Juvenile polyarteritis: Results of a multicenter survey of 110 children

Juvenile polyarteritis: Results of a multicenter survey of 110 children

Autor Ozen, S. Google Scholar
Anton, J. Google Scholar
Arisoy, N. Google Scholar
Bakkaloglu, A. Google Scholar
Besbas, N. Google Scholar
Brogan, P. Google Scholar
Garcia-Consuegra, J. Google Scholar
Dolezalova, P. Google Scholar
Dressler, F. Google Scholar
Duzova, A. Google Scholar
Ferriani, VPL Google Scholar
Hilario, MOE Google Scholar
Ibanez-Rubio, M. Google Scholar
Kasapcopur, O. Google Scholar
Kuis, W. Google Scholar
Lehman, TJA Google Scholar
Nemcova, D. Google Scholar
Nielsen, S. Google Scholar
Oliveira, S. K. Google Scholar
Schikler, K. Google Scholar
Sztajnbok, F. Google Scholar
Terreri, M. T. Google Scholar
Zulian, F. Google Scholar
Woo, P. Google Scholar
Instituição Hacettepe Univ
Istanbul Univ
Hosp Sant Joan Deu Barcelona
Hosp Univ La Paz
Great Ormond St Hosp Sick Children
Univ Coll Med Sch
Kinderklin MHH Hannover
Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
Universidade Federal do Rio de Janeiro (UFRJ)
Universidade do Estado do Rio de Janeiro (UERJ)
Univ Utrecht
Hosp Special Surg
Sanford Weill Med Ctr
Charles Univ
State Univ Rigshosp
Univ Louisville
Univ Padua
Resumo Objective To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected.Study design Pediatricians were asked to classify their patients in to one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients.Results the girl:boy ratio was 56:54, with a mean age of 9.05 +/- 3.57 years. the cases were classified as: 33 (30%) cutaneous PAN; 5 (4.6%) classic PAN associated with hepatitis B surface antigen (HBs Ag); 9 (8.1%) microscopic polyarteritis of adults associated with antineutrophil cytoplasmic antibodies (ANCA); and 63 (57.2%) systemic PAN. Cutaneous PAN was disease confined to the skin and musculoskeletal system. All patients with HBs Ag-associated classic PAN were diagnosed with renal angiograms. Antiviral treatment was administered in most cases. Microscopic PAN patients had pulmonary-renal disease, in combination or separately. ANCA was present in 87%, and 2 patients progressed to end-stage renal failure. Patients classified with systemic PAN had multiple system involvement, almost all had constitutional symptoms, and all had elevated acute phase reactants. Corticosteroids and cyclophosphamide were the first choices of immunosuppressive treatment. the overall mortality was 1.1%.Conclusions There were remarkable differences among pediatric patients with PAN, with different clinical manifestations and overall better survival and lower relapse rates when compared with adults.
Idioma Inglês
Data de publicação 2004-10-01
Publicado em Journal of Pediatrics. New York: Mosby-Elsevier, v. 145, n. 4, p. 517-522, 2004.
ISSN 0022-3476 (Sherpa/Romeo, fator de impacto)
Publicador Elsevier B.V.
Extensão 517-522
Fonte http://dx.doi.org/10.1016/j.jpeds.2004.06.046
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:000224417500020
Endereço permanente http://repositorio.unifesp.br/handle/11600/27965

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