Clinical spectrum and outcome of immunoglobulin A vasculitis in children: A 10-year clinical study.

BACKGROUND: Immunoglobulin A (IgA) vasculitis is the most common vasculitides of childhood. The aim of this study was to characterise the disease and identify clinical and laboratory factors associated with renal involvement.

METHODS: Medical records of children aged ≤18 years with the diagnosis of IgA vasculitis according to the EULAR/PRES criteria were retrospectively reviewed for demographic data, clinical manifestations and laboratory tests. Factors associated with renal involvement were determined using multivariable logistic regression analysis.

RESULTS: A total of 113 patients were involved in the study. There were 57.5% of males (male: female ratio of 1.4:1). The mean (SD) age was 7.1 (3.0) years. Most of the patients presented during the fall (37.2%) and winter (29.2%). The most common presenting complaints were skin rash (83.2%) and arthralgia (9.7%). The most frequent presentation of the skin was palpable purpuric rash, except for two patients (1.8%) presenting with blistering eruptions in the form of hemorrhagic bullae and ecchymoses of both extremities. We have encountered one patient with pulmonary-renal syndrome. Renal involvement was found in 27.4% of the patients. In the multivariate analysis, the strongest factors associated with renal involvement were older age and elevated creatinine level. Factors that improved model fit were higher platelet and lower white blood cell counts, elevated anti-streptolysin O and a history of preceding infection.

CONCLUSION: The clinical profile of the disease in our cohort was similar to other reports. However, we have encountered a few severe and rare atypical presentations. Over a quarter of children in our setting had renal involvement. Children presenting with these risk factors need to be closely monitored and long-term follow-up is recommended.