EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
OBJECTIVE
To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA).


METHODS
Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis <or=18 years. Step 2: blinded classification by consensus panel of a representative sample of 280 cases. Step 3: statistical (sensitivity, specificity, area under the curve and kappa-agreement) and nominal group technique consensus evaluations.


RESULTS
827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classified as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classification of c-PAN required a systemic inflammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of five criteria: (1) skin involvement; (2) myalgia/muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classification of c-WG required three of six criteria: (1) histopathological evidence of granulomatous inflammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classification of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of five criteria: (1) pulse deficit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) elevated acute phase reactant.


CONCLUSIONS
European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society propose validated classification criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specificity.
Ozen
Seza
S
Hacettepe University Children's Hospital, Department of Pediatric Nephrology and Rheumatology, Sihhiye, Ankara 06100, Turkey. sezaozen@hacettepe.edu.tr
Pistorio
Angela
A
Iusan
Silvia M
SM
Bakkaloglu
Aysin
A
Herlin
Troels
T
Brik
Riva
R
Buoncompagni
Antonella
A
Lazar
Calin
C
Bilge
Ilmay
I
Uziel
Yosef
Y
Rigante
Donato
D
Cantarini
Luca
L
Hilario
Maria Odete
MO
Silva
Clovis A
CA
Alegria
Mauricio
M
Norambuena
Ximena
X
Belot
Alexandre
A
Berkun
Yackov
Y
Estrella
Amparo Ibanez
AI
Olivieri
Alma Nunzia
AN
Alpigiani
Maria Giannina
MG
Rumba
Ingrida
I
Sztajnbok
Flavio
F
Tambic-Bukovac
Lana
L
Breda
Luciana
L
Al-Mayouf
Sulaiman
S
Mihaylova
Dimitrina
D
Chasnyk
Vyacheslav
V
Sengler
Claudia
C
Klein-Gitelman
Maria
M
Djeddi
Djamal
D
Nuno
Laura
L
Pruunsild
Chris
C
Brunner
Jurgen
J
Kondi
Anuela
A
Pagava
Karaman
K
Pederzoli
Silvia
S
Martini
Alberto
A
Ruperto
Nicolino
N
D000293:Adolescent; D002648:Child; D004812:Epidemiologic Methods; D014890:Granulomatosis with Polyangiitis / Q000145:classification* / Q000175:diagnosis; D006801:Humans; D011695:IgA Vasculitis / Q000145:classification* / Q000175:diagnosis; D007391:International Cooperation; D010488:Polyarteritis Nodosa / Q000145:classification* / Q000175:diagnosis; D013625:Takayasu Arteritis / Q000145:classification* / Q000175:diagnosis; D009626:Terminology as Topic