Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study

The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between GPA patients with and without CNS involvement. Seventy-seven GPA patients were studied. Of these, 9 (11.7%) developed CNS manifestations. At the time of CNS involvement, all patients had increased acute phase reactants, and all but one had vasculitic manifestations in multiple systems and increased ANCA titers. CNS manifestations included the following: sensor/sensorimotor symptomatology (33.3%), severe headache and hearing loss (33.3%), delirium/seizures (22.2%), diplopia (11.1%), and cerebellar symptoms (11.1%). At initial GPA diagnosis, patients with CNS involvement, compared to those without, had ENT involvement more frequently (77.8 versus 25.4%, p = 0.004) along with a lower disease activity (BVAS) while during the overall disease course, they experienced lung vasculitis less frequently (44.4 vs. 79.4%, p = 0.02). Comparisons between the two groups did not reveal any differences regarding the long-term outcomes, including relapse rate, treatment-related adverse events, and patient survival. CNS involvement was recorded in 11.7% of our GPA patients. At disease onset, ENT involvement and lower BVAS scores were more common in GPA patients with CNS manifestations. Based on our results, CNS involvement did not affect the long-term outcomes of GPA patients.