SYSTEMIC DISORDERS WITH RHEUMATIC MANIFESTATIONS: Edited by John Stone and Arezou KhosroshahiAdult primary central nervous system vasculitis an updateSalvarani, Carloa; Brown, Robert D. Jrb; Hunder, Gene G.c Author Information aUnit of Rheumatology, Arcispedale S. Maria Nuova, Reggio Emilia, Italy bDepartment of Neurology, Mayo Clinic cProfessor Emeritus, Mayo Clinic College of Medicine, Rochester, Minnesota, USA Correspondence to Dr Carlo Salvarani, Rheumatology Unit, Arcispedale S. Maria Nuova, V.le Risorgimento N80, 42100 Reggio Emilia, Italy. Tel: +39 522 296 616; fax: +39 522 295 836; e-mail: [email protected] Current Opinion in Rheumatology: January 2012 - Volume 24 - Issue 1 - p 46-52 doi: 10.1097/BOR.0b013e32834d6d76 Buy Metrics Abstract Purpose of review The aim of this review was to cover the recent major advances made in the fields of clinical assessment, diagnosis and treatment of adult primary central nervous system vasculitis (PCNSV). Recent findings To prevent misdiagnosis, particularly with the reversible cerebral vasoconstriction syndromes, new criteria on the basis of the levels of certainty of the diagnosis of PCNSV have been proposed. Advances in the neuroimaging techniques visualizing the wall of intracranial blood vessels have improved the capacity to distinguish inflammatory from noninflammatory vascular lesions. These techniques could play in the future an important role in the diagnosis of PCNSV. Studies of larger numbers of cases have revealed a more varied histopathological inflammatory picture and disclosed an association with amyloid angiopathy. It has also been recognized that PCNSV is a heterogeneous disorder encompassing clinical subsets that differ in terms of prognosis and therapy. Finally, differently from earliest reports that suggested a poor prognosis with a fatal outcome in the majority of the cases, a large recent study from Mayo Clinic has described a more favorable course with good response to therapy and a favorable outcome in 81% of cases. Summary Our better understanding of the PCNSV spectrum and its subsets will facilitate early recognition. This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes. © 2012 Lippincott Williams & Wilkins, Inc.