IgG4-Related Kidney Disease Associated With End-Stage Kidney Disease, Renal Pseudotumor, and Renal Vein Thrombosis

Cureus. 2022 Mar 4;14(3):e22837. doi: 10.7759/cureus.22837. eCollection 2022 Mar.


IgG4 related disease (IgG4-RD) is a systemic autoimmune disease characterized by tissue invasion with IgG4-producing plasma cells, resulting in tissue dysfunction. IgG4-RD can affect the kidney in various forms, including renal mass, tubulointerstitial disease, and glomerulonephritis. IgG4-RD can mimic other autoimmune diseases and neoplasms, and as such, maintaining a high index of suspicion is the key to timely diagnosis and treatment. In this paper, we present a case of IgG4-RD that presented with pseudotumor and severe renal dysfunction that progressed to end-stage kidney disease (ESKD), associated with a rare finding of renal vein thrombosis (RVT).

Keywords: end stage kidney disease (eskd); igg 4 disease; igg4 related pseudotumor; plasma cell tumor; renal vein thrombosis.

Publication types

  • Case Reports