Thread by @Vasculitis2022

Parallel Session II: Translational Vasculitis

Clinical features and treatment of patients with severe skin changes in IgA vasculitis: multicenter study

Pediatric Rheumatology European Society: Vasculitis Working Group

Dr Marija Jelusic

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Aim:
- evaluate prevalence of severe skin manifestations
- propose treatment
- describe clinical course, laboratory findings, and outcome

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Methods:
- Observational retrospective multicenter study
- Medical records were used
- Pediatric patients with severe IgA (bullae, ulceration, & necrotic lesion)
- Control group with IgA but no severe skin manifestation

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Results:
- 41 patients were identified with severe cutaneous manifestations
- 26 males (63.4%)
- Age of onset: median 8.75
- patients with severe disease were older (8.75 vs 7.17 years)

Infection was the possible trigger in 57.7%

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97.5% of patients received glucocorticoids (local/systemic)

Patients with severe cutaneous manifestations received significantly higher frequency of systemic glucocorticoids (90.2% vs 38.9%) compared to controls

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Update since abstract submission

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Preliminary conclusion:

- Patients with severe cutaneous IgA manifestation have more renal and GI involvement and more likely to receive more frequent systemic glucocorticoids

- Most patient with renal and severe cutaneous IgA manifestation had good prognosis

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