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Abstract: SA-PO246

IgG4-Related Disease with an Unusual Presentation

Session Information

Category: Onconephrology

  • 1700 Onconephrology


  • Paredes, William Mauricio, SBH Health System, Bronx, New York, United States
  • Flores Chang, Marjorie Mailing, SBH Health System, Bronx, New York, United States
  • Flores Chang, Bessy Suyin, SBH Health System, Bronx, New York, United States

Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated disease that can present with tumefactive lesions, fibrosis, and polyclonal IgG4-positive plasma cell infiltrate in nearly any anatomic site. It can manifest as lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia, and often mimics other hematologic conditions. However, the lack of a relationship between IgG4-related kidney disease and monoclonal gammopathy of renal significance (MGRS) warrants further investigation.

Case Description

56-year-old male with spastic paraparesis secondary to cervical myelopathy due to severe cervical stenosis (C4-C5) with a history of chronic longstanding nasal congestion and obstruction. Started to have hyporexia, anosmia, weight loss, fatigue, rash and xerostomia for approximately 4 months. Accompanied by renal failure which was first detected 2 months prior to the onset of his symptoms. Urinalysis was negative and on microscopy had a bland appearance, ultrasound of the kidneys showed a mild bilateral increase in size.
Further workup was relevant for Serum Free Kappa light Chains ratio of 9.7. Serum immunofixation was positive for IgG monoclonal protein with kappa light chain. Due to concern for myelodysplastic disorder, the patient underwent bone marrow biopsy which showed plasma cells that comprised 8-10 % of the nucleated hematopoietic bone marrow well-differentiated cells. There was no detectable clonal plasma cell population on flow cytometry prompting a diagnosis of MGUS for which he underwent kidney biopsy that revealed acute on chronic interstitial nephritis with many IgG4-positive plasma cells, suspicious for IgG4-RD. Currently receiving treatment with Rituximab and renal function has remained stable.


This patient presented with xerostomia, constitutional symptoms, and development of renal failure, workup was diagnostic of MGUS. However, a kidney biopsy was performed and showed acute on chronic interstitial nephritis with IgG4-positive plasma cells, which leads to a diagnosis of IgG4-RD. An increased free light chain concentration has been reported in a variety of inflammatory and autoimmune diseases and reflects the polyclonal B lymphocyte activation in these pathologies. This presentation is fairly uncommon and the intention of this abstract is to make physicians aware of this for the future and warrant further investigation.