A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children
Authors' conclusion
This series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden.
Study context
- Population
- Children diagnosed with recurrent or persisting igA vasculitis (IgAV) at Alder Hey Children's Hospital (Liverpool, UK)
- Participants
- 13 children
- Exposure
- Recurrent disease vs Persisting disease
- Recurrent diseasen = 4
- Persisting diseasen = 9
- Outcome
- Characteristics of atypical IgAV
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Median age at first presentation, years [range]
- 10.2 [2.6-15.5]
- Female:male ratio
- 1.2:1
- Re-presented with purpuric rash
- All children
- Joint involvement
- 92% (12/13)
- Disease-modifying anti-rheumatic drugs (DMARDs) used
- 8/13 (62%)
- Median time from first presentation to diagnosis of atypical disease, months
- 18.4 [5.3-150.8]
- Time from first presentation to treatment, months
- 24.1 [1.8-95.4]
- Admitted at least once during follow up
- 8/13 (62%)
- Re-presented at least once to ED
- 10/13 (77%)
- Referred to psychology services
- 5 (38%)
- Reported feelings of frustration
- 7 (54%)
Observational data: associations, not effects.
Further findings
| Outcome | Values | P value |
|---|---|---|
| Age at first presentation, atypical vs non-complicated disease courseAtypical cohort significantly older | Non-complicated cohort median 5.5 (range 0.6–16.7) years | P = 0.003 |
- Age at first presentation, atypical vs non-complicated disease course — Median age at first presentation, whole series (13 children)
- 10.2 years [2.6-15.5]
DMARD use
- Children treated with DMARDs
- 8/13 (62%)
Time from first presentation to diagnosis of atypical disease and to treatment
- Median time to diagnosis of atypical disease
- 18.4 months [5.3-150.8]
- Time to treatment
- 24.1 months [1.8-95.4]
Corticosteroid use by renal involvement
- Corticosteroid use significantly higher with renal involvement
- P = 0.026
Health-care use and disease burden during follow-up
- Admitted at least once
- 8/13 (62%)
- Re-presented at least once to the ED
- 10/13 (77%)
- Referred to psychology services
- Five (38%)
- Reported feelings of frustration
- 7 (54%)
Original publication
A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children. Pediatr Rheumatol Online J. Published 14 August 2023. doi:10.
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