Objective: The aim of this study was to describe the presentation and outcomes of patients with granulomatosis with polyangiitis (GPA) presenting with neurologic involvement according to ACR criteria.

Methods: Consecutive newly diagnosed GPA patients who had follow-up for at least six months between 2013 and 2018 at Amir-A'lam hospital, Tehran University of Medical Sciences, were retrospectively analyzed.

Results: Patients were divided into two groups, those with nervous system involvement at either disease diagnosis or follow-up (89 patients) and those without neurological symptoms until the last follow-up (131 patients). From all patients reviewed in this study, 68 (30.9%) patients died during the follow-up period. Among dead patients, 18 (20.2%) were in the non-neurologic, and 50 (38.2%) were in the neurologic group. The median (IQR) of Birmingham Vasculitis Activity Score (BVAS) in 220 patients was 11.0 (18.0-8.0) in total, 10.0 (14.5-7.50), and 12.0 (21.0-8.0) in non-neurologic and neurologic groups, respectively. The score of BVAS in the neurologic group was significantly higher than in the non-neurologic group (p=0.039). Of 131 patients, sensory neuropathy was found in 99 patients (75.5%). 95 patients (72.5%) complained of hearing loss, diagnosed as sensory-neural hearing loss and 27 patients (20.6%) complained of headache. 13 patients (9.9%) had cerebrovascular events, 5 (3.8%) had an episode of seizure or loss of consciousness (LOC), and 3 (2.3%) had mononeuritis multiplex. Two patients (1.5%) were diagnosed with meningitis and 2 (1.7%) with encephalitis.

Conclusion: According to this study, neurological symptoms are an undeniable part of GPA patients, which are associated with disease severity, prognosis, and response to treatment.