IgG4-related disease; a newly recognized entity with expanding spectrum 

Abstract

IgG4-related disease (IgG4RD) represents a newly recognized group of diseases affecting a wide spectrum of organs and systems of the human body. The disease has certain characteristic gross, morphologic, immunohistochemical and serological features. Its accurate diagnosis is important for optimal treatment and prognostication. There is a strong need for clinicopathological correlation to arrive at the correct diagnosis of this condition. Strict criteria must be used for identifying unusual cases of the disease in unusual locations. 

Keywords: IgG4-related disease, Plasma cells, Renal biopsy, Tubulointerstitial nephritis