Re: What is frequency of Chrohn's patients also having PSC?

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Denmark:

Scand J Gastroenterol. 1997 Jun;32(6):604-10.

Hepatobiliary dysfunction and primary sclerosing cholangitis in

patients with Crohn's disease.

Rasmussen HH, Fallingborg JF, Mortensen PB, Vyberg M, Tage-Jensen U,

Rasmussen SN

Dept. of Medical Gastroenterology, Aalborg Hospital, Denmark.

BACKGROUND: Only a few studies have attempted to determined the

prevalence of long-standing abnormal liver function and primary

sclerosing cholangitis (PSC) in patients with Crohn's disease (CD).

The aim of the study was to determine the prevalence of long-standing

abnormal liver function test results and to describe the clinical,

biochemical, and histologic findings in patients with large-duct

classic PSC and small-duct PSC (that is, normal cholangiogram) in

patients with CD during a 15-year period. METHODS: Patients with CD

and long-standing abnormal liver function results were investigated

individually with endoscopic retrograde cholangiography and liver

biopsy. RESULTS: Of 262 consecutive patients with CD, 38 (15%) had

long-standing increased alkaline phosphatase (ALP) values (mean, 1065

U/l; range, 321-4165 U/l). Of these, 10 patients were classified as

having hepatic disease (4%), of which 9 had PSC and 1 had a non-

specific reactive hepatitis. Of nine patients with PSC (3.4%), three

were classified as having large-duct PSC; five, small-duct PSC; and

one, unclassified. In patients with large-bowel CD (n = 102) the

prevalence of PSC was 9%. Mean age at diagnosis of PSC was 35 years

(22-46 years), and the female to male ratio, 7:2. All PSC patients

had large-bowel involvement (P < 0.00015), and two of them developed

colonic carcinoma of the large bowel (P < 0.01). All cases of small-

duct PSC were stage 1, whereas large-duct PSC were stage 2-3. During

the observation period (mean, 5.4 years) no PSC patients died.

CONCLUSIONS: The results of our study indicate that PSC is the major

hepatic disease in patients with CD and long-standing abnormal liver

function tests and is approximately as prevalent as in ulcerative

colitis. Patients with PSC and CD may have a milder liver disease

than patients with PSC and ulcerative colitis, perhaps because large-

duct PSC is less common in patients with CD. Cholangiograms and liver

biopsies are both needed to evaluate the extent of the disease.

PMID: 9200295

Hungary:

World J Gastroenterol. 2003 Oct;9(10):2300-7.

Association of extraintestinal manifestations of inflammatory bowel

disease in a province of western Hungary with disease phenotype:

results of a 25-year follow-up study.

Lakatos L, Pandur T, G, Balogh Z, Kuronya P, Tollas A, Lakatos

PL

1st Department of Medicine, Csolnoky F. Province Hospital, Veszprem,

Hungary. laklaci@...

AIM: IBD is a systemic disease associated with a large number of

extraintestinal manifestations (EIMs). Our aim was to determine the

prevalence of EIMs in a large IBD cohort in Veszprem Province in a 25-

year follow-up study. METHODS: Eight hundred and seventy-three IBD

patients were enrolled (ulcerative colitis/UC/: 619, m/f: 317/302,

mean age at presentation: 38.3 years, average disease duration: 11.2

years; Crohn's disease/CD/: 254, m/f: 125/129, mean age at

presentation: 32.5 years, average disease duration: 9.2 years).

Intestinal, extraintestinal signs and laboratory tests were monitored

regularly. Any alteration suggesting an EIMs was investigated by a

specialist. RESULTS: A total of 21.3% of patients with IBD had EIM

(UC: 15.0%, CD: 36.6%). Age at presentation did not affect the

likelihood of EIM. Prevalence of EIMs was higher in women and in CD,

ocular complications and primary sclerosing cholangitis (PSC) were

more frequent in UC. In UC there was an increased tendency of EIM in

patients with a more extensive disease. Joint complications were more

frequent in CD (22.4% vs UC 10.2%, P<0.01). In UC positive family

history increased the risk of joint complications (OR:3.63). In CD

the frequency of type-1 peripheral arthritis was increased in

patients with penetrating disease (P=0.028). PSC was present in 1.6%

in UC and 0.8% in CD. Dermatological complications were present in

3.8% in UC and 10.2% in CD, the rate of ocular complications was

around 3% in both diseases. Rare complications were

glomerulonephritis, autoimmune hemolytic anaemia and celiac disease.

CONCLUSION: Prevalence of EIM in Hungarian IBD patients is in

concordance with data from Western countries. The high number of EIM

supports a role for complex follow-up in these patients. PMID:

14562397.

United Kingdom:

World J Gastroenterol. 2008 Jan 21;14(3):331-7.

Primary sclerosing cholangitis, autoimmune hepatitis and overlap

syndromes in inflammatory bowel disease.

Saich R, Chapman R

The Department of Gastroenterology, The Radcliffe Hospital,

Headley Way, Headington, Oxford, Oxfordshire, United Kingdom.

r.saich@...

Primary sclerosing cholangitis (PSC) is a chronic progressive

disorder of unknown aetiology characterised by chronic inflammation

and stricture formation of the biliary tree. Symptoms include itch

and lethargy and in advanced cases cholangitis and end-stage liver

disease, however increasing numbers of asymptomatic individuals are

being identified. The disease is rare in the general population but

is strongly associated with inflammatory bowel disease (IBD)

affecting up to 5% of patients with ulcerative colitis, with a

slightly lower prevalence (up to 3.6%) in Crohn's disease. The

strength of this association means that the vast majority (> 90%) of

patients with PSC also have IBD, although many may have only mild

gastro-intestinal symptoms. Usually IBD presents before PSC, although

vice-versa can occur and the onset of both conditions can be

separated in some cases by many years. Mean age of diagnosis of PSC

is in the fifth decade of life with a strong male predominance. Risk

is increased in those with a family history of PSC, suggesting a

genetic predisposition and the disease is almost exclusive to non-

smokers. The ulcerative colitis associated with PSC is

characteristically mild, runs a quiescent course, is associated with

rectal sparing, more severe right sided disease, backwash ileitis and

has a high risk of pouchitis post-colectomy. Most worrisome is the

high risk of colorectal malignancy which necessitates routine

colonoscopic surveillance. Cholangiocarcinoma is also a frequent

complication of PSC with a 10%-15% lifetime risk of developing this

condition. Treatment with high dose ursodeoxycholic acid offers some

chemoprotective effects against colorectal malignancy and may

decrease symptoms, biochemical and histological progression of liver

disease. Small duct PSC patients characteristically have normal

cholangiography, and liver biopsy is required for diagnosis, it

appears to have a more favourable prognosis. Autoimmune Hepatitis

(AIH) is also more prevalent in patients with IBD, with up to 16% of

patients with AIH also having ulcerative colitis. A small subgroup of

patients have a AIH-PSC overlap syndrome and the management of these

patients depends on liver histology, serum IgM levels,

autoantibodies, degree of biochemical cholestasis and cholangiography

as some of these patients may respond to immunosuppression. PMID:

18200656.

Best regards,

Dave

(father of (23); PSC 07/03; UC 08/03)

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