Hepatitis C virus acute quadriparetic vasculitic neuropathy responsive to cyclophosphamide.
OBJECTIVE
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.
Souayah
N
N
New Jersey Medical School, Newark, NJ, USA. souayani@umdnj.edu
Sander
H W
HW
Menkes
D L
DL
Khella
S L
SL
D000208:Acute Disease; D058186:Acute Kidney Injury / Q000188:drug therapy / Q000276:immunology / Q000821:virology; D000328:Adult; D003520:Cyclophosphamide / Q000494:pharmacology* / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D016174:Hepacivirus / Q000276:immunology; D006526:Hepatitis C / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000276:immunology / Q000821:virology*; D011782:Quadriplegia / Q000188:drug therapy / Q000276:immunology / Q000821:virology*; D012074:Remission Induction / Q000379:methods; D012131:Respiratory Insufficiency / Q000188:drug therapy / Q000276:immunology / Q000821:virology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy / Q000276:immunology / Q000821:virology*
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide