IgG4-Related Sclerosing Cholangitis
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AUTOIMMUNE, CHOLESTATIC, AND BILIARY DISEASES (S GORDON AND CL BOWLUS, SECTION EDITORS)
IgG4-Related Sclerosing Cholangitis Akash Singh 1
&
Virendra Singh 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review IgG4-related sclerosing cholangitis (ISC) is a frequent occurrence. However, its diagnosis is difficult. This review summarizes the clinical features, pathogenesis, differential diagnosis, and management of ISC. Recent Findings A precise diagnosis of ISC is important. Diagnosis is based on the Japanese criteria which has recently been provided. The characteristic features of plasma cell infiltration and raised IgG4 levels along with classical histopathological and imaging findings help in diagnosis. Steroid therapy is very effective in ISC. Immunomodulatory drugs have also shown promising results. Summary Diagnostic approach of ISC mainly involves imaging modalities. Japanese diagnostic criteria is important in its diagnosis. It is also extremely important to differentiate IgG4 sclerosing cholangitis from various other cholangiopathies. Steroid therapy stays the treatment of choice. The role of other immunomodulators needs to be researched and reciprocated in clinical setting before it replaces steroid therapy. Keywords Autoimmune cholangiopathy . Primary sclerosing cholangitis . Biliary strictures
Introduction The recognition of IgG4-related disease as a systemic condition came into being after initial similarities between autoimmune pancreatitis (AIP) and IgG4 were found out. This prompted studying the pathologies in detail, as a result of which IgG4-related fibroinflammatory conditions in other organs outside pancreas were found. Mikulicz disease (head and neck), Küttner tumor (salivary gland), interstitial pneumonitis (lungs), interstitial nephritis (kidney), and Riedel thyroiditis (thyroid) were all found to have one thing in common, i.e., increased serum IgG4 concentrations, and pathologically, there was presence of IgG4-positive plasma cells [1]. IgG4related sclerosing cholangitis (ISC) also known as autoimmune cholangiopathy is a chronic inflammatory disease of the biliary system which is commonly seen as part of IgG4This article is part of the Topical Collection on Autoimmune, Cholestatic, and Biliary Diseases * Akash Singh [email protected] 1
Department of Hepatology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
related systemic disease [2••]. The diagnosis of ISC is based on well-established diagnostic criteria [3••]. However, sometimes, differentiating it from primary sclerosing cholangitis and cholangiocarcinoma becomes difficult [3••]. The correlation of ISC and autoimmune pancreatitis is of paramount importance as it has been observed that around 90% of cases also have autoimmune pancreatitis, in particular, type 1 autoimmune pancreatitis showing the classic lymphoplasmacytic infiltration [4•]. ISC is seen in up to 39% of patients with autoimmune pancreatitis [5]. The etiopathogenesis of ISC is still not
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