Multifocal lesions with pancreatic atrophy in IgG4-related autoimmune pancreatitis: report of a case

Abstract

We herein report a case of IgG4-related autoimmune pancreatitis (AIP). A 72-year-old male with jaundice visited our hospital complaining of epigastralgia. A blood chemistry analysis revealed elevated serum levels of total bilirubin and DUPAN-II. Computed tomography (CT) revealed irregularly shaped pancreatic masses with a stricture of the main pancreatic duct (MPD) in the head and tail that were interposed by marked atrophy with MPD dilation in the body. F-18 fluorodeoxyglucose (FDG)-positron emission tomography/CT revealed abnormally intense FDG uptake only at the masses. During surgery, another small tumor was also found in the atrophied body; therefore, a total pancreatectomy was performed under the diagnosis of multiple pancreatic cancers. The histological analysis revealed fibrosis with dense and diffuse infiltrations of lymphocytes and IgG4-positive plasma cells. The pancreatic parenchyma of the body was firmly replaced by fibrosis. AIP can lead to the formation of multiple pancreatic lesions, and thus the correct diagnosis is occasionally difficult to establish in atypical cases.

Multifocal Mass Lesions in Autoimmune Pancreatitis

“…There are a few reports of AIP with multifocal mass lesions. Only 9 cases of AIP with multiple mass lesions have been reported, including our case (Table 1 ) [ 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. In these cases, the patient age ranged from 50 to 72 years (average: 61 years) and included 7 males and 2 females.…”

“…There have only been a few cases of AIP with multifocal mass lesions [ 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. We herein report a rare case of multifocal mass lesions in AIP and include pertinent bibliographical comments.…”

Exocrine Pancreatic Insufficiency Possibly Related to Atypical Chronic Graft-versus-Host Disease

“…The differential diagnosis for pancreatic atrophy includes chronic pancreatitis, autoimmune pancreatitis, pancreatectomy, immune checkpoint inhibitor therapy, type 2 diabetes mellitus, and pancreatic GVHD [4][5][6][7][8]. Chronic GVHD presenting with pancreatic atrophy is rare, and its natural history and pathophysiology are poorly defined.…”