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

Authors

  • Azin Ghaffari Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran Author
  • Mitra Mehrazma Oncopathology Research Center, Iran University of Medical Sciences, Tehran, Iran Author
  • Shahrzad Ossareh Nephrology Section, Department of Medicine, Hasheminejad Kidney Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran Author

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

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Published

2024-04-23

Issue

Section

CASE REPORT | Kidney Diseases

How to Cite

Case of IgG4-related Disease with Crescentic Glomerulonephritis: An Unusual Presentation of the Disease. (2024). Iranian Journal of Kidney Diseases, 18(2), 133-135. https://ijkd.org/index.php/ijkd/article/view/7788

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