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Pregled bibliografske jedinice broj: 1215676

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


Sestan, Mario; Kifer, Nastasia; Sozeri, Betul; Demir, Ferhat; Ulu, Kadir; Silva, Clovis A.; Campos, Reinan T; Batu, Ezgi Deniz; Koker, Ova; Sapina, Matej et al.
Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients // Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Prag, Češka Republika, 2022. str. 206-206 doi:10.1186/s12969-022-00729-z (poster, međunarodna recenzija, sažetak, znanstveni)


CROSBI ID: 1215676 Za ispravke kontaktirajte CROSBI podršku putem web obrasca

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

Autori
Sestan, Mario ; Kifer, Nastasia ; Sozeri, Betul ; Demir, Ferhat ; Ulu, Kadir ; Silva, Clovis A. ; Campos, Reinan T ; Batu, Ezgi Deniz ; Koker, Ova ; Sapina, Matej ; Srsen, Sasa ; Gagro, Alenka ; Rodrigues Fonseca, Adriana ; Rodrigues, Marta ; Rigante, Donato ; Filocamo, Giovanni ; Baldo, Francesco ; Heshin-Bekenstein, Merav ; Janne Kataja, Janne ; Held, Martina ; Frkovic, Marijan ; Ozen, Seza ; Jelusic, Marija ; on behalf of PReS Vasculitis Working Party

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022) / - , 2022, 206-206

Skup
28. kongres Europskog društva za pedijatrijsku reumatologiju

Mjesto i datum
Prag, Češka Republika, 20.09.2022. - 23.09.2022

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

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

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti



POVEZANOST RADA


Projekti:
IP-2019-04-8822 - Histološki, klinički, laboratorijski i genski prediktori ishoda bolesnika s Henoch-Schönleinovom purpurom i nefritisom (PURPURAPREDICTORS) (Jelušić, Marija, HRZZ - 2019-04) ( CroRIS)

Ustanove:
Medicinski fakultet, Zagreb,
KBC Split,
Klinički bolnički centar Zagreb

Poveznice na cjeloviti tekst rada:

doi

Citiraj ovu publikaciju:

Sestan, Mario; Kifer, Nastasia; Sozeri, Betul; Demir, Ferhat; Ulu, Kadir; Silva, Clovis A.; Campos, Reinan T; Batu, Ezgi Deniz; Koker, Ova; Sapina, Matej et al.
Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients // Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Prag, Češka Republika, 2022. str. 206-206 doi:10.1186/s12969-022-00729-z (poster, međunarodna recenzija, sažetak, znanstveni)
Sestan, M., Kifer, N., Sozeri, B., Demir, F., Ulu, K., Silva, C., Campos, R., Batu, E., Koker, O. & Sapina, M. (2022) Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients. U: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022) doi:10.1186/s12969-022-00729-z.
@article{article, author = {Sestan, Mario and Kifer, Nastasia and Sozeri, Betul and Demir, Ferhat and Ulu, Kadir and Silva, Clovis A. and Campos, Reinan T and Batu, Ezgi Deniz and Koker, Ova and Sapina, Matej and Srsen, Sasa and Gagro, Alenka and Rodrigues Fonseca, Adriana and Rodrigues, Marta and Rigante, Donato and Filocamo, Giovanni and Baldo, Francesco and Heshin-Bekenstein, Merav and Janne Kataja, Janne and Held, Martina and Frkovic, Marijan and Ozen, Seza and Jelusic, Marija}, year = {2022}, pages = {206-206}, DOI = {10.1186/s12969-022-00729-z}, keywords = {cutaneous manifestations, IgA vasculitis, IgA vasculitis nephritis, risk factors}, doi = {10.1186/s12969-022-00729-z}, title = {Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients}, keyword = {cutaneous manifestations, IgA vasculitis, IgA vasculitis nephritis, risk factors}, publisherplace = {Prag, \v{C}e\v{s}ka Republika} }
@article{article, author = {Sestan, Mario and Kifer, Nastasia and Sozeri, Betul and Demir, Ferhat and Ulu, Kadir and Silva, Clovis A. and Campos, Reinan T and Batu, Ezgi Deniz and Koker, Ova and Sapina, Matej and Srsen, Sasa and Gagro, Alenka and Rodrigues Fonseca, Adriana and Rodrigues, Marta and Rigante, Donato and Filocamo, Giovanni and Baldo, Francesco and Heshin-Bekenstein, Merav and Janne Kataja, Janne and Held, Martina and Frkovic, Marijan and Ozen, Seza and Jelusic, Marija}, year = {2022}, pages = {206-206}, DOI = {10.1186/s12969-022-00729-z}, keywords = {cutaneous manifestations, IgA vasculitis, IgA vasculitis nephritis, risk factors}, doi = {10.1186/s12969-022-00729-z}, title = {Severe cutaneous manifestations in IgA vasculitis are associated with a more severe clinical course: the experience from the largest international cohort of patients}, keyword = {cutaneous manifestations, IgA vasculitis, IgA vasculitis nephritis, risk factors}, publisherplace = {Prag, \v{C}e\v{s}ka Republika} }

Časopis indeksira:


  • Current Contents Connect (CCC)
  • Web of Science Core Collection (WoSCC)
    • Science Citation Index Expanded (SCI-EXP)
    • SCI-EXP, SSCI i/ili A&HCI
  • Scopus
  • MEDLINE


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