IgG4 related interstitial nephritis: A case report and review of literature.

IgG4 interstitial nephritis is a recently described entity. A middle-aged gentleman with bilateral parotid enlargement, hepatosplenomegaly and generalized lymphadenopathy was referred to us for evaluation of renal failure. He had trace proteinuria and large kidneys. Kidney biopsy revealed interstitial nephritis with characteristic storiform fibrosis. Immunohistochemistry demonstrated intense staining for IgG4-secreting plasma cells in the interstitium.