Outcomes in ANCA-associated vasculitides

Immunosuppressive therapy significantly improved survival of patients with ANCA-associated vasculitides (AAV). However, the adverse effects of therapy, particularly glucocorticosteroids, along with the accumulation of organ damage and a deterioration in the quality of life, have become the leading factors contributing to the disease burden. The development of a core outcome set and assessment tools is a current problem of research methodology to improve their quality and uniformity of data. Disease activity and damage assessed by the Birmingham Vasculitis Activity Score (BVAS v.3) and the Vasculitis Damage Index (VDI), respectively, as well as patient-reported outcomes (PRO) and mortality are approved as the core set domains. The main assessment tools for PRO include the AAV-specific PRO questionnaire (AAV-PRO), the Medical Outcomes Study Short-Form 36 (SF-36 v.2) and three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference). There are also a number of tools specific for the particular manifestations of the disease, such as glomerulonephritis, asthma or chronic rhinosinusitis.