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

OBJECTIVES: 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.

CONCLUSION: 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.

Medienart:

E-Artikel

Erscheinungsjahr:

2010

Erschienen:

2010

Enthalten in:

Zur Gesamtaufnahme - volume:69

Enthalten in:

Annals of the rheumatic diseases - 69(2010), 5 vom: 21. Mai, Seite 798-806

Sprache:

Englisch

Beteiligte Personen:

Ozen, Seza [VerfasserIn]
Pistorio, Angela [VerfasserIn]
Iusan, Silvia M [VerfasserIn]
Bakkaloglu, Aysin [VerfasserIn]
Herlin, Troels [VerfasserIn]
Brik, Riva [VerfasserIn]
Buoncompagni, Antonella [VerfasserIn]
Lazar, Calin [VerfasserIn]
Bilge, Ilmay [VerfasserIn]
Uziel, Yosef [VerfasserIn]
Rigante, Donato [VerfasserIn]
Cantarini, Luca [VerfasserIn]
Hilario, Maria Odete [VerfasserIn]
Silva, Clovis A [VerfasserIn]
Alegria, Mauricio [VerfasserIn]
Norambuena, Ximena [VerfasserIn]
Belot, Alexandre [VerfasserIn]
Berkun, Yackov [VerfasserIn]
Estrella, Amparo Ibanez [VerfasserIn]
Olivieri, Alma Nunzia [VerfasserIn]
Alpigiani, Maria Giannina [VerfasserIn]
Rumba, Ingrida [VerfasserIn]
Sztajnbok, Flavio [VerfasserIn]
Tambic-Bukovac, Lana [VerfasserIn]
Breda, Luciana [VerfasserIn]
Al-Mayouf, Sulaiman [VerfasserIn]
Mihaylova, Dimitrina [VerfasserIn]
Chasnyk, Vyacheslav [VerfasserIn]
Sengler, Claudia [VerfasserIn]
Klein-Gitelman, Maria [VerfasserIn]
Djeddi, Djamal [VerfasserIn]
Nuno, Laura [VerfasserIn]
Pruunsild, Chris [VerfasserIn]
Brunner, Jurgen [VerfasserIn]
Kondi, Anuela [VerfasserIn]
Pagava, Karaman [VerfasserIn]
Pederzoli, Silvia [VerfasserIn]
Martini, Alberto [VerfasserIn]
Ruperto, Nicolino [VerfasserIn]
Paediatric Rheumatology International Trials Organisation (PRINTO) [VerfasserIn]

Links:

Volltext

Themen:

Consensus Development Conference
Journal Article
Research Support, Non-U.S. Gov't
Validation Study

Anmerkungen:

Date Completed 15.06.2010

Date Revised 19.04.2022

published: Print

Citation Status MEDLINE

doi:

10.1136/ard.2009.116657

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM19755279X