Primary Central Nervous System Vasculitis (PACNS) and Secondary Vasculitis Associated with Systemic Autoimmune Diseases

CNS vasculitis comprises a heterogeneous group of disorders characterized by inflammation of the blood vessels within the central nervous system (CNS). They can be primary (PACNS) and restricted to the CNS, or secondary, associated with systemic autoimmune diseases such as systemic lupus erythematosus, Behçet’s disease, or granulomatosis with polyangiitis.

PACNS – Primary Angiitis of the Central Nervous System. It is a rare form of primary CNS vasculitis presenting without extra-neurological manifestations. It is the most common form of CNS vasculitis, with an incidence of 2.4 cases per million people per year. This vasculitis affects medium- and small-caliber vessels of the brain and spinal cord.

Secondary Vasculitis. Secondary vasculitides affecting the CNS present with both neurological and extra-neurological manifestations. Vasculitis should be suspected in the presence of constitutional symptoms typical of autoimmune diseases (low-grade fever, asthenia, anorexia, weight loss, arthralgia, myalgia). Systemic vasculitides that can cause CNS involvement include: giant cell arteritis, Takayasu’s arteritis, polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis.

Vasculitis (infectious and/or inflammatory) represents a potentially devastating group of pathologies that often affect a young to middle-aged adult population. When a vasculitis with CNS involvement is suspected, hospitalization is mandatory to perform the necessary diagnostic investigations and initiate appropriate therapy.”