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Comparative Study
. 2016 Oct;68(10):2514-26.
doi: 10.1002/art.39729.

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

David A Cabral  1 , Debra L Canter  2 , Eyal Muscal  2 , Kabita Nanda  3 , Dawn M Wahezi  4 , Steven J Spalding  5 , Marinka Twilt  6 , Susanne M Benseler  6 , Sarah Campillo  7 , Sirirat Charuvanij  8 , Paul Dancey  9 , Barbara A Eberhard  10 , Melissa E Elder  11 , Aimee Hersh  12 , Gloria C Higgins  13 , Adam M Huber  14 , Raju Khubchandani  15 , Susan Kim  16 , Marisa Klein-Gitelman  17 , Mikhail M Kostik  18 , Erica F Lawson  19 , Tzielan Lee  20 , Joanna M Lubieniecka  21 , Deborah McCurdy  22 , Lakshmi N Moorthy  23 , Kimberly A Morishita  24 , Susan M Nielsen  25 , Kathleen M O'Neil  26 , Andreas Reiff  27 , Goran Ristic  28 , Angela B Robinson  29 , Angelyne Sarmiento  24 , Susan Shenoi  3 , Mary B Toth  30 , Heather A Van Mater  31 , Linda Wagner-Weiner  32 , Jennifer E Weiss  33 , Andrew J White  34 , Rae S M Yeung  35 ; ARChiVe Investigators Network within the PedVas Initiative
Collaborators, Affiliations
Free article
Comparative Study

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

David A Cabral et al. Arthritis Rheumatol. 2016 Oct.
Free article

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.

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