Purpose: Hepatitis C viral [HCV] infection is a chronic multisystem disorder that may have an indolent course initially. Peripheral neuropathy associated with cryoglobulinemia and a systemic vasculitis is a well-described complication of HCV infection. But this neuropathy is not known to have a late-onset acute fulminant phase. This acute fulminant phase is characterized by quadriparesis associated with pulmonary and/or renal insufficiency, and it may occur despite adequate treatment for HCV infection. The purpose of this study is to report that patients treated for chronic HCV infection may manifest a secondary progressive acute fulminant neuropathy associated with respiratory and/or renal insufficiency that is responsive to cyclophosphamide.
Methods: Case series retrospective data analysis.
Results: Three patients with biopsy-proven HCV associated vasculitic neuropathy manifested a secondary progressive acute fulminant course resulting in quadriparesis within 5 years of the initial diagnosis. Complete remission was achieved with cyclophosphamide therapy such that all patients became ambulatory.
Conclusions: HCV-associated vasculitic neuropathy may manifest a secondary phase, which is acute, fulminant and progressive that is superimposed on an otherwise slowly progressive disorder. Cyclophosphamide therapy may abort progression and induce remission of this acute fulminant phase.