engleski

Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients

Introduction: Although skin changes in IgA vasculitis (IgAV) are most commonly typical, in about 2% of children, the most severe changes may develop, including hemorrhagic vesicles, bullae, ulcerations or necroses. Objectives: We investigated whether such changes were also associated with a more severe clinical course of the disease and the need for more intensive therapy. Methods: The retrospective multinational study was conducted in 11 tertiary university medical centers. Patients were diagnosed according to the EULAR/PRES/PRINTO criteria. Data were analyzed descriptively and using the Fisher’s exact and χ2 test. Results: A total of 64 patients with the most severe cutaneous manifestations in IgAV were included in the study, of which 41 (64.1%) were male, with a mean (standard deviation, SD) age of 8.49 (4.06) years at the disease onset. They were older than control group of 596 IgAV patients who did not develop bullae or necroses, with a mean (SD) age of 7.17 (3.57) years (p=0.006). The median (25-75p) time from the onset of the first symptom to the first bullous/necrotic change was 5 (2-10.5) days. The total duration of bullous/necrotic changes was 10 (7-16) days. The most important triggers of IgAV were infections, which were present in 68.8% of patients. The distribution of bullae and necroses followed the distribution of the purpuric changes. Scars and pigmentation changes persisted in 48.5% of children. The patients with severe cutaneous manifestations developed nephritis more frequently (40.6% vs. 20.6%, p=0.001), particularly with a combination of hematuria and proteinuria. The renal disease outcome was worse than the control group (p=0.001). They were more likely to have an affected gastrointestinal system (64.1% vs. 45.5%, p=0.007) and to develop the most severe gastrointestinal manifestations (p<0.001). The majority of these patients (90.6%) were treated: 84.4% of them received systemic glucocorticoids and methylprednisolone was the most frequently used with a median (25-75p) cumulative dose of 12 (6-88.7) mg/kg for a median (25-75p) of 12.5 (4- 30) days, while 57.8% were treated with nonsteroidal antiinflammatory drugs for 7 (3.5- 18.5) days. Other drugs were administered sporadically. They were significantly more often treated with systemic glucocorticoids (84.4% vs. 37.2%, p < 0.001). Conclusion: This is the largest international cohort study showing that IgAV patients with severe cutaneous manifestations developed nephritis with worse renal outcome more frequently compared to the controls, requiring systemic glucocorticoids. These patients also presented severe gastrointestinal involvement. Support: Croatian Science Foundation, IP-2019-04- 8822.

cutaneous manifestations ; IgA vasculitis ; IgA vasculitis nephritis ; risk factors

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