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Abstract: TH-PO0428

Cryoglobulinemic Glomerulonephritis from Unusual Plasma Cell Differentiation in the Setting of Sjogren Syndrome

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

  • Yelamanchi, Aditya, University of California Davis, Davis, California, United States
  • Llamas, Marielle, University of California Davis, Davis, California, United States
  • Gupta, Rajib K., University of California Davis, Davis, California, United States
  • Ananthakrishnan, Shubha, University of California Davis, Davis, California, United States
Introduction

Cryoglobulinemic glomerulonephritis (Cryo GN) is classically a membranoproliferative pattern of injury usually in the setting of infections, autoimmune or lymphoproliferative disorders. We describe a case of cryo GN from an unusual plasmacytic differentiation of B cell neoplasm in the setting of Sjogren’s syndrome.

Case Description

66 year old female of Hmong descent with type II diabetes, latent tuberculosis s/p treatment, hypertension, Sjogren's syndrome presenting with shortness of breath, nonpruritic petechial rash and swelling of both feet. Exam reveals diffuse lymphadenopathy as well. Creatinine 1.96 mg/dL at baseline. Nephrology was consulted for AKI with peak creatinine up to 3 mg/dL and active urine sediment (UA with 40+ RBC, 5-20 WBC). Positive serologies included ANA, anti-SSA Ab IgG, RF 528.6 IU/mL (reference <12.5). C3/C4 levels were low (31 and <5 respectively). Cryoglobulin testing was negative on three separate occasions. Negative serologies included hepatitis B and C antibodies, HIV, dsDNA, MPO, PR3 and anti-GBM antibody. Serum IFE showed 2 adjacent bands characterized as IgM kappa. Monoclonal B cell clone was not detected in flow cytometry suggesting against lymphoma. Bone marrow biopsy was unrevealing. Lymph node biopsy notable for plasma cell neoplasm with positive CD138 and MUM-1. She was treated with CyBorD targeting plasma cells with good renal recovery. Creatinine down to 1.2 mg/dL at discharge.

Discussion

Sjogren’s syndrome is an autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands leading to progressive destruction of glands and production of autoantibodies. The occurrence of B-cell non-Hodgkin’s lymphoma represents a known major complication of SS. In this setting, the treatment for cryo GN is directed at treating the B cell clone. However, our case represents a unique and extreme plasma cell differentiation in the lymph nodes that responded well to a plasma cell-based therapy with resolution of the cryoglobulinemia.