Assessment of the item selection and weighting in the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis

Abstract

Objective. To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items.Methods. This study used the BVAS/WG data from the Wegener's Granulomatosis Etanercept Trial. The scoring frequencies of the 34 predefined items and any "other" items added by clinicians were calculated. Using linear regression with generalized estimating equations in which the physician global assessment (PGA) of disease activity was the dependent variable, we computed weights for all predefined items. We also created variables for clinical manifestations frequently added as other items, and computed weights for these as well. We searched for the model that included the items and their generated weights yielding an activity score with the highest R 2 to predict the PGA. Results. We analyzed 2,044 BVAS/WG assessments from 180 patients; 734 assessments were scored during active disease. The highest R 2 with the PGA was obtained by scoring WG activity based on the following items: the 25 predefined items rated on >5 visits, the 2 newly created fatigue and weight loss variables, the remaining minor other and major other items, and a variable that signified whether new or worse items were present at a specific visit. The weights assigned to the items ranged from 1 to 21. Compared with the original BVAS/WG, this modified score correlated significantly more strongly with the PGA. Conclusion. This study suggests possibilities to enhance the item selection and weighting of the BVAS/WG. These changes may increase this instrument's ability to capture the continuum of disease activity in WG.

Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

“…In spite of the slight variations in frequencies of clinical features, our findings overall support the conclusions of the PRINTO registry report, in that when compared to the adult case series (using the same comparator groups as used in that report ), adult patients showed lower frequencies of constitutional, respiratory, and renal involvement, and a higher frequency of conductive hearing loss. Unlike the PRINTO series, we did not demonstrate a higher frequency of ENT findings in our pediatric cohort when compared to adults.…”

EULAR Sjögren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren's syndrome

“…Any scale quantifying physicians' judgement of disease activity is a simplification of a complex mental process. For that purpose, two main gold standards have been used in the development of disease activity indices: (1) the PhGA28–30 and (2) the intention-to-treat approach 31 32. The PhGA was used for the development of the systemic lupus disease activity index (SLEDAI) in systemic lupus erythematosus (SLE),28–30 whereas the intention-to-treat approach was used for the development of the British Isles lupus assessment group (BILAG) for SLE31 and the DAS for rheumatoid arthritis 32.…”

“…For that purpose, two main gold standards have been used in the development of disease activity indices: (1) the PhGA28–30 and (2) the intention-to-treat approach 31 32. The PhGA was used for the development of the systemic lupus disease activity index (SLEDAI) in systemic lupus erythematosus (SLE),28–30 whereas the intention-to-treat approach was used for the development of the British Isles lupus assessment group (BILAG) for SLE31 and the DAS for rheumatoid arthritis 32. However, unlike rheumatoid arthritis that quasi-exclusively affects the articular system and in which therapeutic decisions are reproducible, the multisystemic nature of primary SS makes therapeutic decisions more variable.…”

A New Vasculitis Activity Score for Predicting Death in Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Patients

“…BVAS is a more precise measurement of the dissemination of vasculitis than JVAS, but its clinical use has potential limitations because (1) to evaluate the 68-item lists in 9 separate organ systems is complex in a clinical setting, and (2) weights of 1–3 were determined empirically [23]. Therefore, the present results suggest that compared with BVAS, assessment by JVAS [19] may be more feasible for evaluating disease severity and death in MPO-ANCA-associated microscopic vasculitis with renal involvement in Japan.…”