Publication:
Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study

Issued Date

2016-10-01

Resource Type

Article

ISSN

23265205
23265191

DOI

10.1002/art.39729

Other identifier(s)

2-s2.0-84988844556

Rights

Mahidol University

Rights Holder(s)

SCOPUS

Bibliographic Citation

Arthritis and Rheumatology. Vol.68, No.10 (2016), 2514-2526

Suggested Citation

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 Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. Arthritis and Rheumatology. Vol.68, No.10 (2016), 2514-2526. doi:10.1002/art.39729 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/40724

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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

Author(s)

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

Other Contributor(s)

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
 Rigshospitalet
 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

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.

Keyword(s)

Immunology and Microbiology
 Medicine

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URI

https://repository.li.mahidol.ac.th/handle/20.500.14594/40724

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Scopus 2016-2017