Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

The objective of this study is to report the clinical presentation and long-term outcome of patients with non-systemic vasculitic neuropathy (NSVN) seen at our neuromuscular center. In this retrospective analysis, we assessed medical records of 60 patients with biopsy-proven NSVN (39 men, 21 women; median age: 64 years, 24-80), who were seen at our department between 1999 and 2008 and were followed up until 2014. The initial neurological findings, laboratory and neurophysiological data, treatment regimens, and outcome were analyzed in all patients. NSVN was mostly asymmetric (48/60, 80%), sensorimotor (45/60, 75%), and painful (38/60, 63%), with walking impairment as one major sign (51/60, 85%). No compound action potentials could be recorded in 29/60 (48%) sural nerves (later biopsied side) and in 6/60 (10%) tibial (motor) nerves. Pathology of sural nerve was informative in all cases irrespective of neurophysiological findings and prior immunosuppression. After initial treatment with i.v. methylprednisolone, all patients reported overall improvement. Of the 46 patients who were followed for >1 year, those with mild to moderate affliction were stable with azathioprine (19/46, 41%), while 18/46 (39%) patients were treated with cyclophosphamide and other immunosuppressants due to progression or relapse. At 4 years, 24/46 (52%) patients had either discontinued (n = 21) or had primarily refused immunosuppressive treatment (n = 3) without relapse. Age younger than the group median of 64 years was associated with better outcome. No patient evolved to systemic vasculitis. NSVN is a potentially treatable disorder of the peripheral nervous system.