Atypical presentation of IgG4-related disease

Abstract

IgG4-related disease is a clinical entity which is characterized by tumefaction and fibrosis of affected organs, elevated serum IgG4 level, and tissue infiltration by IgG4-positive plasma cells. Most often, it affects the pancreas, main salivary and lacrimal glands, retroperitoneum. In this article an atypical manifestation of IgG4-related disease. The first manifestation of the disease was erroneously diagnosed as cutaneous systemic sclerosis. Pancreatic manifestation of the disease occurs during the second and the third relapse of the disease 11 and 13 years later correspondingly and were combined in the latter case with the coffee ground emesis, hepatic artheriitis, thrombosis of splenic artery and vein and diffuse peritonitis. Thus, the manifestation of IgG4-related disease may imitate some other clinical conditions, and therefore physicians must be aware of such a rare but multifaceted disorder in daily clinical practice.

Keywords:

IgG4-related disease, systemic sclerosis, Raynaud’s phenomenon, retroperitoneal fibrosis