A Rare Case of Hepatitis C-Associated Cryoglobulinemic Duodenal Vasculitis

Abstract

Introduction: Intestinal involvement of cryoglobulinemia is an uncommon manifestation of severe vasculitis. We describe the case of a woman who developed acute renal insufficiency and progressive gastrointestinal symptoms after initiating hepatitis C (HCV) therapy. A 65-year-old, non-cirrhotic woman with HCV, genotype 2 (prior null responder to interferon), with known cryoglobulinemia maintained on mycophenolate mofetil (MMF), was started on sofosbuvir and ribavirin. HCV RNA viral load decreased from 5,453,939 IU/mL to <15 IU/mL after 2 weeks and was undetectable after 4 weeks. Soon after initiation, she developed severe anemia managed by ribavirin dose reduction and MMF discontinuation. She subsequently developed 2 weeks of abdominal pain, vomiting, and diarrhea, out of proportion to diffuse abdominal tenderness on examination. Ribavirin was discontinued upon admission, followed by sofosbuvir, yet symptoms persisted. An upper endoscopy revealed diffuse duodenal mucosal sloughing with active oozing and underlying dusky pigmented lesions, suspicious for vasculitis. A recurrence of cryoglobulinemia was suspected, in the setting of low complement levels, elevated rheumatoid factor, and absence of anti-nuclear and anti-neutrophil cytoplasmic antibodies. She developed acute kidney injury with a peak creatinine of 2.77 mg/dL, from a baseline of 1.1 mg/dL. Urine electrolytes, urinalysis with proteinuria, and an unremarkable renal ultrasound suggested an intrinsic cause. A confirmatory renal biopsy revealed membranous proliferative glomerulonephritis consistent with HCV-associated cryoglobulinemia. After receiving high-dose glucocorticoids, the first dose of rituximab, and plasmapheresis, her symptoms rapidly improved, as did renal function. Few studies have identified cases of cryoglobulinemia in the setting of successful HCV treatment; however, symptoms are generally mild and cutaneous. Intestinal vasculitis, a rare manifestation of severe cryoglobulinemia often complicated by perforation, requires prompt immunosuppression and/or plasmapheresis. Premature discontinuation of MMF during HCV treatment was likely the cause. Our case adds to the existing small body of published literature on intestinal cryoglobulinemic vasculitis and highlights the complex, viral mediated, immunologic mechanism and its management.Figure 1