Abstract
IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.
Highlights
Sylvia Drazilova,1 Eduard Veseliny,1 Patricia Denisa Lenartova,2 Dagmar Drazilova,3 Jakub Gazda,1 Ivica Grgurevic,4 Martin Janicko,1 and Peter Jarcuska 1
Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. e diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram
Canadian Journal of Gastroenterology and Hepatology the bile duct wall; it usually presents with other organ manifestations, especially with autoimmune pancreatitis [5, 6]. e term IgG4-associated sclerosing cholangitis was replaced with the formal name “IgG4-related sclerosing cholangitis” at the 1st International Symposium on IgG4related disease [7]. is nomenclature aims to emphasize the similarities with primary sclerosing cholangitis (PSC) and the strong need to distinguish between the two diseases, as they have diametrically different treatment and prognoses
Summary
IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary manifestation of IgG4-related disease (IgG4-RD). In 2011, Kanno et al conducted the Nationwide Epidemiological Survey of Autoimmune Pancreatitis in Japan with the following results: the overall prevalence rate of AIP was 4.6 cases per 100,000 inhabitants and the annual incidence rate was 1.4 per 100,000 inhabitants [18] Based on this information and the fact that IgG4-SC is present in about 40% of patients with AIP, we can indirectly conclude that the incidence of IgG4-SC is approximately 0.5 new cases per 100,000 inhabitants and the prevalence is 1.8 cases per 100,000 inhabitants [19]. In 2020, Tanaka et al published the first epidemiological study evaluating 1,045 IgG4-SC patients from 532 centers in Japan and showed that the prevalence of IgG4-SC in 2018 was 2.18 (95% confidence interval, 2.13–2.23) per 100,000 inhabitants, which is comparable to other data [20]. If IgG4-SC occurs in women, the IgG4-SC without AIP is more common [13]
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