A Pilot Study of Etanercept in the Treatment of Primary Sclerosing Cholangitis

Epstein, Mark P.; Kaplan, Marshall M.

doi:10.1023/b:ddas.0000011827.87103.2e

Cited by 76 publications

(36 citation statements)

References 15 publications

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“…The agents found to have little effect include corticosteroids, budesonide, 140 tacrolimus, 141 methotrexate, 116,142,143 mycophenolate mofetil, 144 pentoxyfylline, 145 silymarin, 146 and etanercept. 147 Trials of antibiotics such as metronidazole 148 and minocycline have been promising but inconclusive. A small study of docosahexaenoic acid which improves CFTR function is currently underway.…”

Section: Dr Marshall Kaplan (Tufts-new England Medicalmentioning

confidence: 99%

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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Section: Dr Marshall Kaplan (Tufts-new England Medicalmentioning

confidence: 99%

Primary sclerosing cholangitis: Summary of a workshop

et al. 2006

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“…Treatments which have been tried in an attempt to prevent progression of the liver disease to cirrhosis and to decrease the risk of complications especially malignancy include tacrolimus [50] , methotrexate [51] , corticoteroids [52] , D-penillamine [53] , etanercept [54] , cyclosporin [55] , azathioprine, 6-mercaptopurine [56] and pentoxifylline [57] . None of these agents have demonstrated any consistent benefit.…”

Section: Psc Treatmentmentioning

confidence: 99%

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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“…This drug failed to show a significant efficacy in this pilot study, but was helpful in treating pruritus [91]. …”

Section: Treatmentmentioning

confidence: 99%

Extraintestinal Manifestations of Crohn’s Disease

et al. 2007

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In each case of extraintestinal manifestations of Crohn’s disease, active disease, if present, should be treated to induce remission, which may positively influence the course of most concomitant extraintestinal manifestations. For some extraintestinal manifestations, however, a specific treatment should be introduced. This latter part of disease management will be discussed in this chapter, in particular for pyoderma gangrenosum, uveitis, spondylarthropathy – axial arthropathy – and primary sclerosing cholangitis, which have also been described in quiescent Crohn’s disease. Few new drugs for the treatment of extraintestinal manifestations of Crohn’s disease have been developed in the past and only the role of infliximab has increased in Crohn’s disease-related extraintestinal manifestations. Drugs specifically aimed at this treatment, stemming from a few randomized controlled studies or case series, are sulfasalazine, 5-ASA, corticosteroids, azathioprine or 6-mercaptopurine, methotrexate, infliximab, adalimumab, etanercept and cyclosporine or tacrolimus. Unfortunately, because of the paucity of data in this field, the best evidence presented and discussed in this article for the treatment of these extraintestinal manifestations is extrapolated from patients that for the most part did not suffer from Crohn’s disease.

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A Pilot Study of Etanercept in the Treatment of Primary Sclerosing Cholangitis

Cited by 76 publications

References 15 publications

Primary sclerosing cholangitis: Summary of a workshop

Primary sclerosing cholangitis: Summary of a workshop

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

Extraintestinal Manifestations of Crohn’s Disease

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