%0 Journal Article %J Ital J Pediatr %D 2017 %T ANCA-associated vasculitis in childhood: recent advances. %A Calatroni, Marta %A Oliva, Elena %A Gianfreda, Davide %A Gregorini, Gina %A Allinovi, Marco %A Ramirez, Giuseppe A %A Bozzolo, Enrica P %A Monti, Sara %A Bracaglia, Claudia %A Marucci, Giulia %A Bodria, Monica %A Sinico, Renato A %A Pieruzzi, Federico %A Moroni, Gabriella %A Pastore, Serena %A Emmi, Giacomo %A Esposito, Pasquale %A Catanoso, Mariagrazia %A Barbano, Giancarlo %A Bonanni, Alice %A Vaglio, Augusto %K Age Distribution %K Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis %K Antibodies, Antineutrophil Cytoplasmic %K Child %K Child, Preschool %K Churg-Strauss Syndrome %K Female %K Granulomatosis with Polyangiitis %K Humans %K Incidence %K Male %K Microscopic Polyangiitis %K Rare Diseases %K Risk Assessment %K Severity of Illness Index %K Sex Distribution %K Survival Rate %X

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome. Herein, we review the main clinical and therapeutic aspects of childhood-onset ANCA-associated vasculitis, and provide preliminary data on demographic characteristics and organ manifestations of an Italian multicentre cohort.

%B Ital J Pediatr %V 43 %P 46 %8 2017 May 05 %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/28476172?dopt=Abstract %R 10.1186/s13052-017-0364-x %0 Journal Article %J J Rheumatol %D 2017 %T Predictors of Relapse after Discontinuing Systemic Treatment in Childhood Autoimmune Chronic Uveitis. %A Simonini, Gabriele %A Bracaglia, Claudia %A Cattalini, Marco %A Taddio, Andrea %A Brambilla, Alice %A de Libero, Cinzia %A Pires Marafon, Denise %A Caputo, Roberto %A Cimaz, Rolando %K Adolescent %K Antirheumatic Agents %K Autoimmune Diseases %K Child %K Child, Preschool %K Female %K Humans %K Male %K Predictive Value of Tests %K Recurrence %K Retrospective Studies %K Treatment Outcome %K Uveitis %K Withholding Treatment %X

OBJECTIVE: To identify clinical predictors of relapse in childhood autoimmune chronic uveitis after stopping systemic treatment.

METHODS: A retrospective, multicenter, cohort study.

RESULTS: Ninety-four children in remission, receiving no treatments and with at least a 6-month followup, were enrolled. A higher probability of maintaining remission after discontinuing treatment was shown in idiopathic compared with juvenile idiopathic arthritis uveitis (Mantel-Cox chi-square = 23.21) if inactivity had been obtained within 6 months from starting systemic treatment (Mantel-Cox chi-square = 24.17) and by antitumor necrosis factor-α treatment (Mantel-Cox chi-square = 6.43).

CONCLUSION: Type of disease, time, and type of systemic therapy to achieve inactivity predict different duration of uveitis remission after treatment withdrawal.

%B J Rheumatol %V 44 %P 822-826 %8 2017 06 %G eng %N 6 %1 http://www.ncbi.nlm.nih.gov/pubmed/28365583?dopt=Abstract %R 10.3899/jrheum.161336 %0 Journal Article %J J Rheumatol %D 2013 %T Safety and efficacy of infliximab and adalimumab for refractory uveitis in juvenile idiopathic arthritis: 1-year followup data from the Italian Registry. %A Zannin, Maria E %A Birolo, Carolina %A Gerloni, Valeria M %A Miserocchi, Elisabetta %A Pontikaki, Irene %A Paroli, Maria P %A Bracaglia, Claudia %A Shardlow, Alison %A Parentin, Fulvio %A Cimaz, Rolando %A Simonini, Gabriele %A Falcini, Fernanda %A Corona, Fabrizia %A Viola, Stefania %A De Marco, Riccardo %A Breda, Luciana %A La Torre, Francesco %A Vittadello, Fabio %A Martini, Giorgia %A Zulian, Francesco %K Adolescent %K Antibodies, Monoclonal %K Antibodies, Monoclonal, Humanized %K Antirheumatic Agents %K Arthritis, Juvenile %K Child %K Child, Preschool %K Female %K Follow-Up Studies %K Humans %K Infant %K Italy %K Male %K Registries %K Treatment Outcome %K Tumor Necrosis Factor-alpha %K Uveitis %X

OBJECTIVE: To evaluate safety and efficacy of adalimumab (ADA) and infliximab (IFX) for the treatment of juvenile idiopathic arthritis-related anterior uveitis (JIA-AU).

