The pancreatic and extrapancreatic manifestations of IgG4-related disease

The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio >40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems. We present a pictorial essay demonstrating the spectrum of imaging findings for IgG4-related disease, including dacryosialadenitis, variety of renal lesions, tumefactive thickening of the extraocular muscles and orbital nerve, sclerosing cholangitis, and type I pancreatitis. Imaging plays an important role in diagnosis, screening for multiorgan involvement, and follow-up of the disease.