Abstract: SA-PO242
Persistent HCV-Related Cryoglobulinemic Glomerulonephritis Following Sustained Virologic Remission and Rituximab Monotherapy
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Woodell, Tyler, Oregon Health & Science University, Portland, Oregon, United States
- Ribakare, Divine, Oregon Health & Science University, Portland, Oregon, United States
- Avasare, Rupali S., Oregon Health & Science University, Portland, Oregon, United States
- Andeen, Nicole K., Oregon Health & Science University, Portland, Oregon, United States
Introduction
Hepatitis C virus (HCV)-related cryoglobulinemia is treated with direct-acting antiviral therapy and immunosuppression if severe end-organ damage is present. We present a patient with persistent HCV-related cryoglobulinemic glomerulonephritis (GN) years after sustained virologic remission (SVR) refractory to rituximab monotherapy. With the help of our oncology colleagues we are targeting therapy to a low grade lymphoproliferative disorder producing cryoglobulins.
Case Description
A 62 year-old Caucasian man presented with palpable purpura on his lower extremities, nephrotic syndrome, and acute kidney injury in 2010. Serologic workup was notable for HCV (peak viral load 1.3 million iU/mL), depressed C4 level, markedly elevated rheumatoid factor (>650 iU/mL, nl 0 – 14) and type II cryoglobulinemia (monoclonal IgM kappa and polyclonal IgG); SPEP, UPEP, and serum free light chains were unremarkable. Kidney biopsy showed membranoproliferative GN with IgM kappa deposits, suggestive of cryoglobulinemic GN. A bone marrow biopsy did not show a clonal cell population. He achieved SVR at three months following treatment with simeprevir and sofosbuvir, but his nephrotic range proteinuria and purpura persisted. In 2015, the patient was treated with two courses of rituximab, however he continued to have nephrotic range proteinuria and cryoglobulinemia. A kidney biopsy was repeated and showed membranoproliferative GN with hyaline thrombi as well as subendothelial and mesangial deposits without a clear substructure, and a low-grade CD-20+ B cell lymphoma. He is currently being treated with rituximab and bendamustine for low grade B cell lymphoma. At last follow-up proteinuria improved from 8.1 to 4.2 grams/day; eGFR declined from 30 to 18ml/min/1.73m2.
Discussion
HCV-related cryoglobulinemic GN following SVR is increasingly recognized. This case of cryoglobulinemia highlights the intersection between HCV (typically types II-III) and lymphoproliferative disorders (typically type I), and draws attention to the need for appropriately targeted therapies. Given the growing use of direct-acting antiviral agents for HCV, novel therapeutic strategies are needed to effectively treat refractory cases of HCV-related cryoglobulinemic GN.