Standardized neurologic evaluations of 128 patients with Wegener granulomatosis

Objective: To assess the frequency and type of neurologic involvement in a cohort of patients with generalized Wegener granulomatosis (WG). Patients and Methods: In a prospective analysis the clinical, electrophysiologic, radiological, and serologic data of 128 patients have been studied over a median observation period of 19 months (range, 1-60 months). Results: Sixty-four patients (50%) revealed central or peripheral nervous system involvement. Peripheral neuropathy (PN) affected 56 patients, in 9 cases the central nervous system was involved, and in 6 cases the cranial nerves were involved. Thirty-one patients showed a distal symmetrical polyneuropathy, 25 a mononeuritis multiplex. Within the first 2 years of the disease course 47 of the 56 patients had developed their PN, sometimes as the initial symptom of WG. Patients with PN were significantly more often male (34 of 65 patients) than female (22 of 63 patients, P = .04), were significantly older at the onset of WG (median age, 53 vs 44 years; P = .001), had a significantly larger disease extent (P = .001), and had higher classic antineutrophil cytoplasmic antibody titers (P = .002) than neurologically unaffected patients. Response to immunosuppression was moderate concerning peripheral nervous system manifestations. Conclusions: Peripheral neuropathy is frequent in generalized WG, occurring early in the disease course. As PN can be the first and sole symptom of a beginning systemic vasculitis, it is important that in cases of PN of an unclear origin, interdisciplinary investigations are initiated to detect, treat, and closely follow-up a possible underlying WG, especially as these patients seem to have a more severe disease course.