David A. CabralDebra L. CanterEyal MuscalKabita NandaDawn M. WaheziSteven J. SpaldingMarinka TwiltSusanne M. BenselerSarah CampilloSirirat CharuvanijPaul DanceyBarbara A. EberhardMelissa E. ElderAimee HershGloria C. HigginsAdam M. HuberRaju KhubchandaniSusan KimMarisa Klein-GitelmanMikhail M. KostikErica F. LawsonTzielan LeeJoanna M. LubienieckaDeborah McCurdyLakshmi N. MoorthyKimberly A. MorishitaSusan M. NielsenKathleen M. O'NeilAndreas ReiffGoran RisticAngela B. RobinsonAngelyne SarmientoSusan ShenoiMary B. TothHeather A. Van MaterLinda Wagner-WeinerJennifer E. WeissAndrew J. WhiteRae S.M. YeungDavid A. CabralAngelyne SarmientoQun YangVictor EspinosaJoanna LubienieckiJaime GuzmanKristin HoughtonRoss PettyLori TuckerMary B. TothSusanne BenselerMarinka TwiltMichael BeresfordEileen BaildamMarisa Klein-GitelmanMichael MillerMegan CurranTaunton SouthwoodRaju KhubchandaniNorman T. IlowiteDawn M. WaheziSusan KimFatma DedeogluRobert FuhlbriggeMelissa HazenMary Beth SonRobert SundelAndreas ReiffDiane BrownKatherine MarzanAnusha RamanathanBracha ShahamCiaran DuffyMatthew AdamsRudolf ValentiniMargalit RosenkranzDaniel KietzElaine CassidyKathryn TorokMara BeckerLawrence K. JungSteven SpaldingAndrew ZeftAnne EberhardBett GottliebCagri TorunerLinda Wagner-WeinerKaren OnelCharles SpencerDeidre De RanieriMelissa TesherAndrew EichenfieldLisa ImundoHeather Van MaterC. Egla RabinovichLaura SchanbergJeffery DvergstenMark FriswellRae YeungBrian FeldmanBC Children's HospitalTexas Children's HospitalChildren's Hospital and Regional Medical CenterThe Childen's Hospital at MontefioreCleveland Clinic FoundationAlberta Children's HospitalCentre universitaire de santé McGill, Hôpital de Montreal Pour EnfantsMahidol UniversityNew Janeway Childrens Health and Rehabilitation CentreChildren's Medical CenterUniversity of FloridaUniversity of UtahChildren's Hospital ColumbusIWK Health CentreBreach Candy HospitalChildren's Hospital BostonAnn & Robert H. Lurie Children's Hospital of ChicagoSaint Petersburg State Pediatric Medical UniversityUniversity of California, San FranciscoStanford University School of MedicineSimon Fraser UniversityUniversity of California, Los AngelesRutgers Robert Wood Johnson Medical SchoolRigshospitaletRiley Children's HospitalChildren's Hospital Los AngelesMother and Child Health Care Institute of SerbiaRainbow Babies and Children's Hosp.Akron Children's HospitalDuke University Medical CenterUniversity of ChicagoThe Joseph M. Sanzari Children's HospitalSt. Louis Children's HospitalHospital for Sick Children University of Toronto2018-12-112019-03-142018-12-112019-03-142016-10-01Arthritis and Rheumatology. Vol.68, No.10 (2016), 2514-252623265205232651912-s2.0-84988844556https://repository.li.mahidol.ac.th/handle/20.500.14594/40724© 2016, American College of Rheumatology Objective: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). Methods: The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. Results: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil. Conclusion: Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding.Mahidol UniversityImmunology and MicrobiologyMedicineArticleSCOPUS10.1002/art.39729