Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

Broomé, Ulrika; Glaumann, Hans; Lindstöm, Eva; Lööf, Lars; Almér, Sven; Prytz, Hanne; Sandberg-Gertzén, Hanna; Lindgren, Stefan; Fork, Frans-Thomas; Järnerot, Gunnar; Olsson, Rolf

doi:10.1016/s0168-8278(02)00036-3

Cited by 120 publications

(71 citation statements)

References 22 publications

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“…Small duct PSC appears to have a more favourable prognosis with a median survival 29.5 years compared to 17 years for classical PSC [78][79][80][81] , although occasionally patients with small duct PSC progress to large duct disease. immune mediated liver disease including autoimmune hepatitis (AIH).…”

Section: Small Duct Pscmentioning

confidence: 97%

Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease

Saich¹,

Chapman²

2008

WJG

117

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(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 immunosupression. INTRODUCTIONThe first association between colonic ulceration and liver disease was made in 1874 by Thomas CH, who described a young man who died of a 'much enlarged, fatty liver in the presence of ulceration of the colon' [1] . The association was confirmed by James Lister in 1899, who reported a patient with ulcerative colitis and secondary diffuse hepatitis [2] . Over the next 100 years it has become well established that there is a close relationship between inflammatory bowel disease and various hepatobiliary disorders. These disorders are listed in Table 1. PRIMARY SCLEROSING CHOLANGITISFirst described by Smith and Loe in 1965 [3] Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by inflammation, fibrosis and stricture formation in medium and large sized ducts in the biliary tree [4,5] . Common symptoms include itch and lethargy although many patients (up to 45%) are asymptomatic, even with advanced disease [5] . The disease is strongly associated with Inflammatory Bowel Disease and may be detected as an incidental finding of a raised serum alkaline phosphatase. www.wjgnet.comRe b e c c a S a i c h , Ro g e r C h a p m a n , AbstractPrimary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown aetiology characterised by chronic inflammation and stricture formation of the biliary tree.

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Section: Small Duct Pscmentioning

confidence: 97%

Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease

Saich¹,

Chapman²

2008

WJG

117

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“…90 In contrast, the presence of IBD was not a part of the criteria to classify small-duct PSC used in Europe. 91,92 Initial studies with limited follow-up suggested that patients with small-duct PSC have a better prognosis than those with its large-duct counterpart. [90][91][92] Subsequently, a large multicenter study encompassing patients of the previous studies of small-duct PSC with a longer follow-up showed that approximately 80% of patients with smallduct PSC had IBD, either at the time of diagnosis of liver disease or at follow-up.…”

Section: Managementmentioning

confidence: 99%

“…91,92 Initial studies with limited follow-up suggested that patients with small-duct PSC have a better prognosis than those with its large-duct counterpart. [90][91][92] Subsequently, a large multicenter study encompassing patients of the previous studies of small-duct PSC with a longer follow-up showed that approximately 80% of patients with smallduct PSC had IBD, either at the time of diagnosis of liver disease or at follow-up. 93 Of these patients with concurrent IBD, 78% had UC, 21% had Crohn's colitis, and 1 patient had collagenous colitis.…”

Section: Managementmentioning

confidence: 99%

Hepatopancreatobiliary manifestations and complications associated with inflammatory bowel disease

Navaneethan¹,

Shen²

2010

Inflammatory Bowel Diseases

179

127

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Diseases involving the hepatopancreatobiliary (HPB) system are frequently encountered in patients with inflammatory bowel disease (IBD). Hepatobiliary manifestations constitute some of the most common extraintestinal manifestations of IBD. They appear to occur with similar frequency in patients with Crohn's disease or ulcerative colitis. HPB manifestations may occur in following settings: 1) disease possibly associated with a shared pathogenetic mechanism with IBD including primary sclerosing cholangitis (PSC), small-duct PSC/pericholangitis and PSC/autoimmune hepatitis overlap, acute and chronic pancreatitis related to IBD; 2) diseases which parallel structural and physiological changes seen with IBD, including cholelithiasis, portal vein thrombosis, and hepatic abscess; and 3) diseases related to adverse effects associated with treatment of IBD, including drug-induced hepatitis, pancreatitis (purine-based agents), or liver cirrhosis (methotrexate), and reactivation of hepatitis B, and biologic agent-associated hepatosplenic lymphoma. Less common HPB manifestations that have been described in association with IBD include autoimmune pancreatitis (AIP), IgG4-associated cholangitis (IAC), primary biliary cirrhosis (PBC), fatty liver, granulomatous hepatitis, and amyloidosis. PSC is the most significant hepatobiliary manifestation associated with IBD and poses substantial challenges in management requiring a multidisciplinary approach. The natural disease course of PSC may progress to cirrhosis and ultimately require liver transplantation in spite of total proctocolectomy with ileal-pouch anal anastomosis. The association between AIP, IAC, and elevated serum IgG4 in patients with PSC is intriguing. The recently reported association between IAC and IBD may open the door to investigate these complex disorders. Further studies are warranted to help understand the pathogenesis of HPB manifestations associated with IBD, which would help clinicians better manage these patients. An interdisciplinary approach, involving gastroenterologists, hepatologists, and, in advanced cases, general, colorectal, and transplant surgeons is advocated.

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“…8,9,24 This pattern is probably not due to lead time bias, in that only 12% to 16% of patients with small duct PSC progress to large duct disease (in 5-to 6-year follow up). Furthermore, some patients develop cirrhosis without obvious large duct involvement.…”

mentioning

confidence: 95%

Primary sclerosing cholangitis: Summary of a workshop

et al. 2006

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Primary sclerosing cholangitis (PSC) is a rare but important liver disease that leads to cirrhosis and need for liver transplantation in a high proportion of cases. The disease occurs in approximately 1 per 100,000 population per year, usually presents in adulthood, and affects men more often than women. Typical serum biochemical results, autoantibodies and liver biopsy are suggestive but not diagnostic of PSC, the diagnosis requiring cholangiographic demonstration of stricturing and dilatation of the intra-and/or extra-hepatic bile ducts. The natural history of PSC is variable, the average survival being 12 to 17 years. The cause of PSC is still unknown. Although considered an autoimmune disease, PSC has several atypical features and a strong genetic component. The therapy of PSC is unsatisfactory. Standard doses of ursodeoxycholic acid (UDCA) lead to improvements in biochemical abnormalities but not in histology, cholangiographic appearance or survival. Several innovative therapies have been tried in PSC, but with scant evidence of benefit. For patients with high grade strictures, endoscopic dilatation is beneficial. Liver transplantation is successful for end-stage liver disease due to PSC and improves survival. PSC may recur after transplantation but is rarely progressive. The most dreaded complication of PSC is cholangiocarcinoma. Diagnosis of this highly malignant tumor is difficult, and there are no biomarkers for its early detection. Liver transplantation for cholangiocarcinoma has an exceedingly poor outcome, although transplantation with neoadjuvant chemoirradiation holds promise in selected patients. Thus, significant opportunities remain for basic and clinical research into the cause, natural history, and therapy of PSC. 44:746-764.)

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Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

Cited by 120 publications

References 22 publications

Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease

Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease

Hepatopancreatobiliary manifestations and complications associated with inflammatory bowel disease

Primary sclerosing cholangitis: Summary of a workshop

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