AB0582 NEUROLOGIC INVOLVEMENT IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS –EXPERIENCE FROM A PORTUGUESE CENTER

Abstract

Background Involvement of peripheral (PNS) and central nervous system (CNS) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been reported in 15-60% and 5-15% of patients (pts), respectively. PNS involvement occurs mainly in eosinophilic granulomatosis with polyangiitis (EGPA) and CNS involvement has been almost exclusively described in granulomatosis with polyangiitis (GPA). Neurologic involvement is associated with a higher morbidity and implies more immunosuppressive treatment in most pts. Objectives Evaluate prevalence and clinical features of PNS/CNS involvement in pts with ANCA-vasculitis in a Portuguese center. Methods Retrospective analysis of ANCA-vasculitis pts with neurologic involvement followed in our department from January 2016 to December 2018. Data was collected from the Portuguese database REUMA.PT and included demographic data, vasculitis subtype, date of diagnosis, neurologic manifestations and treatment approach. Results In total, 11 pts were identified, mostly female (7/11) with mean age of 61±13.3 years (yrs). Five pts had EGPA, 5 GPA and 1 microscopic polyangiitis (MPA). Neurologic involvement was part of disease presentation in 8 pts (5 EGPA, 3 GPA). The other 3 pts had neurologic involvement 7±2.2 yrs after vasculitis diagnosis. At the time of neurologic involvement diagnosis, Birmingham Vasculitis Activity Score was 8.8±4.3. PNS involvement occurred in 9 pts, all presenting mononeuritis multiplex (MM). From these, 5 had EGPA, 3 GPA and 1 MPA. CNS involvement was reported in 3 pts, 2 with GPA and 1 with EGPA. Pachymeningitis was diagnosed in 1 GPA pt with persistent headache, refractory to analgesics, and thickening and enhancement of the dura mater on postcontrast magnetic resonance imaging. Two pts had cranial mononeuropathy, 1 with EGPA and MM who developed VI palsy 2 yrs after vasculitis diagnosis and 1 pt with GPA and VII palsy at disease presentation who developed XII palsy 8 yrs later. All pts were treated with prednisolone (1mg/kg/day), mostly in combination with other immunosuppressive drugs. The choice of the treatment was based on the age of the pt and other comorbidities. Detailed treatment of these pts and subsequent responses are shown in table 1. In our cohort there were 2 pts with serious infections: 1 with oropharyngeal and esophageal candidiasis and another with bacteraemia to Pseudomonas aeruginosa. Both pts were under immunosuppressive agents including corticosteroids and rituximab or cyclophosphamide. Conclusion Neurologic involvement was part of disease presentation in most pts and the commonest manifestation was mononeuritis multiplex. PDN was prescribed to all pts, in most cases in association with other immunosuppressive drugs. Cyclophosphamide and rituximab (RTX) were used as induction treatment, and mycophenolate mofetil, azathioprine and RTX as maintenance. Intravenous human immunoglobulin was used in pts colonized by multiresistant microorganisms/severe infection with immunosuppression and as a bridging therapy to further immunosuppression. Most pts achieved clinical improvement, documented in electromyography.