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Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):45-51. Epub 2019 Feb 18.

Long-term outcomes of patients with primary angiitis of the central nervous system.

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Department of Rheumatic & Immunologic Disease, Cleveland Clinic, Cleveland, OH, USA.
Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Department of Rheumatic & Immunologic Disease, Cleveland Clinic, Cleveland, OH, USA.
Summa Health Medical Group, Akron, OH, USA.
Department of Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, USA.
Department of Radiology, University Hospitals, Cleveland, OH, USA.
Department of Radiology, Cleveland Clinic, Cleveland, OH, USA.



Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the brain and spinal cord, which often presents with severe cognitive and functional deficits. Despite progress in diagnosis, little is still known about long-term outcomes. Our aim was to evaluate long-term functional capabilities, quality of life, and depression, and to determine the effect of treatment duration on patient outcomes.


We identified patients by ICD-9 codes for cerebral angiitis, and included them if they met two of the three following criteria: inflammatory cerebrospinal fluid (CSF), cerebral angiogram typical of vasculitis, or findings of vasculitis on pathologic examination of brain tissue. Disability was assessed by the Barthel Index, quality of life was assessed by EuroQol, and depression was assessed with Patient Health Questionnaire.


Seventy-eight patients met the inclusion criteria, of which 27 responded to the questionnaire (34.6%). Mean follow-up of those who responded was 5.5 years (± 4.7). Nineteen of 27 patients (70.4%) had mild disability; meanwhile, 5 (18.5%) had severe disability. Fourteen of 27 patients (51.9%) had no mobility problem, 18 (66.7%) had no problems with self-care, 15 (55.6%) had no problems with usual activities, 14 (51.9%) had no pain, and 8 (29.6%) had no anxiety. Approximately 70% of patients had minimal or no depression.


This is the longest reported follow-up of patients with PACNS described in the literature to date. Most patients had mild long-term disability and minimal to no depression, which may be reflective of treatment advances.

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