Comparison of Patient Outcomes in Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome

Elisabeth Ray¹, Didem Saygin¹, Seby John², William Messner³, Ken Uchino⁴, Leonard H. Calabrese⁵ and Rula A Hajj-Ali⁶, ¹Rheumatology, Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic, Cleveland, OH, ³Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, ⁴Cerebrovascular Center, Cleveland Clinic Foundation, Cleveland, OH, ⁵Rheumatic & Immunologic Disease, Cleveland Clinic, Cleveland, OH, ⁶Rheumatic and Immunologic Dis, Cleveland Clinic, Cleveland, OH

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: angiitis, CNS Vasculitis, outcomes and reversible cerebrovascular vasoconstriction

Session Information

Date: Tuesday, November 15, 2016

Title: Vasculitis - Poster III: Rarer Vasculitides

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is an important mimic of Primary Angiitis of the Central Nervous System (PACNS). Distinct clinical and diagnostic features differentiate both entities. However no available data exist in comparing the long term outcome of these two patient populations. The aim of this study is to compare the long term outcomes of PACNS and RCVS.

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 and reversible cerebral vasoconstriction syndrome, and the diagnoses were further agreed upon by 2 rheumatologists in the department who have particular expertise in these diseases. 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).

Results: Of the 191 patients with RCVS, 45 responded to the questionnaires (23.5%). Of the 78 patients with PACNS, 27 responded to the questionnaires (34.6%). Median follow-up (from diagnosis to date of questionnaires) of those who responded with PACNS was 66 months (+/- 54 months) and with RCVS was 78 months (4-254 months). RCVS patients had significantly better functional capacity than PACNS as measured by Barthel Index (mean 95.9 in RCVS, 79.4 in PACNS, p = 0.022). RCVS patients scored significantly better in the self-care subscale of EuroQOL, although the remainder of the quality of life measures (mobility, usual activities, and anxiety/depression) were not statistically significant. Most patients with RCVS and PACNS scored low (minimal depression) on PHQ-9, and the mean PHQ-9 score between both groups was not statistically different (p = 0.683).

Conclusion: In a comparison of outcomes of patients with PACNS and RCVS, patients with RCVS were found to have better outcomes in terms of functional capabilities, and may have better quality of life. However, they appear to have a similar rate of depression.

Disclosure: E. Ray, None; D. Saygin, None; S. John, None; W. Messner, None; K. Uchino, None; L. H. Calabrese, None; R. A. Hajj-Ali, None.

To cite this abstract in AMA style:

Ray E, Saygin D, John S, Messner W, Uchino K, Calabrese LH, Hajj-Ali RA. Comparison of Patient Outcomes in Primary Angiitis of the Central Nervous System and Reversible Cerebral Vasoconstriction Syndrome [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/comparison-of-patient-outcomes-in-primary-angiitis-of-the-central-nervous-system-and-reversible-cerebral-vasoconstriction-syndrome/. Accessed .

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