Central Nervous System Disease in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis

Abstract

Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease. Cerebral and spinal vasculitis due to GPA and MPA presents with focal and general neurologic abnormalities.

Keywords: Antineutrophil cytoplasmic antibodies (ANCA); Chronic hypertrophic pachymeningitis; Eosinophilic granulomatosis with polyangitiis (Churg-Strauss syndrome); Granulomatosis with polyangiitis; Microscopic polyangiitis; Pituitary pseudoadenoma.