Juvenile polyarteritis: results of a multicenter survey of 110 children.
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.


METHODS
Pediatricians were asked to classify their patients into 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.
Department of Pediatrics, Hacettepe University, Ankara, Turkey. sezaozen@hacettepe.edu.tr <sezaozen@hacettepe.edu.tr>
Seza
S
Ozen
Jordi
J
Anton
Nil
N
Arisoy
Aysin
A
Bakkaloglu
Nesrin
N
Besbas
Paul
P
Brogan
Julia
J
García-Consuegra
Pavla
P
Dolezalova
Frank
F
Dressler
Ali
A
Duzova
Virgínia Paes Leme
VP
Ferriani
Maria Odete Esteves
MO
Hilário
Mercedes
M
Ibáñez-Rubio
Ozgur
O
Kasapcopur
Wietse
W
Kuis
Thomas J A
TJ
Lehman
Dana
D
Nemcova
Susan
S
Nielsen
Sheila Knupp
SK
Oliveira
Kenneth
K
Schikler
Flavio
F
Sztajnbok
Maria Teresa
MT
Terreri
Francesco
F
Zulian
Patricia
P
Woo
D000293:Adolescent; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D007223:Infant; D008297:Male; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D016896:Treatment Outcome; D014421:Turkey; D014481:United States