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Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis

Overview of attention for article published in Orphanet Journal of Rare Diseases, October 2016
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Title
Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis
Published in
Orphanet Journal of Rare Diseases, October 2016
DOI 10.1186/s13023-016-0523-y
Pubmed ID
Authors

Michele Iudici, Pierre Quartier, Benjamin Terrier, Luc Mouthon, Loïc Guillevin, Xavier Puéchal

Abstract

The data from cohorts of childhood-onset granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) remain scarce and heterogeneous. We aimed to analyse the features at presentation, therapeutic approaches and the disease course of these rare diseases. Electronic searches of Medline and the Cochrane Central Register of Controlled trials database were conducted. We also checked the reference lists of the studies included and other systematic reviews, to identify additional reports. We included all cohorts, cross-sectional studies or registries reporting features at presentation or outcomes in patients with a diagnosis of childhood-onset GPA or MPA (age <18 years). The pooled prevalence of clinical manifestations at presentation, ANCA and induction therapies for GPA and MPA was calculated. We reviewed 570 full texts and identified 14 studies on GPA and 8 on MPA. Childhood-onset GPA and MPA occurred predominantly in female subjects during adolescence. For GPA, ear-nose-throat (ENT) disease (pooled prevalence 82 % [95 % CI 78-87]), constitutional symptoms (73 % [95 % CI 55-88]), renal (65 % [95 % CI 49-79]), and lower respiratory tract (61 % [95 % CI 48-74]) manifestations were the most frequently reported at presentation. Renal disease was a hallmark of MPA (94 % [95 % CI 89-97]). ANCA were detected in >90 % of children with GPA or MPA. Combined corticosteroids and cyclophosphamide was the most frequently used first remission-inducing treatment for GPA (76 % [95 % CI 69-82]) and MPA (62 % [95 % CI 20-96]). Relapses occurred more frequently in GPA (67-100 %) than in MPA (25-50 %). The leading causes of death were the disease itself, and infections. Childhood-onset MPA and GPA remain severe diseases with frequent relapses and a high cumulative morbidity. Survival and disease-free survival need to be improved.

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The data shown below were collected from the profiles of 3 X users who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 76 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 76 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 10 13%
Other 9 12%
Researcher 8 11%
Student > Ph. D. Student 8 11%
Student > Doctoral Student 7 9%
Other 20 26%
Unknown 14 18%
Readers by discipline Count As %
Medicine and Dentistry 44 58%
Biochemistry, Genetics and Molecular Biology 4 5%
Nursing and Health Professions 3 4%
Economics, Econometrics and Finance 2 3%
Arts and Humanities 1 1%
Other 5 7%
Unknown 17 22%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 26 October 2016.
All research outputs
#14,683,641
of 23,509,982 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,621
of 2,704 outputs
Outputs of similar age
#181,324
of 317,448 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#29
of 39 outputs
Altmetric has tracked 23,509,982 research outputs across all sources so far. This one is in the 35th percentile – i.e., 35% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,704 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.8. This one is in the 36th percentile – i.e., 36% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 317,448 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 40th percentile – i.e., 40% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 39 others from the same source and published within six weeks on either side of this one. This one is in the 20th percentile – i.e., 20% of its contemporaries scored the same or lower than it.