METHODS: Starting January 2007, patients with JIA-AU treated with IFX and ADA were managed by a standard protocol and data were entered into the National Italian Registry (NIR). At baseline, all patients were refractory to standard immunosuppressive treatment and/or were corticosteroid-dependent. Data recorded every 3 months included uveitis course, number/type of ocular complications, drug-related adverse events (AE), treatment change or withdrawal, and laboratory measures. Data of patients treated for at least 1 year were retrieved from the NIR and analyzed using descriptive statistics. Treatment efficacy was based on change in uveitis course and in number of ocular complications.

RESULTS: Up to December 2009, data for 108 patients with JIA-AU treated with anti-tumor necrosis factor-α agents were recorded in the NIR and data from 91, with at least 12 months' followup, were included in the study. Forty-eight patients were treated with IFX, 43 with ADA. Forty-seven patients (55.3%) achieved remission of AU, 28 (32.9%) had recurrent AU, and 10 (11.8%) maintained a chronic course. A higher remission rate was observed with ADA (67.4% vs 42.8% with IFX; p = 0.025). Ocular complications decreased from 0.47 to 0.32 per subject. Five patients experienced resolution of structural complications. No patient reported serious AE; 8 (8.8%) experienced 11 minor AE (9 with IFX, 2 with ADA).

CONCLUSION: IFX and ADA appear to be effective and safe for treatment of refractory JIA-related uveitis, with a better performance of ADA in the medium-term period.

%B J Rheumatol %V 40 %P 74-9 %8 2013 Jan %G eng %N 1 %1 http://www.ncbi.nlm.nih.gov/pubmed/23118110?dopt=Abstract %R 10.3899/jrheum.120583 %0 Journal Article %J Ann Rheum Dis %D 2010 %T 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. %A Ruperto, Nicolino %A Ozen, Seza %A Pistorio, Angela %A Dolezalova, Pavla %A Brogan, Paul %A Cabral, David A %A Cuttica, Ruben %A Khubchandani, Raju %A Lovell, Daniel J %A O'Neil, Kathleen M %A Quartier, Pierre %A Ravelli, Angelo %A Iusan, Silvia M %A Filocamo, Giovanni %A Magalhães, Claudia Saad %A Unsal, Erbil %A Oliveira, Sheila %A Bracaglia, Claudia %A Bagga, Arvind %A Stanevicha, Valda %A Manzoni, Silvia Magni %A Pratsidou, Polyxeni %A Lepore, Loredana %A Espada, Graciela %A Kone-Paut, Isabella %A Paut, Isabelle Kone %A Zulian, Francesco %A Barone, Patrizia %A Bircan, Zelal %A Maldonado, Maria del Rocio %A Russo, Ricardo %A Vilca, Iris %A Tullus, Kjell %A Cimaz, Rolando %A Horneff, Gerd %A Anton, Jordi %A Garay, Stella %A Nielsen, Susan %A Barbano, Giancarlo %A Martini, Alberto %K Adolescent %K Biopsy %K Child %K Delphi Technique %K Granulomatosis with Polyangiitis %K Humans %K International Cooperation %K Internet %K Polyarteritis Nodosa %K Purpura, Schoenlein-Henoch %K Reproducibility of Results %K Takayasu Arteritis %X

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.

%B Ann Rheum Dis %V 69 %P 790-7 %8 2010 May %G eng %N 5 %1 http://www.ncbi.nlm.nih.gov/pubmed/20388738?dopt=Abstract %R 10.1136/ard.2009.116624