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Abstract: FR-PO719

Hepatitis C Negative Type II Cryoglobulinemic Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Lawson, Cameron T., East Carolina University, Greenville, North Carolina, United States
  • Mohsen, Adham, East Carolina University, Greenville, North Carolina, United States
  • Myers, Iskra, East Carolina University, Greenville, North Carolina, United States
Introduction


Mixed cryoglobulinemia typically follows a chronic, smoldering course. Here we present a case of rapidly progressive mixed cryoglobulinemic glomerulonephritis (GN) in a hepatitis C negative patient.

Case Description

A 67-year-old female with a history of chronic kidney disease stage III and ophthalmologic Sjogren’s syndrome presented to the emergency department for labile blood pressures. Labs were notable for serum creatinine 1.49 mg/dL, positive anti SS-A, anti-B. C3 28 (low), C4 < 3, and negative hepatitis panel. Urine protein: creatinine ratio was 2.2 g/day. Additional labs showed cryoglobulin level < 10% and was identified as Type II cryoglobulinemia monoclonal IgM kappa and polyclonal IgG. A kidney biopsy showed diffuse proliferative GN consistent with cryoglobulinemic GN. The patient’s condition continued to decline with worsening creatinine and raising concern for rapidly progressive GN secondary to cryoglobulinemic GN. Patient was initiated on high-dose steroids and plasmapheresis (PLEX). Subsequently, she received Rituximab. Despite these aggressive measures, oliguria and uremia persisted requiring dialysis.

Discussion

Mixed cryoglobulinemic GN is a rare cause of rapidly progressive GN that is commonly associated with hepatitis C viral infections. Sjogren disease is responsible for <10% of HCV-negative mixed cryoglobulinemias. Prompt treatment with steroids, PLEX, cyclophosphamide, or rituximab is essential due to the significant morbidity and mortality associated with this condition.