Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.
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 (E...
| Publicado en: | Arthritis & Rheumatology Vol. 68; no. 10; pp. 2514 - 2527 |
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| Autores principales: | , , , , , , , , , , , , , , , , , , , |
| Formato: | algorithm research tables/charts Journal Article |
| Publicado: |
Wiley-Blackwell
Oct2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=118371408&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 118371408 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 23265191 H06R jtl: Arthritis & Rheumatology issn: 23265191 maglogo: N pubinfo: dt: Oct2016 vid: 68 iid: 10 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 118371408 118371408 118371408 10.1002/art.39729 118371408 ppf: 2514 ppct: 13 formats: tig: atl: Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. aug: au: 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. affil: British Columbia Children's Hospital, Vancouver, British Columbia, Canada sug: subj: Vasculitis Diagnosis Vasculitis Symptoms Granulomatosis with Polyangiitis Diagnosis Granulomatosis with Polyangiitis Symptoms Demography Granulomatosis with Polyangiitis Classification Vasculitis Classification Diagnosis, Differential Granulomatosis with Polyangiitis Drug Therapy Vasculitis Drug Therapy Comparative Studies Retrospective Design Descriptive Statistics Data Analysis Software Chi Square Test Fisher's Exact Test T-Tests Mann-Whitney U Test P-Value Child Adolescence Female Male Human Funding Source Child: 6-12 years Adolescent: 13-18 years Female Male ab: 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. pubtype: Academic Journal doctype: algorithm research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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