Document Detail

EULAR/PRINTO/PRES criteria for Henoch-Sch?nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria.
MedLine Citation:
PMID:  20413568     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: To validate the previously proposed classification criteria for Henoch-Sch?nlein 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.
Seza Ozen; Angela Pistorio; Silvia M Iusan; Aysin Bakkaloglu; Troels Herlin; Riva Brik; Antonella Buoncompagni; Calin Lazar; Ilmay Bilge; Yosef Uziel; Donato Rigante; Luca Cantarini; Maria Odete Hilario; Clovis A Silva; Mauricio Alegria; Ximena Norambuena; Alexandre Belot; Yackov Berkun; Amparo Ibanez Estrella; Alma Nunzia Olivieri; Maria Giannina Alpigiani; Ingrida Rumba; Flavio Sztajnbok; Lana Tambic-Bukovac; Luciana Breda; Sulaiman Al-Mayouf; Dimitrina Mihaylova; Vyacheslav Chasnyk; Claudia Sengler; Maria Klein-Gitelman; Djamal Djeddi; Laura Nuno; Chris Pruunsild; Jurgen Brunner; Anuela Kondi; Karaman Pagava; Silvia Pederzoli; Alberto Martini; Nicolino Ruperto;
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Publication Detail:
Type:  Consensus Development Conference; Journal Article; Research Support, Non-U.S. Gov't; Validation Studies    
Journal Detail:
Title:  Annals of the rheumatic diseases     Volume:  69     ISSN:  1468-2060     ISO Abbreviation:  Ann. Rheum. Dis.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-23     Completed Date:  2010-06-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372355     Medline TA:  Ann Rheum Dis     Country:  England    
Other Details:
Languages:  eng     Pagination:  798-806     Citation Subset:  IM    
Hacettepe University Children's Hospital, Department of Pediatric Nephrology and Rheumatology, Sihhiye, Ankara 06100, Turkey.
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MeSH Terms
Epidemiologic Methods
International Cooperation
Polyarteritis Nodosa / classification*,  diagnosis
Purpura, Schoenlein-Henoch / classification*,  diagnosis
Takayasu Arteritis / classification*,  diagnosis
Terminology as Topic
Wegener Granulomatosis / classification*,  diagnosis

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