Primary Systemic Vasculitides (PSV) are a heterogeneous group of diseases. Outcome
scores are important to evaluate vasculitis patients in a more structured and standard way and these
help physicians to predict patients with poor prognosis or high risk of relapse. Furthermore, we
need reliable outcome measures for clinical trials. There are a number of vasculitis outcome scores
available in the clinical practice with different strengths and limitations. These are mainly measures
of disease activity, disease damage, response to treatment and quality of life. Birmingham Vasculitis
Activity Score (BVAS) and its pediatric version aim to evaluate a wide scope of PSV. On the
other hand, some outcome studies have focused on a single vasculitis type since the whole group
includes different diseases with heterogeneous clinical features.
The aim of this review is to provide an overview on outcome measures currently being used in the
evaluation of patients with PSV. We mainly focus on immunoglobulin A vasculitis/Henochschönlein
purpura, Kawasaki disease, anti-neutrophil cytoplasmic antibody (ANCA)-associated
vasculitis, polyarteritis nodosa, Takayasu arteritis and Behçet’s disease.