TY - JOUR T1 - EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. JF - Ann Rheum Dis Y1 - 2010 A1 - Ruperto, Nicolino A1 - Ozen, Seza A1 - Pistorio, Angela A1 - Dolezalova, Pavla A1 - Brogan, Paul A1 - Cabral, David A A1 - Cuttica, Ruben A1 - Khubchandani, Raju A1 - Lovell, Daniel J A1 - O'Neil, Kathleen M A1 - Quartier, Pierre A1 - Ravelli, Angelo A1 - Iusan, Silvia M A1 - Filocamo, Giovanni A1 - Magalhães, Claudia Saad A1 - Unsal, Erbil A1 - Oliveira, Sheila A1 - Bracaglia, Claudia A1 - Bagga, Arvind A1 - Stanevicha, Valda A1 - Manzoni, Silvia Magni A1 - Pratsidou, Polyxeni A1 - Lepore, Loredana A1 - Espada, Graciela A1 - Kone-Paut, Isabella A1 - Paut, Isabelle Kone A1 - Zulian, Francesco A1 - Barone, Patrizia A1 - Bircan, Zelal A1 - Maldonado, Maria del Rocio A1 - Russo, Ricardo A1 - Vilca, Iris A1 - Tullus, Kjell A1 - Cimaz, Rolando A1 - Horneff, Gerd A1 - Anton, Jordi A1 - Garay, Stella A1 - Nielsen, Susan A1 - Barbano, Giancarlo A1 - Martini, Alberto KW - Adolescent KW - Biopsy KW - Child KW - Delphi Technique KW - Granulomatosis with Polyangiitis KW - Humans KW - International Cooperation KW - Internet KW - Polyarteritis Nodosa KW - Purpura, Schoenlein-Henoch KW - Reproducibility of Results KW - Takayasu Arteritis AB -

OBJECTIVES: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

METHODS: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis

RESULTS: A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a kappa-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall kappa of 0.79 (95% CI 0.73 to 0.84).

CONCLUSION: EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

VL - 69 IS - 5 U1 - http://www.ncbi.nlm.nih.gov/pubmed/20388738?dopt=Abstract ER - TY - JOUR T1 - Predictors of poor response to methotrexate in polyarticular-course juvenile idiopathic arthritis: analysis of the PRINTO methotrexate trial. JF - Ann Rheum Dis Y1 - 2010 A1 - Vilca, Iris A1 - Munitis, Pablo Garcia A1 - Pistorio, Angela A1 - Ravelli, Angelo A1 - Buoncompagni, Antonella A1 - Bica, Blanca A1 - Campos, Lucia A1 - Häfner, Renate A1 - Hofer, Michael A1 - Ozen, Seza A1 - Huemer, Christian A1 - Bae, Sang Cheol A1 - Sztajnbok, Flavio A1 - Arguedas, Olga A1 - Foeldvari, Ivan A1 - Huppertz, Hans Iko A1 - Gamir, María Luz A1 - Magnusson, Bo A1 - Dressler, Frank A1 - Uziel, Yosef A1 - van Rossum, Marion A J A1 - Hollingworth, Peter A1 - Cawkwell, Gail A1 - Martini, Alberto A1 - Ruperto, Nicolino KW - Adolescent KW - Antibodies, Antinuclear KW - Antirheumatic Agents KW - Arthritis, Juvenile KW - Child KW - Child, Preschool KW - Disability Evaluation KW - Female KW - Follow-Up Studies KW - Humans KW - Immunosuppressive Agents KW - Male KW - Methotrexate KW - Prognosis KW - Treatment Outcome AB -

OBJECTIVES: To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis.

METHODS: Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology International Trials Organization (PRINTO) MTX trial. Bivariate and logistic regression analyses were used to identify baseline predictors of poor response according to the American College of Rheumatology pediatric (ACR-ped) 30 and 70 criteria.

RESULTS: In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients. With multivariate logistic regression analysis, the most important determinants of ACR-ped 70 non-responders were: disease duration > 1.3 years (OR 1.93), ANA negativity (OR 1.77), Childhood Health Assessment Questionnaire (CHAQ) disability index > 1.125 (OR 1.65) and the presence of right and left wrist activity (OR 1.55). Predictors of ACR-ped 30 non-responders were: ANA negativity (OR 1.92), CHAQ disability index > 1.14 (OR 2.18) and a parent's evaluation of child's overall well-being < or = 4.69 (OR 2.2).

CONCLUSION: The subgroup of patients with longer disease duration, ANA negativity, higher disability and presence of wrist activity were significantly associated with a poorer response to a 6-month MTX course.

VL - 69 IS - 8 U1 - http://www.ncbi.nlm.nih.gov/pubmed/20525842?dopt=Abstract ER -