570.

Here's a page from this paper describing PSC diagnosis and staging:

_________________

Patients with PSC are at high risk for acute and recurrent episodes

of bacterial cholangitis. In these patients, choleclocholithiasis,

dominant stricture, or bile duct cancer should be considered as the

precipitating factor and should prompt cholangiography. In addition to

extraction of stones and balloon dilatation with or without stenting,

broad-spectrum antibiotics therapy is necessary.

Table 7. SYMPTOMS AND SIGNS AT DIAGNOSIS IN PRIMARY SCLEROSING

CHOLANGITIS

Symptom or Sign Frequency (%)

Symptom

Fatigue 75

Pruritus 70

Jaundice 65

Weight loss 40

Fever 35

Sign

Hepatomegaly 55

Jaundice 50

Splenomegaly 30

Hyperpigmentation 25

Xanthomas 4

Inflammatory Bowel Disease

Ulcerative colitis 70-75

Crohn's disease 5-8

Like patients with PBC, patients with PSC have an increased

prevalence of associated disorders. The associated conditions are

ulcerative colitis (in 70%-75% of patients), Crohn's colitis (in 5%-

8% of patients), pancreatitis (in 10%-25% of patients), and

diabetes mellitus (in 5%-10% of patients). Ulcerative colitis in

patients with PSC often shows extensive involvement of the colon but,

paradoxically, often follows a relatively benign course. Rare

associations with PSC include sicca syndrome, Riedel's thyroiditis,

retroperitoneal fibrosis, celiac disease, and autoimmune hemolytic

anemia.

Diagnosis

The diagnosis of PSC is based on a combination of clinical (Table 7),

biochemical, radiologic, and, in some cases, pathologic finding.

Radiologic Features (Cholangiographic Findings)

Diffuse multifocal annular strictures of intrahepatic or

extrahepatic bile ducts

Short bandlike strictures

Diverticulum-like outpouchings

Histologic Criteria (Ludwig Staging System)

Portal stage (stage I)

Portal hepatitis (limited to limiting plate)

Periportal stage (stage II)

Periportal fibrosis/inflammation beyond limiting plate

Septal stage (stage III)

Septal fibrosing/bridging necrosis

Cirrhotic stage (stage IV)

Biliary cirrhosis

Biochemical Tests

Almost all patients with PSC have elevated serum alkaline phosphatase

levels, usually three to five times normal. Similarly, most have a

mild increase in serum AST or ALT. Serum bilirubin levels fluctuate,

but high levels suggest progression of the disease or development of

complications such as cholangiocarcinoma or dominant strictures with

or without cholangitis. Tests related to copper metabolism are almost

always abnormal in patients with PSC. Several non-organ-specific

autoantibodies can be found in patients with PSC, in particular ANCA,

but none of them is disease specific.

Radiologic Features

Cholangiography is the most important diagnostic test. Endoscopic

retrograde cholangiopancreatography is the procedure of choice, 142

but in some patients with extensive involvement of the common bile

duct in whom ERCP is unsuccessful, percutaneous transhepatic

cholangiography for visualization of the distal intrahepatic bile

ducts is indicated. In most cases of PSC the characteristic

cholangiographic changes described in Figure 2 can be seen.

Although highly suggestive of PSC, these cholangiographic features

are not unique to PSC. Other diffuse liver diseases, such as hepatic

metastasis, advanced cirrhosis, polycystic liver disease, and

lymphoma, may produce similar deformities of the bile ducts, and they

should be excluded. Rarely, the pancreatic duct may be involved and

demonstrate abnormalities suggestive of chronic pancreatitis.

_________________

From our own experience with our son, (diagnosed with PSC,

stage II this summer) both an ERCP and liver biopsy are required to

accurately stage the disease. But not all Medical Centers are equally

capable of staging PSC from liver biopsy samples! It was only when we

had 's liver biopsy samples sent from Indiana to Mayo Clinic

(Rochester, MN) that we were able to get a definitive answer in our

son's case.

Hope this answer's your questions?

Best regards,

Dave

http://home.insightbb.com/~rhodesdavid/

[Guest guest]

Deb,

This was a good overview that posted regarding staging. I

would only want to make a couple of points as to why you had not

heard this terminology before.

