Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroid therapy. This case report is based on a 24-year-old man with IgG4-related type 1 autoimmune pancreatitis (AIP) who also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. The main complaints were dry cough, nasal congestion with sneezing, sore throat, and fever. Necessary investigations were performed and based on the International Consensus Diagnostic Criteria, the diagnosis of AIP type 1 was confirmed, which is a pancreatic manifestation of IgG4-RD. He was subsequently treated with prednisolone and azathioprine and is showing a good response to the treatment.