Small-duct primary sclerosing cholangitis: Prevalence and natural history

Angulo, Paul; Maor-Kendler, Y.; Donlinger, Jessica; Lindor, Keith D.

doi:10.1016/s0016-5085(00)85742-x

Cited by 6 publications

(6 citation statements)

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“…10 The prevalence of IBD among the two groups was very similar in the present study (88% in small and 83% in large duct patients). However, the proportion of patients with Crohn's disease in small duct patients was much higher than that previously reported in large duct PSC.…”

Section: Discussionsupporting

confidence: 78%

“…These patients with a clinical diagnosis of PSC but without the typical intra-and/or extrahepatic cholangiographic changes have been identified as having small duct PSC. [7][8][9] Only a small number of patients with small duct PSC have so far been reported [10][11] and the natural history of patients fulfilling the criteria for small duct PSC has been largely unexplored. 11 It has been proposed that patients with small duct PSC would eventually develop large duct PSC.…”

mentioning

confidence: 99%

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Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

Bjõrnsson

Boberg²,

Cullen³

et al. 2002

Gut

190

123

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Section: Discussionsupporting

confidence: 78%

mentioning

confidence: 99%

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

Bjõrnsson

Boberg²,

Cullen³

et al. 2002

Gut

190

123

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“…89 The Mayo Clinic's criteria for the diagnosis of small-duct PSC mandates the presence of coexisting IBD. 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.…”

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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“…While the patients have laboratory and histological features similar to PSC, the cholangiogram is typically normal 80 . The Mayo Clinic's criteria for the diagnosis of small duct PSC mandate the presence of coexisting IBD 81 . In contrast, the presence of IBD was not a part of the criteria to classify small duct PSC used in Europe 82,83 .…”

Section: Small Duct Pscmentioning

confidence: 99%

Hepatobiliary disorders and complications of inflammatory bowel disease

Venkatesh

Navaneethan

Shen³

2011

J of Digest Diseases

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One of typical examples of liver-gut cross talk is the interaction and impact of inflammatory bowel disease (IBD) and hepatobiliary (HB) abnormalities on each other's disease course. There are several layers of association between IBD and HB diseases: (i) HB diseases and IBD share pathogenetic mechanisms; (ii) HB diseases parallel structural and pathophysiological changes seen with IBD; and (iii) hepatic toxicity is associated with medical therapy for IBD. Interdisciplinary approach, involving gastroenterologists, hepatologists and, in advanced cases, general, colorectal, and liver transplant surgeons, is advocated.

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Small-duct primary sclerosing cholangitis: Prevalence and natural history

Cited by 6 publications

References 0 publications

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

Hepatopancreatobiliary manifestations and complications associated with inflammatory bowel disease

Hepatobiliary disorders and complications of inflammatory bowel disease

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