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 - Methotrexate withdrawal at 6 vs 12 months in juvenile idiopathic arthritis in remission: a randomized clinical trial. JF - JAMA Y1 - 2010 A1 - Foell, Dirk A1 - Wulffraat, Nico A1 - Wedderburn, Lucy R A1 - Wittkowski, Helmut A1 - Frosch, Michael A1 - Gerss, Joachim A1 - Stanevicha, Valda A1 - Mihaylova, Dimitrina A1 - Ferriani, Virginia A1 - Tsakalidou, Florence Kanakoudi A1 - Foeldvari, Ivan A1 - Cuttica, Ruben A1 - Gonzalez, Benito A1 - Ravelli, Angelo A1 - Khubchandani, Raju A1 - Oliveira, Sheila A1 - Armbrust, Wineke A1 - Garay, Stella A1 - Vojinovic, Jelena A1 - Norambuena, Ximena A1 - Gamir, María Luz A1 - García-Consuegra, Julia A1 - Lepore, Loredana A1 - Susic, Gordana A1 - Corona, Fabrizia A1 - Dolezalova, Pavla A1 - Pistorio, Angela A1 - Martini, Alberto A1 - Ruperto, Nicolino A1 - Roth, Johannes KW - Adolescent KW - Antirheumatic Agents KW - Arthritis, Juvenile KW - ATP-Binding Cassette Transporters KW - Calgranulin B KW - Child KW - Child, Preschool KW - Female KW - Humans KW - Infant KW - Male KW - Methotrexate KW - Predictive Value of Tests KW - Prospective Studies KW - Recurrence KW - Remission Induction AB -

CONTEXT: Novel therapies have improved the remission rate in chronic inflammatory disorders including juvenile idiopathic arthritis (JIA). Therefore, strategies of tapering therapy and reliable parameters for detecting subclinical inflammation have now become challenging questions.

OBJECTIVES: To analyze whether longer methotrexate treatment during remission of JIA prevents flares after withdrawal of medication and whether specific biomarkers identify patients at risk for flares.

DESIGN, SETTING, AND PATIENTS: Prospective, open, multicenter, medication-withdrawal randomized clinical trial including 364 patients (median age, 11.0 years) with JIA recruited in 61 centers from 29 countries between February 2005 and June 2006. Patients were included at first confirmation of clinical remission while continuing medication. At the time of therapy withdrawal, levels of the phagocyte activation marker myeloid-related proteins 8 and 14 heterocomplex (MRP8/14) were determined.

INTERVENTION: Patients were randomly assigned to continue with methotrexate therapy for either 6 months (group 1 [n = 183]) or 12 months (group 2 [n = 181]) after induction of disease remission.

MAIN OUTCOME MEASURES: Primary outcome was relapse rate in the 2 treatment groups; secondary outcome was time to relapse. In a prespecified cohort analysis, the prognostic accuracy of MRP8/14 concentrations for the risk of flares was assessed.

RESULTS: Intention-to-treat analysis of the primary outcome revealed relapse within 24 months after the inclusion into the study in 98 of 183 patients (relapse rate, 56.7%) in group 1 and 94 of 181 (55.6%) in group 2. The odds ratio for group 1 vs group 2 was 1.02 (95% CI, 0.82-1.27; P = .86). The median relapse-free interval after inclusion was 21.0 months in group 1 and 23.0 months in group 2. The hazard ratio for group 1 vs group 2 was 1.07 (95% CI, 0.82-1.41; P = .61). Median follow-up duration after inclusion was 34.2 and 34.3 months in groups 1 and 2, respectively. Levels of MRP8/14 during remission were significantly higher in patients who subsequently developed flares (median, 715 [IQR, 320-1 110] ng/mL) compared with patients maintaining stable remission (400 [IQR, 220-800] ng/mL; P = .003). Low MRP8/14 levels indicated a low risk of flares within the next 3 months following the biomarker test (area under the receiver operating characteristic curve, 0.76; 95% CI, 0.62-0.90).

CONCLUSIONS: In patients with JIA in remission, a 12-month vs 6-month withdrawal of methotrexate did not reduce the relapse rate. Higher MRP8/14 concentrations were associated with risk of relapse after discontinuing methotrexate.

TRIAL REGISTRATION: isrctn.org Identifier: ISRCTN18186313.

VL - 303 IS - 13 U1 - http://www.ncbi.nlm.nih.gov/pubmed/20371785?dopt=Abstract ER -