Background/Purpose:

ANCA-associated vasculitis (AAV) is a rare group of diseases comprising Granulomatosis with polyangiitis (Wegener’s; GPA), Microscopic Polyangiitis (MPA), and Churg-Strauss Syndrome (CSS). These diseases often result in severe morbidity and frequent relapses. The Birmingham Vasculitis Activity Score v3 (BVAS) is a well-established and validated tool to measure AAV activity. However, current tools do not include patient-reported outcomes to assess for disease activity. The Multi-Dimensional Health Assessment Questionnaire (MDHAQ) has been documented to be effective in many rheumatic diseases. Therefore, we compared BVAS scores to a patient-only index termed the “Routine Assessment of Patient Index Data 3” (RAPID3) on an MDHAQ.

Methods:

Patients with AAV treated by one rheumatologist at Rush University Medical Center in Chicago, IL from Jan 2010 to May 2012 were asked to participate and given MDHAQ to complete for 4 consecutive visits approximately every 6 months.  An independent investigator scored RAPID3, which comprises 3 Core Data Set measures on the MDHAQ for function, pain, and patient global assessment (PATGL) and takes 5 seconds to score; scores range from 0 to 30, with higher scores being worse. BVAS was calculated at each patient visit; scores range from 0 to 63, with worse disease being higher. Both scores were compared using Spearman non-parametric correlations. BVAS was also compared to PATGL, which is a visual analogue scale from 0 to 10 and is one of the Core Data Set measures in RAPID3. Linear regression was used to adjust for age, sex, ethnicity, RAPID3 version language, type of the disease, duration of the disease, years of schooling and type of insurance. P ≤ 0.5 was considered significant. The institutional Review Board approved the study.

Results:

Twenty-nine patients with AAV consented and were included in the study, 22 had GPA, 5 MPA and 2 CSS. The mean age was 54.1 years, 77% were females, 69% Caucasians, 23% Hispanics, and 8% African-Americans. The mean duration of disease was 4.3 years. The mean BVAS at first visit was 6.1 ± 0.9 (range: 0-17), RAPID3 was 8 ± 1.3 (range: 0-22.7), and PTGA was 3.6 ± 0.5. RAPID3 correlated with BVAS at each visit (rho =0.45, 0.75, 0.73, 0.54 with p values of 0.02, <0.0001, 0.002, and 0.05 for visits 1 to 4, respectively) and PATGL correlated with BVAS at 3 out of 4 visits, independently of RAPID3 (rho= 0.24, 0.75, 0.64, 0.59 with p values of 0.23, <0.0001, 0.01, 0.01 for visits 1 to 4, respectively).

Conclusion:

RAPID3, a patient-only index, is correlated significantly with the BVAS. RAPID3 can be calculated in 5 seconds and does not require physician input, laboratory or imaging information. PATGL, a one simple measure of patient global assessment, may also reflect disease activity. As patient-relevant outcomes become increasingly important to insurers and to society, it is critical to identify valid patient-reported markers of vasculitis activity; RAPID3 permit longitudinal assessments of disease activity at any medical facility by any physician or even away from physician’s offices. In the face of increased expenses and busy practices, such instruments may help document patient status and add to clinical decisions.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/patient-reported-outcomes-in-anca-associated-vasculitis-a-prospective-comparison-between-birmingham-vasculitis-activity-score-and-routine-assessment-of-patient-index-data-3/