Review article

https://doi.org/10.20471/acc.2023.62.02.14

Primary Angiitis of the Central Nervous System – a Diagnostic Challenge

Joško Mitrović ; Division of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, Croatia; School of Medicine, University of Zagreb, Zagreb, Croatia; Faculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, Croatia
Majda Golob ; Division of Clinical Immunology, Allergology and Rheumatology, Department of Internal Medicine, Dubrava University Hospital, Zagreb, Croatia *
Ines Lazibat ; Faculty of Pharmacy and Biochemistry, University of Zagreb, Zagreb, Croatia; Josip Juraj Strossmayer University of Osijek, Faculty of Dental Medicine and Health, Osijek, Croatia; Dubrava University Hospital, Zagreb, Croatia

* Corresponding author.

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare and severe disease
confined to the central nervous system, i.e., the brain and spinal cord. The etiology, pathogenesis and
immune mechanism of PACNS have not yet been completely elucidated. The diagnosis is challenging;
it is based upon constellation of clinical picture, cerebrospinal fluid analysis, imaging methods or tissue
biopsy as the gold standard. In differential diagnosis of PACNS, it is necessary to rule out infectious, malignant
or systemic inflammatory diseases, as well as reversible cerebral vasoconstriction syndrome. Immunosuppressants
are cornerstone therapy for PACNS, although evidence-based strategies for the management
are lacking so far. PACNS is an entity with considerable morbidity and mortality. Awareness
of this rare and heterogeneous disease is crucial for establishing early diagnosis and treatment initiation.

Keywords

Vasculitis; Central nervous system; Immunosuppressive therapy

Hrčak ID:

308770

URI

https://hrcak.srce.hr/308770

Publication date:

1.8.2023.

Article data in other languages: croatian

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