Abstract Objectives The aim of this report is to describe a single centre cohort study and its evolutionary stages into a trial-ready cohort with the vision of stopping kidney failure secondary to IgAV. Methods The IgA Vasculitis Study was established as a single-centre, cross-sectional cohort study recruiting children with a clinical diagnosis of IgAV and has evolved into a trial-ready framework. Sociodemographic and clinical data, as well as corresponding biosamples, were collected longitudinally and the natural history of the first 100 recruits is provided. Results The IgA Vasculitis Study commenced in June 2019. The first 100 children recruited to the study had a mean age of 7.3 ± 3.7 years, male: female ratio 1.5:1. At presentation, all children had a lower limb-predominant rash, 76% had musculoskeletal involvement, 43% gastrointestinal involvement, and 23% met the definition of nephritis. Most children (54%) were discharged after six months, however 17% required paediatric nephrology input. The mean timing of onset for nephritis was 25.5 ± 22.9 days following disease presentation (range 0.0–101 days). Fourteen children with IgAV-N received immunosuppression. Children with an older age, residing in more affluent areas, had GI involvement, or had a positive urine dipstick (for proteinuria and/or haematuria) at presentation had a greater odds ratio for developing nephritis. Children with IgAV-N had statistically significantly more hospital visits and unplanned hospital admissions (p 0.001). Conclusion Nephritis remains a serious consequence of IgAV with little evidence to guide management, and this report outlines an exemplar study to advance the field towards better interventions.
Williams et al. (2025) studied this question.