Primary angiitis of the central nervous system (PACNS) is a rare and life-threatening form of vasculitis confined to the CNS. A timely diagnosis is a real challenge because clinical manifestations of PACNS are diverse and nonspecific. Headaches, cerebrospinal fluid inflammation and abnormal brain magnetic resonance imaging are prevalent. When PACNS is suspected, a thorough investigation is mandatory to rule out several potential simulators and confirm the diagnosis. Treatment of PACNS is also a challenge involving competing forces, which include the threat of serious adverse effects of potent immunosuppressive agents and the risk of neurological deteriorations due to insufficient immunosuppressant therapy. Efforts are ongoing to delineate subtypes requiring different therapeutic approaches and having distinct prognoses. Despite recent progress, PACNS is still fatal in as much as one-sixth of cases. Long-term follow-up is mandatory in patients with PACNS.
Concomitant therapy with statins and immunosuppressive agents in childhood primary angiitis of the central nervous system: Comment on the article by Cellucci et al
