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Minerva Gastroenterologica e Dietologica 2020 Dec 03

DOI: 10.23736/S1121-421X.20.02794-4


language: English

IgG4-related autoimmune liver disease

Gabriele CAPURSO 1, 2, 3, Federica PEDICA 1, 4, Diego PALUMBO 1, 5, Emanuel DELLA TORRE 1, 3, 6

1 Università Vita-Salute San Raffaele,IRCCS San Raffaele Scientific Institute, Milan, Italy; 2 Pancreato-Biliary Endoscopy and Endosonography Division, IRCCS San Raffaele Scientific Institute, Milan, Italy; 3 Division of Pancreatic Surgery, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy; 4 Pathology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy; 5 Clinical and Experimental Radiology Unit, Experimental Imaging Center, IRCCS San Raffaele Scientific Institute, Milan, Italy; 6 Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy


The term IgG4-related autoimmune liver disease (AILD) refers to hepato-biliary manifestations of IgG4-related disease (IgG4-RD) including IgG4-related sclerosing cholangitis and IgG4-related pseudotumors. The association of some forms of autoimmune hepatitis to IgG4-RD remains controversial. Although autoimmune phenomena have not been clearly observed in IgG4-AILD, perturbation of the adaptive immune system and activation of the humoral response represent established pathophysiological hallmarks and potential therapeutic targets. Clinical manifestations of IgG4-AILD are virtually indistinguishable from bile duct cancer or primary sclerosing cholangitis, and are due to mass forming lesions and thickening of the biliary tract that progressively lead to biliary ducts obstruction. There are no current reliable biomarkers for IgG4-AILD and diagnosis should rely on the integration of clinical, serological, radiological, and histological findings. In analogy to most IgG4-RD manifestations, and in contrast to its major mimickers, IgG4-AILD promptly responds to glucocorticoids but frequently relapses, thus requiring long-term maintenance therapy to avoid progressive fibrosclerotic disease and liver cirrhosis. Accumulating evidence on the efficacy of B-cell depletion therapy in patients with systemic IgG4-RD is gradually changing the treatment paradigm of IgG4-AILD and biologics will be increasingly used also for gastroenterological manifestations of IgG4-RD to spare glucocorticoids and traditional immunosuppressive agents. Looking ahead, identification of reliable biomarkers and of miniinvasive strategies to obtain informative biopsies from the biliary tree represent unavoidable priorities to optimize diagnosis and management of IgG4-AILD.

KEY WORDS: IgG4-related disease; IgG4; Autoimmune pancreatitis; Cholangitis; Pseudotumor; Liver; Pancreas; Hepatitis

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