PERIPHERAL NEUROPATHY WITH NECROTIZING VASCULITIS IN RHEUMATOID-ARTHRITIS - A CLINICOPATHOLOGICAL AND PROGNOSTIC STUDY OF 32 PATIENTS

Objective. To examine the clinicopathologic features of the noncompres sive neuropathies in rheumatoid arthritis (RA). Methods. We studied 32 patients with RA and peripheral neuropathy whose nerve and/or muscle biopsy specimens exhibited necrotizing vasculitis. Morphologic analysi s of nerve specimens included light and electron microscopy studies an d teased fiber preparation, Survival was evaluated, and the prognostic values of clinical, biologic, and pathologic features were assessed b y Cox proportional hazards model. A prognostic assessment based on the significant variables was devised to estimate the probability of surv ival of any individual patient. Results. Epi- and/or perineurial vascu litis was observed with the same frequency in the 17 patients with sen sory and motor deficit and the 15 patients with sensory neuropathies a nd was associated with axonal degeneration of an average of 77.7% of t he nerve fibers, The mean followup was 7.2 years, and the overall surv ival rate at 5 years was 57%. A full prolonged remission of the vascul itis was observed in 53% of the patients; relapse occurred in 25%, The factors correlated with mortality, in decreasing order of significanc e, were clinical cutaneous vasculitis (P = 0.0003), neuropathy affecti ng 3 or 4 limbs (P = 0.03), and depressed level of C4 (P < 0.05). The prognostic assessment indicated a wide range of 5-year probabilities o f survival, from <1% to 93%. Conclusion. Necrotizing vasculitis is res ponsible for the different patterns of noncompressive neuropathies in RA, including mononeuritis multiplex and distal symmetric sensory or s ensorimotor neuropathy. Cutaneous vasculitis, multifocal neuropathy, a nd depressed C4 level were the 3 independent variables which best pred icted mortality, We propose a prognostic assessment according to these variables, to stratify patients to receive more aggressive or less ag gressive therapy.