Neurologic Involvement in Granulomatosis with Polyangiitis: A Comparative Study

: 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 undergone 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 the deceased patients, 18 (20.2%) were in the non-neurologic group, and 50 (38.2%) were in the neurologic group. The median (IQR) of 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 the non-neurologic and the 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%). In total, 95 patients (72.5%) complained of hearing loss, which was diagnosed as sensory–neural hearing loss; 27 patients (20.6%) complained of headache; 13 (9.9%) had a history of 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 two (1.7%) with encephalitis. Conclusion: According to this study, neurological symptoms are an undeniable part of the disease course for GPA patients, and these symptoms are associated with disease severity, prognosis, and response to treatment.