Re: Tim (U.K.) ... what are the different stages of PSC?

[Guest guest]

Hi Tim;

As your message was addressed to " others " I thought I would try to

reply concerning your PSC staging questions. A good article on this

is by Angulo and Lindor at the Mayo Clinic, Rochester, MN:

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:

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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]

Thanks .

You do well to comb all this research so others like me don't have

to! I have a reasonable idea of things but don't have the time &

energy at present to spend too long on it. I am fortunate now to have

been transferred to the top doctor in the uk for psc. I realise now

from what u say I am at stage 4, and indeed last time I saw my

doctors they said i will need tx prob within 2 years. I also have to

think about how long i can work as fatigue gets bad. I had 2 weeks

off over christmas and felt great whereas leading up to xmas i was a

zombie. I guess work takes it out of u (+my 3 kids!).

I hope does well. I always think it must be worse being a

parent of someone with this than having it yourself. I know I wd

rather have it than my kids!

Thanks 4 yr help.

Tim

> Hi Tim;

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