Pemphigus Disease Activity Measurements: Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, and Pemphigus Vulgaris Activity Score Publisher



Z Rahbar ZIBA
Source: Published:2024

Abstract

Pemphigus is a group of rare skin conditions with blisters in the skin and mucosa that are associated with autoantibodies against intercellular adhesion molecules. Effective therapies can improve its devastating morbidities and mortality. Using a uniform, consensus-based and validated disease activity measure is essential to multicenter clinical trials for new therapies, meta-analysis of smaller studies, and evidence-based practice. However, more than 116 outcome measures exist to assess pemphigus disease activity. The most comprehensively validated measures are Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS). The consensus-based standards for the selection of health status measurement instruments (COSMIN) study recommends investigation for the measurement properties of reliability, validity, and responsiveness. Here, PDAI, ABSIS, and PVAS are presented and compared based on their measurement properties including reliability, feasibility, and correlation with anti-desmoglein levels using one large prospective study (n = 100) comparing all three measures. Additionally, a review of additional properties including validity, responsiveness, and severity cutoffs is included using updated studies. PDAI has the highest reliability and is least affected by the variability in rater’s pemphigus expertise. All three pemphigus measures have acceptable convergent validity with Physician Global Assessment, but PVAS seems to have the highest value. PVAS is the fastest and simplest score to complete. Responsiveness studies may be needed for comprehensive validation. There are inconsistencies in severity cutoffs. Recently, PDAI, as the internationally accepted pemphigus disease measure, has been increasingly used in different studies around the world. © 2025 Elsevier B.V., All rights reserved.