Background/Purpose: Primary angiitis of the CNS (PACNS) is a rare form of vasculitis confined to the brain and spinal cord, which often presents with severe cognitive and functional deficits.  Although we have witnessed a tremendous progress in the recognition and diagnosis of this disease, there is still little known about the quality of life that these patients have following a diagnosis of PACNS. The aim of this study is 1) to evaluate patients’ functional capabilities, quality of life, and frequency of depression after being diagnosed and treated for PACNS; and 2) to determine the effect of treatment duration on patient outcomes.

Methods: This is a cross-sectional study from a tertiary referral center. Eligible patient records were identified via retrospective chart review using the ICD-9 code for cerebral angiitis. Patients were included if they met 2 of the 3 following criteria: inflammatory CSF, angiogram typical of vasculitis, or vasculitis present on pathology of the brain. In addition, the diagnosis of PACNS was further agreed upon by 2 rheumatologists in the department who have particular expertise in this disease. Self-reported questionnaires were prospectively collected from eligible subjects. Measure of disability was assessed by the Barthel Index (BI), quality of life was assessed by EuroQOL (EQ-5D-5L), and depression was assessed with Patient Health Questionnaire (PHQ-9). Treatment history was collected from patient report and chart review.

Results: 1855 patient records were identified using the ICD-9 code for cerebral angiitis. 78 patients met inclusion criteria, of which 27 responded to the questionnaires (34.6%). Median follow-up of those who responded was 5.5 years (+/- 4.7 years) since diagnosis of PACNS. Using the Barthel Index scale, 19 of 27 patients (70.4%) scored 85 or more, indicating mild disability. Meanwhile, five (18.5%) patients scored 25 or less, indicating severe disability. Using the EQ-5D-5L questionnaire, 14 of 27 patients (51.9%) had no problems with mobility, 18 (66.7%) had no problems with self-care, 15 (55.6%) had no problems with usual activities, and 14 (51.9%) had no problems with pain, but only 8 (29.6%) had no problems with anxiety. Approximately 70% of patients had minimal or no depression using both the PHQ-9 scale and EuroQOL. There was a slight positive relationship between several of the outcome measures and duration of immunosuppressive therapy, although it is not statistically significant.  

Conclusion: This is the longest reported follow-up of patients with PACNS described in the literature to-date. Most patients have mild long-term disability and minimal to no depression, which may be reflective of the advances of treatment. The data shows a slight tendency towards better scores being associated with longer treatments. However, larger studies are needed to verify the extent and strength of these relationships.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/patient-outcomes-in-primary-angiitis-of-the-central-nervous-system/