Background IgA vasculitis (IgAV) is commonly marginalized disease in adults. Clinical data are scarce and mostly limited to patients with significant renal disease. Predictors of severity in acute adult IgAV are unknown.
Objectives To create a simple semi-quantitative score predicting the severity of adult IgAV and to aid the management of adult IgAV.
Methods We performed a paper chart review of adult, histologically proven IgAV cases, diagnosed between 01.01.2010 and 31.12.2014 at our secondary/tertiary rheumatology center. The disease activity was assessed using BVAS-3. Predictors of severe disease were identified and an IgAV severity score (IgAVSS) was constructed.
Results During the 60-month observation period, 129 new IgAV cases (58% male) were identified. The median (interquartile range (IQR), range) age was 64.2 (40.4–77.3, 18-92) years. Skin, gastrointestinal (GI), renal and joint involvement was present in 129 (purpura limited to lower limbs in 54.3% and generalized above waist in 45.7%), 48 (severe in 13), 65 (severe in 16) and 58 patients, respectively. Males and patients with generalized skin purpura had more severe disease (p=0.019 for male vs. female gender and p=0.0005 for generalized vs. limited purpura). Patients aged 45–75 years had more commonly severe GI and severe renal disease than younger (<45) or older (>75) ones (38.5% vs 31.6% vs 12.5% in case of severe GI disease and in 40.9% vs 9.5% vs 22.7% in case of severe renal disease respectively). The IgAVSS was constructed by assigning weights to patient age, gender and extent of purpura at presentation. It is a simple sum of the three domains (Table 1A). IgAVSS rendered good delineation between mild uneventful and severe IgAV when the cut off was set at ≥1.5 points. The difference in BVAS-3 between IgAVSS <1.5 and IgAVSS ≥1.5 was significant, p=0.003. (Table 1B).
Conclusions Gender, patient age and extent of purpura predict the severity of adult IgAV. We propose a semi quantitative score the IgAVSS which is calculated based on clinical characteristics only as a decision making aid to the physician in a busy outpatient clinic setting to distinguish patients who might have a more severe disease requiring hospital management from those who can be managed on the outpatient basis.
Disclosure of Interest None declared