Background Peripheral nerve involvement is relatively frequently encountered in patients with granulomatosis with polyangiitis (GPA). Central nervous system (CNS) manifestations are reported to occur in about 10% of GPA patients.
Objectives We aimed to estimate the prevalence of CNS involvement in Greek patients with GPA, describe the related clinical characteristics, and identify possible predicting factors for its occurrence. We also compared the clinical picture and long term outcomes of GPA patients with and without CNS involvement.
Methods The medical charts of all patients with ANCA-associated and biopsy proven small vessel vasculitis (AAV), diagnosed in our hospital between 1995–2015, were retrospectively reviewed and GPA patients with CNS involvement were identified. Demographics, serological and clinical features, at the time of AAV diagnosis, of CNS involvement and during the follow-up time, were recorded. Comparisons of disease characteristics and outcomes, including patient survival, relapse rate and treatment-related adverse events, were performed between GPA patients with and without CNS involvement.
Results 77 patients with GPA were identified in our AAV registry. Of these, nine (11.7%) developed CNS manifestations, either at clinical presentation (33.3%) or during the follow-up (66.7%). At the time of CNS involvement, all patients were characterized by increased acute phase reactants and all but one patients had vasculitic manifestations in several other organs/systems and increased titers of ANCA. CNS symptomatology included: sensor and/or sensorimotor symptomatology (33.3%), severe headache and hearing loss (33.3%), delirium and seizures (22.2%), diplopia (11.1%) and cerebellar symptoms (11.1%). Findings from MRI were: cerebral ischemic lesions (55.6%), focal dural thickening with enhancement (22.2%), orbital mass formation (11.1%) and mastoiditis causing facial nerve palsy bilaterally (11.1%). Patients with CNS involvement, compared to those without, at initial AAV diagnosis, experienced vasculitic manifestations of the ENT system more frequently (77.8% versus 25.4%, p=0.004) and they had a lower disease activity, as assessed by the BVAS score, while during the overall course of the disease experienced lung vasculitis less frequently (44.4% vs. 79.4%, p=0.02). Comparisons between GPA patients with and without CNS manifestations did not reveal any differences in long-term outcomes including relapse rate rate/100 person-months, (95% CI) [1.812 (0.920–3.229) Vs 1.033, (0.757–1.378), respectively] (p=0.171), survival (Mantel-Cox test, p=0.244) and treatment-related adverse events
Conclusions CNS involvement was recorded in 11.7% of our GPA patients, either during the initial phase or as a late disease sequela. ENT involvement and low BVAS score at disease onset 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.
Disclosure of Interest None declared