As says, staging requires both ERCP/MRCP testing along with

liver biopsy. Staging of PSC is controversial among doctors for a

couple of reasons. 1) The results from a biopsy only show a small

sample of liver tissue. Therfore, without multiple tests it really

just comes down to luck of the draw. Without multiple tests to show

a pattern the staging may be somewhat inaccurate. 2) Staging does

not always correlate to length of time prior to transplant. One

person may be stage III but remain in that stage for many years with

slow progression while another person classed as stage II may

progress rapidly and need a transplant well before the other person

might. 3) Liver Biopsy is a procedure that does carry risk. Many

doctors do not wish to put patients through this procedure

unnecessarily to yield little practical information. 4) I think

doctors are reluctant to tell patients what stage they are in out of

fear of creating needless worry or stress when stage really has

little to do with ultimate progression and time to transplant. It is

really just one piece of a complex puzzle.

Others may chime in but that is my opinion regarding lack of

importance placed on this " label. "

in Seattle

UC 1991, PSC 2001

> Hi Deb;

>

[Guest guest]

> This was a good overview that posted regarding staging. I

> would only want to make a couple of points as to why you had not

> heard this terminology before.

Someone will please correct me if I am wrong, but in addition to the

points made by about the usefulness of staging PSC, might it also

be that when PSC overlaps with AIH (autoimmune hepatitis) - as it does in

Deb's case as well as in mine - that the possible sequential or

simultaneous nature of the two diseases complicates the whole PSC

staging criteria?

My somewhat repressed memory of my own diagnosis of AIH 17 years ago was

that cirrhosis was already present then and thus, as I understand it, some

of my current cirrhosis is not related to the progression of PSC.

(Maybe? And does it even really matter?)

Best Wishes,

Shauna (28, Graduate Student, AIH'86, Crohns'95, PSC'99, listed@Duke,

MELD=17)

--

Talk to your family about organ donation

http://www.shareyourlife.org/flash%20Coalition%20PSA.swf

[Guest guest]

Hi all,

Thanks for your responses. Now I know why I don't know my staging. I

had a HORRIBLE experience with my first liver biopsy. The doctor I

had then said that I would definately have a diagnosis if I had the

procedure done. When the biopsy showed what was consistant with an

allergic reaction rather than an autoimmune disease as all of my

other clinical symptoms showed, he was at a loss. Of course, I would

have felt more comfortable with continuing to work with him if he'd

even SHOWED UP to do my biopsy. I was fresh young and inexperienced

in the medical world, and when this guy didn't show up, some roving

doctor in the hospital did it. I ended up with the worst pain of my

life (still not topped by anything to do with my liver since and I've

been to the ER for pain meds a number of times - nor was it worse

than childbirth!).

Anyway, my current doctor knows of my aversion to biopsy given this

prior experience and he's waiting for the liver committee to decide

they need it for some reason before putting me through another...

Since staging doesn't indicate time to transplant accurately, I'm not

going to worry about it, but will understand if someone mentions

needing that information in the future. Thanks again, Deb

PS - If anyone is in the Northern VA area looking for an heptologist,

I'd be happy to tell you the name of my current doctor as well as the

name of the lousy one I used to go to...

AIH 1997 (confirmed 2000), PSC 1998, UC 1999, listed for tx 2001

[Guest guest]

Deb

We're in northern Va and are curious about your docs--past and present--in case my partner (age 49, PSC dx'ed in 80s) gets out of the military and needs a referral...Sorry to hear about your bx experiences, by the way--sounds gruesome!

Phoebe

Re: Staging? What is it?

Hi all,Thanks for your responses. Now I know why I don't know my staging. I had a HORRIBLE experience with my first liver biopsy. The doctor I had then said that I would definately have a diagnosis if I had the procedure done. When the biopsy showed what was consistant with an allergic reaction rather than an autoimmune disease as all of my other clinical symptoms showed, he was at a loss. Of course, I would have felt more comfortable with continuing to work with him if he'd even SHOWED UP to do my biopsy. I was fresh young and inexperienced in the medical world, and when this guy didn't show up, some roving doctor in the hospital did it. I ended up with the worst pain of my life (still not topped by anything to do with my liver since and I've been to the ER for pain meds a number of times - nor was it worse than childbirth!). Anyway, my current doctor knows of my aversion to biopsy given this prior experience and he's waiting for the liver committee to decide they need it for some reason before putting me through another... Since staging doesn't indicate time to transplant accurately, I'm not going to worry about it, but will understand if someone mentions needing that information in the future. Thanks again, DebPS - If anyone is in the Northern VA area looking for an heptologist, I'd be happy to tell you the name of my current doctor as well as the name of the lousy one I used to go to...AIH 1997 (confirmed 2000), PSC 1998, UC 1999, listed for tx 2001