Case of IgG4-related Disease with Crescentic Glomerulonephritis: An Unusual Presentation of the Disease

Abstract

IgG4-related disease (IgG4-RD) is a chronic systemic inflammatory 
disease, characterized by tissue infiltration of lymphocytes and 
IgG4-secreting plasma cells, presenting by fibrosis of different 
tissues, which is usually responsive only to oral steroids therapy. 
Kidneys are the most commonly involved organs, exhibiting renal 
insufficiency, tubulointerstitial nephritis, and glomerulonephritis. 
Here, we describe a patient with acute renal insufficiency who 
was presented with edema, weakness, anemia and multiple 
lymphadenopathies. Kidney and lymph node biopsy showed 
crescentic glomerulonephritis in kidneys and lymphoplasmacytic 
infiltration in lymph nodes. After a course of treatment with an 
intravenous pulse of corticosteroid and cyclophosphamide, the 
patient’s symptoms subsided, and kidney function improved.

DOI: 10.52547/ijkd.7788