Primary Central Nervous System Vasculitis

Salvarani, Carlo M.D.
Hunder, Gene G. M.D.
Brown, Robert D. Jr. M.D., M.P.H.

From the Department of Neurology (C.S., R.D.B.) and Division of Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN; and the Division of Rheumatology, Azienda Ospedaliera-IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy (C.S.).

Allan H. Ropper, M.D., Editor

Dr. Salvarani can be contacted at [email protected] or at the Division of Rheumatology, Azienda Ospedaliera-IRCCS di Reggio Emilia, Viale Risorgimento N80, 42100 Reggio Emilia, Italy.

Disclosure forms provided by the authors are available with the full text of this article at NEJM.org.

We thank Caterina Giannini, M.D., John Huston III, M.D., and Teresa J.H. Christianson, B.S., for their collaboration in interpreting the findings from clinical studies on adult primary central nervous system vasculitis. We specifically thank Dr. Giannini for providing the histopathological images shown in Figure 1 and Dr. Huston for providing the images shown in Figure 2 in the current review.

New England Journal of Medicine 391(11):p 1028-1037, September 19, 2024. | DOI: 10.1056/NEJMra2314942

KEY POINTS

Primary Central Nervous System Vasculitis

Primary central nervous system (CNS) vasculitis is a rare, frequently misdiagnosed condition that affects the brain and spinal cord and is characterized by a variety of neurologic symptoms at presentation, such as focal neurologic deficits, headache, and cognitive decline.
Cerebral angiography is often used for diagnosis; however, the specificity is low, and the results must be interpreted with consideration of the patient's medical history, as well as clinical and laboratory findings and the results of magnetic resonance imaging and magnetic resonance angiography. A CNS-tissue biopsy showing vasculitis can provide a definitive diagnosis.
Small-vessel and medium-to-large-vessel inflammatory involvement characterize two subsets of primary CNS vasculitis with different diagnostic methods (biopsy vs. angiography) and distinct clinical characteristics and outcomes.
The differential diagnosis includes reversible cerebral vasoconstriction syndrome, intracranial atherosclerosis, intravascular lymphoma, moyamoya disease and syndrome, secondary cerebral vasculitis (which can occur in connective-tissue diseases), systemic vasculitis, and infections.
Early recognition is important because treatment with glucocorticoids with or without cytotoxic drugs, particularly cyclophosphamide, is effective in many patients and may prevent serious outcomes.