臨床および実験皮膚科学研究ジャーナル
オープンアクセス

概要

A Model Predicting Short Term Severity of IgA Vasculitis in Adults

Alojzija Hocevar, Ziga Rotar, Natasa Kejzar and Matija Tomsic

Background: Predictors of short term severity of adult immunoglobulin A vasculitis (IgAV) are unknown. We aimed to determine clinical features predicting the severity of acute adult IgAV and thus to aid the management of adult IgAV in daily practice.
Methods: Medical records of adult, histologically proven IgAV cases, diagnosed between 01.01.2010 and 30.06.2016 at our secondary/tertiary rheumatology centre were reviewed.
The disease activity was assessed using Birmingham vasculitis activity score-3. Renal disease was defined severe when nephrotic syndrome or nephritic syndrome with acute renal failure developed. Gastrointestinal (GI) disease was severe in case of bloody diarrhoea, ileus or bowel perforation.
Results: During the 78-month observation period, we identified 184 new IgAV cases (57.1% male; 43.5% ever smokers). Skin, GI, renal and joint involvement were present in 184 (generalized purpura above the waist in 47.8%), 63 (severe in 16), 88 (severe in 23), and 81 patients, respectively. Four patients died during acute disease due to vasculitis. Patients with generalized skin lesions had more commonly GI tract, severe GI tract, and kidney involvement. Current smoking was strongly associated with severity of kidney disease. In logistic regression and classification tree model the history of new onset abdominal pain or the presence of generalized purpura in ever smoker emerged as the best predictors of severe GI or renal disease.
Conclusions: Based on clinical characteristics only, the presence of abdominal pain, purpura above the waist and smoking history, seem to be good predictors of short term severity of adult IgAV.