Primary angiitis of the central nervous system in adults and children

Alicia Rodriguez-Pla; Paul A Monach

doi:10.1016/j.rdc.2014.09.004

Primary angiitis of the central nervous system in adults and children

Rheum Dis Clin North Am. 2015;41(1):47-62, viii. doi: 10.1016/j.rdc.2014.09.004.

Authors

Alicia Rodriguez-Pla¹, Paul A Monach²

Affiliations

¹ Section of Rheumatology, Department of Medicine, Boston University School of Medicine, 72 East Concord Street, E533, Boston, MA 02118, USA.
² Section of Rheumatology, Department of Medicine, Boston University School of Medicine, 72 East Concord Street, E533, Boston, MA 02118, USA. Electronic address: pmonach@bu.edu.

PMID: 25399939
DOI: 10.1016/j.rdc.2014.09.004

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disease, although it is increasingly recognized both in adults and children. Little is known about pathogenesis, but efforts at classification into subtypes are being made, and the distinction of PACNS from reversible cerebral vasoconstriction syndrome has been a major advance. The prognosis for improvement, or at least stabilization, of neurologic function is good with prompt and aggressive treatment, but the diagnosis continues to be challenging. Refinement of treatment strategies is needed. Multicenter collaboration may be crucial to make additional progress via randomized trials.

Keywords: CNS vasculitis; PACNS; Primary angiitis of the central nervous system; Vasculitis; cPACNS.

Publication types

Review

MeSH terms

Adult
Child
Cyclophosphamide / therapeutic use*
Glucocorticoids / therapeutic use*
Humans
Immunosuppressive Agents / therapeutic use*
Vasculitis, Central Nervous System / classification
Vasculitis, Central Nervous System / diagnosis
Vasculitis, Central Nervous System / drug therapy*

Substances

Glucocorticoids
Immunosuppressive Agents
Cyclophosphamide