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Publications

Case Reports Journal Article Epublish 1 citation

PubMed: 39224761 DOI: 10.1177/2050313X241279696

Phospholipase A2 receptor-negative membranous nephropathy presenting as a rare renal manifestation of IgG4-related disease.

SAGE Open Med Case Rep 12:2050313X241279696

Authors: Srikar Sama, Alexander Weickhardt, Preethi Subramanian, Pooja Reddy

Affiliations

  • Department of Internal Medicine, University of California San Francisco Fresno, Fresno, CA, USA.
  • Department of Anatomic and Clinical Pathology, University of California San Francisco, San Francisco, CA, USA.
  • Department of Nephrology, Veteran Affairs Central California Health System, Fresno, CA, USA.
  • Department of Rheumatology, Veteran Affairs Central California Health System, Fresno, CA, USA.

Abstract

IgG4-related disease is a fibroinflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells affecting multiple organs. Though the most common renal manifestation of IgG4-related disease is tubulointerstitial nephritis, it can rarely present as secondary membranous nephropathy. We present a case of a 75-year-old male with phospholipase A2 receptor-negative membranous nephropathy as an atypical manifestation of IgG4-related disease. The patient presented with nephrotic syndrome and was found to have elevated serum IgG4 levels and IgG4-positive plasma cells in the kidney biopsy. He was successfully treated with corticosteroids and rituximab, resulting in significant improvement in proteinuria and normalization of IgG4 levels. This case highlights the importance of considering IgG4-related disease in patients with phospholipase A2 receptor-negative membranous nephropathy, especially in those with a history of other organ involvement. Early recognition and treatment of IgG4-related disease are crucial to prevent progressive kidney damage and improve patient outcomes.

MeSH

Glomerulonephritis, Membranous, Cells, Plasma Cells, Immunoglobulin G4-Related Disease, Receptors, Phospholipase A2, Kidney, Rituximab, Phospholipases A2, Phospholipases, Plasma