Central nervous system involvement and mimickers in ANCA associated vasculitis

Central nervous system (CNS) involvement is rare in ANCA-associated vasculitis (AAV). On the other hand, AAV patients may develop complications or other conditions that mimic CNS involvement. We aimed to present the clinical, laboratory and imaging features of our AAV patients with CNS involvement and conditions other than CNS involvement that caused neurologic symptoms.

We surveyed the charts of 430 AAV patients in order to identify patients who were evaluated for neurologic symptoms suggesting CNS involvement. We extracted data on their demographics, AAV features, neurologic symptoms, final diagnoses after neurologic work-up, and their outcomes.

Of 430 AAV patients, 61 patients (14%) (41 GPA, 11 MPA, 9 EGPA; 27 women, 34 men; mean age: 51.6 ± 15.4 years) with neurologic symptoms were identified. At the time of the occurrence of neurologic symptoms, all patients had active disease [median (IQR) BVAS=11.9 (7-15)]. The causes of neurologic symptoms were CNS involvement of AAV in 7 patients (meningeal involvement in 3, ischemic cerebrovascular accident in 2, intracranial hypertension in 1, and cerebral venous sinus thrombosis in 1), other AAV manifestations such as ocular, orbital and nasopharyngeal involvement in 30, and drug-related adverse events or comorbidities mimicking CNS involvement such as infections, atherosclerotic or thromboembolic events in 15 patients. Neurologic work-up did not lead to an underlying condition in 9.

CNS involvement was uncommon, observed in only 1.6% of patients. AAV manifestations other than CNS involvement, as well as complications like infections and cardiovascular disease may mimic CNS involvement in patients with AAV.