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Introduction: Central nervous system (CNS) vasculitis is rare, including in human immunodeficiency virus (HIV), occurring in less than 1% of patients. Systemic vasculitis affecting the CNS is termed secondary CNS vasculitis, whereas primary CNS vasculitis, referred to as primary angiitis of the CNS (PACNS) refers to an extremely rare disease specifically confined to the CNS. Only some cases of PACNS in HIV patients have been reported in literature.

Case Report: We report a case of a 46-year-old female with HIV who developed probable primary CNS vasculitis, which was treated with intravenous cyclophosphamide and glucocorticoids for both induction and maintenance. A systematic literature review regarding PACNS and its therapeutic management is presented in this report. There were no clinical trials for PACNS. Based on the American Academy of Neurology (AAN) classes of evidence for therapeutic effectiveness, most data is of intermediate or weak strength.

Conclusion: This case highlights diagnostic and clinical features of PACNS and provides an overview of the current literature regarding pharmacotherapy. Further case reports and additional studies are needed.

Publisher's Statement

Originally published in the International Journal of Case Reports and Images, 7(10), 644-652. Licensed under CC BY 4.0. This material can be found here.



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