Rheumatology advances in practice · 2025
The IgA Vasculitis Study: the evolution of a cross-sectional cohort into a trial-ready cohort.
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Williams Chloe E C, Marro Julien, Chetwynd Andrew J, Brown Joseph, Skoutelis Nikolaos, Dliso Silothabo, McBurney Catherine, Oni Louise
Аннотация
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 IgA vasculitis (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 years (s.d. 3.7) and a male:female ratio of 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 6 months, however, 17% required paediatric nephrology input. The mean timing of onset for nephritis was 25.5 days (s.d. 22.9) following disease presentation (range 0.0-101 days). Fourteen children with IgAV nephritis (IgAV-N) received immunosuppression. Children with an older age, residing in more affluent areas, with gastrointestinal involvement or a positive urine dipstick (for proteinuria and/or haematuria) at presentation had greater odds of 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. This report outlines an exemplar study to advance the field towards better interventions.
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 years (s.d. 3.7) and a male:female ratio of 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 6 months, however, 17% required paediatric nephrology input. The mean timing of onset for nephritis was 25.5 days (s.d. 22.9) following disease presentation (range 0.0-101 days). Fourteen children with IgAV nephritis (IgAV-N) received immunosuppression. Children with an older age, residing in more affluent areas, with gastrointestinal involvement or a positive urine dipstick (for proteinuria and/or haematuria) at presentation had greater odds of 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. This report outlines an exemplar study to advance the field towards better interventions.