Autoimmune pancreatitis and IgG4-related sclerosing cholangitis

Abstract

AIP and associated extrapancreatic lesions are considered to represent clinical manifestations of IgG4-related sclerosing disease.

Sequential Evaluation of Pancreato-Biliary Findings in a Case with IgG4-Associated Cholangiopathy and Autoimmune Pancreatitis during Corticosteroid Treatment

“…Although it was noticeably an atypical finding, mildly dilated proximal MPD in this case was thought to be related to IgG4-associated disease, since it disappeared completely under corticosteroid treatment. High serum levels of IgG4, the presence of type 1 autoimmune pancreatitis (diffuse type) and corticosteroid responsiveness easily helped us to exclude other possible diseases, such as primary sclerosing cholangitis, cholangiocarcinoma, follicular cholangitis, as the differential diagnosis of this case (6,7). Moreover, histological detection of IgG4 + plasma cells is usually unnecessary for cases like ours, showing typical radiological findings and high serum levels of IgG4.…”

Immunological diseases of the pancreatico-hepatobiliary system: update on etiopathogenesis and cross-sectional imaging findings

“…Gallbladder involvement is also frequent and manifests as diffuse thickening of the gallbladder wall [47]. Endoscopic ultrasonography shows wall thickening in intrahepatic or extrahepatic bile ducts with preservation of the luminal and outer hyperechoic zones [43]. Yang et al reported narrowing of the main portal vein, splenic vein, and superior mesenteric vein in up to 70% cases of AIP [49].…”

“…Relapses of IgG4-related sclerosing cholangitis are also common. In a series of 53 patients, relapse was seen in 53% of patients after corticosteroid withdrawal and 44% of patients after surgery [43].…”

“…AIP predominantly affects men in their 5th-6th decade. Obstructive jaundice is the most frequent complaint, seen in about 75% of affected patients [43]. Diabetes mellitus is seen in up to 50% of affected individuals [44].…”

Maladie fibrosclérosante à IgG4

“…Des Ac non spécifiques du pancréas peuvent être trouvés dans les PAI comme les Ac anti-anhydrase carbonique de type I, II, IV et les Ac anti-lactoferrine[56]. On peut également trouver des AAN, des facteurs rhumatoïdes[25], des anti-TPO, anti-thyroglobuline ainsi qu'une hypocomplémentémie. du dosage des IgG4 sériques pour le diagnostic et le suivi de la maladie fibrosclérosante à IgG4Après quatre semaines de corticothérapie, le taux d'IgG4 est plus bas qu'avant traitement, suggérant qu'il s'agit d'un marqueur utile pour en évaluer l'efficacité, sous réserve d'un taux d'IgG4 sérique élevé initialement[58].…”