Background IgA Vasculitis is a leukocytoclastic vasculitis involving small vessels with depositions of immune complexes containing IgA. There is limited data for the prognosis of adult IgA Vasculitis, with also no damage assessment.
Objectives We aimed to evaluate the clinical characteristics, treatment, outcome and damage of patients with adult IgA Vasculitis.
Methods We assembled a retrospective cohort of patients with adult IgA Vasculitis, from tertiary Rheumatology Centers in Turkey. All data were abstracted from medical records. Birmingham Vasculitis Activity Score (BVAS),prognostic Five Factor Score (FFS) and vasculitis damage index (VDI) were calculated.
Results The study included 52 (male/female:40/12) patients with adult IgA Vasculitis. The mean age was 42.2±17 years. Infection history within 6 weeks before presentation was present in 22 (42.3%) patients (18 upper respiratory tract,3 gastrointestinal and 1 urinary tract). Cutaneous manifestations were the most common clinical manifestations (Table 1). All patients were treated with oral glucocorticoids (GC).As additional immunosuppressive agents, azathiopirine was given to 21 (40.4%) and pulse cyclophosphamide to 11 (21.2%) patients. Twenty-eight patients (53.9%) had follow-up of 28.6 months. Five (17.8%) patients relapsed during follow-up. While 3 relapses were major, 2 of them were minor relapses. At the last visit, disease status was evaluated as active or treatment failure by the treating physician in 6 (21.4%) patients. Mortality was 3.6% (n=1) during follow-up, due to pneumonia. The mean VDI score was 0.6 in the last visit. Nine (32.1%) patients had at least one damage item at the end of follow-up period.
Conclusions Our results showed that approximately one fifth of patients with adult IgA Vasculitis had relapses during follow-up. At the end of follow-up, one third of patients had at least one damage item. Although, 45% of patients had FFS≥1, the mortality rate was observed to be low in the present study.
Disclosure of Interest None declared