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Title: Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study
Authors: David A. Cabral
Debra L. Canter
Eyal Muscal
Kabita Nanda
Dawn M. Wahezi
Steven J. Spalding
Marinka Twilt
Susanne M. Benseler
Sarah Campillo
Sirirat Charuvanij
Paul Dancey
Barbara A. Eberhard
Melissa E. Elder
Aimee Hersh
Gloria C. Higgins
Adam M. Huber
Raju Khubchandani
Susan Kim
Marisa Klein-Gitelman
Mikhail M. Kostik
Erica F. Lawson
Tzielan Lee
Joanna M. Lubieniecka
Deborah McCurdy
Lakshmi N. Moorthy
Kimberly A. Morishita
Susan M. Nielsen
Kathleen M. O'Neil
Andreas Reiff
Goran Ristic
Angela B. Robinson
Angelyne Sarmiento
Susan Shenoi
Mary B. Toth
Heather A. Van Mater
Linda Wagner-Weiner
Jennifer E. Weiss
Andrew J. White
Rae S.M. Yeung
David A. Cabral
Angelyne Sarmiento
Qun Yang
Victor Espinosa
Joanna Lubieniecki
Jaime Guzman
Kristin Houghton
Ross Petty
Lori Tucker
Mary B. Toth
Susanne Benseler
Marinka Twilt
Michael Beresford
Eileen Baildam
Marisa Klein-Gitelman
Michael Miller
Megan Curran
Taunton Southwood
Raju Khubchandani
Norman T. Ilowite
Dawn M. Wahezi
Susan Kim
Fatma Dedeoglu
Robert Fuhlbrigge
Melissa Hazen
Mary Beth Son
Robert Sundel
Andreas Reiff
Diane Brown
Katherine Marzan
Anusha Ramanathan
Bracha Shaham
Ciaran Duffy
Matthew Adams
Rudolf Valentini
Margalit Rosenkranz
Daniel Kietz
Elaine Cassidy
Kathryn Torok
Mara Becker
Lawrence K. Jung
Steven Spalding
Andrew Zeft
Anne Eberhard
Bett Gottlieb
Cagri Toruner
Linda Wagner-Weiner
Karen Onel
Charles Spencer
Deidre De Ranieri
Melissa Tesher
Andrew Eichenfield
Lisa Imundo
Heather Van Mater
C. Egla Rabinovich
Laura Schanberg
Jeffery Dvergsten
Mark Friswell
Rae Yeung
Brian Feldman
BC Children's Hospital
Texas Children's Hospital
Children's Hospital and Regional Medical Center
The Childen's Hospital at Montefiore
Cleveland Clinic Foundation
Alberta Children's Hospital
Centre universitaire de santé McGill, Hôpital de Montreal Pour Enfants
Mahidol University
New Janeway Childrens Health and Rehabilitation Centre
Children's Medical Center
University of Florida
University of Utah
Children's Hospital Columbus
IWK Health Centre
Breach Candy Hospital
Children's Hospital Boston
Ann & Robert H. Lurie Children's Hospital of Chicago
Saint Petersburg State Pediatric Medical University
University of California, San Francisco
Stanford University School of Medicine
Simon Fraser University
University of California, Los Angeles
Rutgers Robert Wood Johnson Medical School
Riley Children's Hospital
Children's Hospital Los Angeles
Mother and Child Health Care Institute of Serbia
Rainbow Babies and Children's Hosp.
Akron Children's Hospital
Duke University Medical Center
University of Chicago
The Joseph M. Sanzari Children's Hospital
St. Louis Children's Hospital
Hospital for Sick Children University of Toronto
Keywords: Immunology and Microbiology;Medicine
Issue Date: 1-Oct-2016
Citation: Arthritis and Rheumatology. Vol.68, No.10 (2016), 2514-2526
Abstract: © 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.
ISSN: 23265205
Appears in Collections:Scopus 2016-2017

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