Clinical practice guidelines for IgG4‐related sclerosing cholangitis

Abstract

IgG4‐related sclerosing cholangitis (IgG4‐ SC ) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4‐related disease. Although clinical diagnostic criteria of IgG4‐ SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4‐ SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions ( CQ s) with clinical statements were developed regarding diagnosis (14 CQ s) and treatment (4 CQ s). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐ SC .

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     Bi Yan, Hart PA, Law R, Clain JE, Farnell MB, Gleeson FC, et al. Obstructive jaundice in autoimmune pancreatitis can be safely treated with corticosteroids alone without biliary stenting. Pancreatology 2016;16:391–6.

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     Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience. Gut. 2013;62:1607–15.

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     Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, et al. Rituximab therapy for refractory biliary strictures in immunoglobulin G4‐associated cholangitis. Clin Gastroenterol Hepatol. 2008;6:364–6.

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     Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4‐related systemic disease. Arthritis Rheumatol. 2010;62:1755–62.

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     Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, et al. Rituximab for IgG4‐related disease: a prospective, open‐label trial. Ann Rheum Dis. 2015;74:1171–7.

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Cited by papers we hold 1

• IgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review · 2022
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