Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. Issue 10 (28th September 2016)

• Record Type: Journal Article
• Authors: Cabral, David A.; Canter, Debra L.; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M.; Spalding, Steven J.; Twilt, Marinka; Benseler, Susanne M.; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, Barbara A.; Elder, Melissa E.; Hersh, Aimee; Higgins, Gloria C.; Huber, Adam M.; Khubchandani, Raju; Kim, Susan; Klein‐Gitelman, Marisa; Kostik, Mikhail M.; Lawson, Erica F.; Lee, Tzielan; Lubieniecka, Joanna M.; McCurdy, Deborah; Moorthy, Lakshmi N.; Morishita, Kimberly A.; Nielsen, Susan M.; O'Neil, Kathleen M.; Reiff, Andreas; Ristic, Goran; Robinson, Angela B.; Sarmiento, Angelyne; Shenoi, Susan; Toth, Mary B.; Van Mater, Heather A.; Wagner‐Weiner, Linda; Weiss, Jennifer E.; White, Andrew J.; Yeung, Rae S. M.;
• Other Names: Cabral David A. investigator.; Sarmiento Angelyne investigator.; Yang Qun investigator.; Espinosa Victor investigator.; Lubieniecki Joanna investigator.; Guzman Jaime investigator.; Houghton Kristin investigator.; Morishita Kimberly investigator.; Petty Ross investigator.; Tucker Lori investigator.; Toth Mary B. investigator.; Benseler Susanne investigator.; Twilt Marinka investigator.; Beresford Michael investigator.; Baildam Eileen investigator.; Klein‐Gitelman Marisa investigator.; Miller Michael investigator.; Curran Megan investigator.; Southwood Taunton investigator.; Khubchandani Raju investigator.; Ilowite Norman T. investigator.; Wahezi Dawn M. investigator.; Kim Susan investigator.; Dedeoglu Fatma investigator.; Fuhlbrigge Robert investigator.; Hazen Melissa investigator.; Son Mary Beth investigator.; Sundel Robert investigator.; Reiff Andreas investigator.; Brown Diane investigator.; Marzan Katherine investigator.; Ramanathan Anusha investigator.; Shaham Bracha investigator.; Duffy Ciaran investigator.; Adams Matthew investigator.; Valentini Rudolf investigator.; Rosenkranz Margalit investigator.; Kietz Daniel investigator.; Cassidy Elaine investigator.; Torok Kathryn investigator.; Becker Mara investigator.; Jung Lawrence K. investigator.; Spalding Steven investigator.; Zeft Andrew investigator.; Eberhard Anne investigator.; Gottlieb Bett investigator.; Toruner Cagri investigator.; Wagner‐Weiner Linda investigator.; Onel Karen investigator.; Spencer Charles investigator.; De Ranieri Deidre investigator.; Tesher Melissa investigator.; Eichenfield Andrew investigator.; Imundo Lisa investigator.; Van Mater Heather investigator.; Egla Rabinovich C. investigator.; Schanberg Laura investigator.; Dvergsten Jeffery investigator.; Friswell Mark investigator.; Yeung Rae investigator.; Feldman Brian investigator.; Levy Deborah investigator.; Silverman Earl D. investigator.; Laxer Ronald investigator.; Schneider Rayfel investigator.; Anton Jordi investigator.; Huber Adam M. investigator.; Lang Bianca A. investigator.; Ramsey Suzanne investigator.; Stringer Elizabeth investigator.; Dancey Paul investigator.; Li Suzanne C. investigator.; Haines Kathleen investigator.; Kimura Yukiko investigator.; Janow Ginger investigator.; Weiss Jennifer investigator.; Wood Mark investigator.; Mason Thomas investigator.; Reed Ann investigator.; Jerath Rita investigator.; Cimaz Rolando investigator.; Graham Thomas B. investigator.; Woodward Amy investigator.; Hummel Donna investigator.; Ristic Goran investigator.; Higgins Gloria C. investigator.; Luqmani Raashid investigator.; Ede Kaleo investigator.; Shishov Michael investigator.; Kingsbury Daniel J. investigator.; Cartwright Victoria investigator.; Lasky Andrew investigator.; Nielsen Susan investigator.; O'Neil Kathleen investigator.; Chira Peter investigator.; Ballinger Susan investigator.; Tarvin Stacey investigator.; Blakley Michael investigator.; Martin Neil investigator.; McDonagh Janet investigator.; Moorthy Lakshmi Nandini investigator.; Boneparth Alexis investigator.; Baszis Kevin investigator.; White Andrew investigator.; Kostik Mikhail investigator.; Shenoi Susan investigator.; Nanda Kabita investigator.; Stevens Anne investigator.; Aminoff Alexandra investigator.; Wallace Carol investigator.; McMahon Anne‐Marie investigator.; Charuvanij Sirirat investigator.; Lee Tzielan investigator.; Balboni Imelda investigator.; Cidon Michal investigator.; Frankovich Jennifer investigator.; Gerstbacher Dana investigator.; Hsu Joyce J. investigator.; Sandborg Christy investigator.; Leahy Alice investigator.; Muscal Eyal investigator.; Myones Barry L. investigator.; Campillo Sarah investigator.; Chédeville Gaëlle investigator.; Scuccimarri Rosie investigator.; Lehman Thomas investigator.; Barinstein Laura investigator.; MacDermott Emma investigator.; Adams Alexa investigator.; Foel Dirk investigator.; Robinson Angela Byun investigator.; Brooks Elizabeth B. investigator.; McCurdy Deborah investigator.; Lawson Erica investigator.; Elder Melissa E. investigator.; Schikler Kenneth N. investigator.; Rosenberg Alan investigator.; Punaro Marilynn investigator.; Nassi Lorien investigator.; Pascual Virginia investigator.; Hersh Aimee investigator.; Inman C. J. investigator.; Stern Sara investigator.; Bohnsack John investigator.; Abramson Leslie investigator.; Ebner Arno investigator.;
• Is Part Of: Arthritis & rheumatology. Volume 68:Issue 10(2016)
• Page Start: 2514
• Page End: 2526
• Publication Date: 2016-09-28
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.39729
• Languages: English
• ISSNs: 2326-5191

Abstract

Abstract : 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.