Juvenile polyarteritis: Results of a multicenter survey of 110 children

Abstract

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.Hacettepe Univ, Fac Med, Dept Pediat, TR-06100 Ankara, TurkeyHacettepe Univ, Dept Pediat Rheumatol, Ankara, TurkeyIstanbul Univ, Istanbul, TurkeyHosp Sant Joan Deu Barcelona, Barcelona, SpainHosp Univ La Paz, Madrid, SpainGreat Ormond St Hosp Sick Children, London, EnglandUniv Coll Med Sch, London, EnglandKinderklin MHH Hannover, Hannover, GermanySch Med, Ribeirao Preto, BrazilUniv São Paulo, BR-14049 Ribeirao Preto, BrazilUniversidade Federal de São Paulo, São Paulo, BrazilUniv Fed Rio de Janeiro, Rio de Janeiro, BrazilUniv Estado Rio de Janeiro, Rio de Janeiro, BrazilUniv Utrecht, Utrecht, NetherlandsHosp Special Surg, New York, NY 10021 USASanford Weill Med Ctr, New York, NY USACharles Univ, Prague, Czech RepublicState Univ Rigshosp, Copenhagen, DenmarkUniv Louisville, Louisville, KY 40292 USAUniv Padua, Padua, ItalyUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc