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

Publication ,  Journal Article
Cabral, DA; Canter, DL; Muscal, E; Nanda, K; Wahezi, DM; Spalding, SJ; Twilt, M; Benseler, SM; Campillo, S; Charuvanij, S; Dancey, P; Hersh, A ...
Published in: Arthritis Rheumatol
October 2016

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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Published In

Arthritis Rheumatol

DOI

EISSN

2326-5205

Publication Date

October 2016

Volume

68

Issue

10

Start / End Page

2514 / 2526

Location

United States

Related Subject Headings

  • United States
  • Rituximab
  • Respiratory Insufficiency
  • Renal Dialysis
  • Proteinuria
  • Plasmapheresis
  • Oxygen Inhalation Therapy
  • Nephrotic Syndrome
  • Mycophenolic Acid
  • Microscopic Polyangiitis
 

Citation

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Cabral, D. A., Canter, D. L., Muscal, E., Nanda, K., Wahezi, D. M., Spalding, S. J., … ARChiVe Investigators Network within the PedVas Initiative, . (2016). 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 Rheumatol, 68(10), 2514–2526. https://doi.org/10.1002/art.39729
Cabral, David A., Debra L. Canter, Eyal Muscal, Kabita Nanda, Dawn M. Wahezi, Steven J. Spalding, Marinka Twilt, et al. “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 Rheumatol 68, no. 10 (October 2016): 2514–26. https://doi.org/10.1002/art.39729.
Cabral DA, Canter DL, Muscal E, Nanda K, Wahezi DM, Spalding SJ, et al. 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 Rheumatol. 2016 Oct;68(10):2514–26.
Cabral, David A., et al. “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 Rheumatol, vol. 68, no. 10, Oct. 2016, pp. 2514–26. Pubmed, doi:10.1002/art.39729.
Cabral DA, Canter DL, Muscal E, Nanda K, Wahezi DM, Spalding SJ, Twilt M, Benseler SM, Campillo S, Charuvanij S, Dancey P, Eberhard BA, Elder ME, Hersh A, Higgins GC, Huber AM, Khubchandani R, Kim S, Klein-Gitelman M, Kostik MM, Lawson EF, Lee T, Lubieniecka JM, McCurdy D, Moorthy LN, Morishita KA, Nielsen SM, O’Neil KM, Reiff A, Ristic G, Robinson AB, Sarmiento A, Shenoi S, Toth MB, Van Mater HA, Wagner-Weiner L, Weiss JE, White AJ, Yeung RSM, ARChiVe Investigators Network within the PedVas Initiative. 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 Rheumatol. 2016 Oct;68(10):2514–2526.
Journal cover image

Published In

Arthritis Rheumatol

DOI

EISSN

2326-5205

Publication Date

October 2016

Volume

68

Issue

10

Start / End Page

2514 / 2526

Location

United States

Related Subject Headings

  • United States
  • Rituximab
  • Respiratory Insufficiency
  • Renal Dialysis
  • Proteinuria
  • Plasmapheresis
  • Oxygen Inhalation Therapy
  • Nephrotic Syndrome
  • Mycophenolic Acid
  • Microscopic Polyangiitis