Abstract: SA-PO244
Novel Approach to Treatment of Mixed Cryoglobulinemia with Kidney Involvement: Case Report
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
- Camargo, Marianne, Icahn School of Medicine at Mount Sinai, New York, New York, United States
- Chang, Tara I., Stanford University School of Medicine, Palo Alto, California, United States
- Isom, Robert T., Stanford University School of Medicine , Palo Alto, California, United States
- Iberri, David Joseph, Stanford Cancer Center, Stanford, California, United States
- O'Shaughnessy, Michelle M., Stanford University, Palo Alto, California, United States
Introduction
Mixed cryoglobulinemia (CG) is rarely associated with hepatitis B virus (HBV) infection. Monoclonal CG can result from an underlying lymphoproliferative disorder. We report a case of mixed CG with treated HBV and a low-grade lymphoproliferative disorder. Adverse reactions to standard therapies motivated a novel proteasome-inhibitor based approach
Case Description
A 67-year-old woman with a 5-year history of mixed CG and membranoproliferative glomerulonephritis experienced cerebrovascular and gastrointestinal ischemia with deteriorating kidney function, persistent hematuria, and proteinuria, while receiving maintenance tacrolimus and prednisone. Prior therapies included mycophenolate (persistent disease activity), rituximab (serum sickness), cyclophosphamide (leukopenia), and azathioprine (persistent disease activity). Labs included: +RhF, +HBV sAg with negative sAb (treated with entecavir with undetectable viral load), negative HCV, +IgM monoclonal gammopathy, and hypogammaglobulinemia. Before further immunosuppressive therapy, she received subcutaneous immunoglobulin for hypogammaglobulinemia. She developed palpable purpura, increasing creatinine (2.1 to 4.3 mg/dL), and increasing cryocrit, consistent with an immunoglobulin-triggered CG vasculitis flare. Given underlying monoclonal gammopathy, we initiated bortezomib and dexamethasone. Her creatinine initially declined to 2.8 mg/dL; however, she then developed severe diarrhea (attributed to bortezomib toxicity). Repeat kidney biopsy revealed 5 of 19 glomeruli with global sclerosis, 6 of 19 with cellular crescents, and large IgM Kappa positive subendothelial cryoglobulin deposits. She received 5 days of plasma exchange and intravenous corticosteroids followed by carfilzomib as an alternate proteasome inhibitor: response pending at time of abstract submission.
Discussion
Unlike prior cases of HBV-associated CG, our patient had persistent disease activity despite viremia clearance. Cryoglobulinemic vasculitis flares following intravenous or subcutaneous immunoglobulin have not been widely reported. A proteasome inhibitor-based approach might represent a novel treatment option when standard therapies fail.