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Hepatitis C Associated Systemic Cryoglobulinemia: Successful Treatment With Plasma Exchange
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Reported by Jules Levin
AASLD, Nov 2-6, 2007, Boston, MA
J. C. Hofmann1; R. G. Gish2
1. Department of Medicine, Division of Immunotherapy, California Pacific Medical Center, San Francisco, CA, USA.
2. Department of Medicine, Division of Hepatology and Complex GI, California Pacific Medical Center, San Francisco, CA, USA.
ABSTRACT
Introduction: Acute treatment of mixed cryoglobulinemia associated with hepatitis C is indicated for systemic disease characterized by renal dysfunction, cutaneous vasculitis, or peripheral neuropathy.
Methods: We reviewed the medical records of 21 patients (pts) who were diagnosed with moderate to severe hepatitis C associated cryoglobulinemia from January, 2005 to May, 2007 and referred for plasmapheresis treatment. The mean age of pts was 54 years (46-64 years old); 11 pts (52%) were male. 18 pts (86%) had HCV genotype 1A or 1B. The median HCV RNA was 733,900 IU/ml (73,620-9,370,000 IU/ml). All pts had positive cryoglobulin studies; median cryocrit was 1.7% (0.3-5.0%). 18 pts (86%) had significant elevation of rheumatoid factor (RF). 79-90% of pts had low complement levels.
Clinical Presentation: 17 pts (81%) presented with renal disease. 11 pts (52%) had chronic renal insufficiency and 6 pts (29%) presented with acute renal failure requiring hemodialysis (HD). Of 17 pts with renal disease, thirteen (76%) had a renal biopsy; 12 pts (92%) had MPGN. 17 pts (81%) had elevated 24-hour urine protein; 13 of these pts (76%) had nephrotic range proteinuria. 13 pts (62%) presented with active vasculitic skin lesions. 7 pts (33%) had peripheral neuropathy.
Treatment: 19 pts (90%) received a course of inpatient plasma exchange (PE) every other day; mean number of PE treatments (txs) was 8.4 (5-12 txs). 2 pts (9.5%) received outpatient PE. After completion of inpatient PE txs, 8 pts (38%) received low dose cyclophosphamide to prevent rebound of immune complex production. 5 pts (24%) underwent weekly rituximab (4-6 doses). 15 pts (71%) started weekly pegylated alpha interferon after completion of PE txs; 12 pts (57%) started daily ribavirin.
Results: 20 pts (95%) experienced clinical improvement. Of 13 pts with nephrotic range proteinuria, 9 pts (69%) had significant decline in urinary protein. 3 of 6 pts (50%) on HD were able to stop dialysis. 17 pts (94%) had a marked decrease in RF with twelve pts (67%) normalizing their RF levels. 12 pts (92%) demonstrated significant improvement in vasculitic skin lesions. 6 pts (86%) experienced slight improvement in symptoms of peripheral neuropathy. 3 pts (14%) with refractory disease required several inpatient courses of PE. 6 pts (29%) received weekly maintenance PE txs.
Conclusion: Plasma exchange is an effective therapy for hepatitis C associated cryoglobulinemia, especially in acute treatment of progressive renal disease and cutaneous vasculitis. Some patients may require maintenance plasmapheresis treatment. Successful long-term response to antiviral therapy is essential in controlling this disease.
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