Evidence map›Paper›PMID 40457436›Full record

ArticlePediatric rheumatology online journal2025

Two unique cases of eosinophilic granulomatosis with polyangiitis in childhood treated with anti-interleukin-5 therapy: infantile-onset and submandibular salivary gland involvement.

Evangelia Constantine, Adam M Bartholomeo, Inna Powers, Jerimiah L Lysinger, Pia J Hauk, Jordan Abbott, Heather H de Keyser, Nicholas J Gilman, Csaba Galambos, Angus Toland and 5 more

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Pediatric rheumatology online journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Evangelia ConstantineSchool of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Adam M BartholomeoDepartment of Pediatrics, Section of Rheumatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Inna PowersDepartment of Pediatrics, Section of Rheumatology, University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Jerimiah L LysingerPediatric Pulmonology, Billings Clinic, Billings, MT, USA.
Pia J HaukDepartment of Pediatrics, Section of Allergy and Immunology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Jordan AbbottDepartment of Pediatrics, Section of Allergy and Immunology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Heather H de KeyserDepartment of Pediatrics, Section of Pulmonary and Sleep Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Nicholas J GilmanSchool of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Csaba GalambosDepartment of Pathology and Laboratory Services, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Angus TolandDepartment of Pathology and Laboratory Services, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Nicholas WillardDepartment of Pathology and Laboratory Services, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Jason WeinmanDepartment of Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
David MongDepartment of Radiology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Clara LinDepartment of Pediatrics, Section of Rheumatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Jessica L BloomDepartment of Pediatrics, Section of Rheumatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA. Jessica.Bloom@childrenscolorado.org.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundANCA-associated vasculitis is a systemic autoimmune disease involving small- and medium-sized blood vessels. Eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg Strauss Syndrome) is the least common form in childhood with few cases reported. We present two unique pediatric cases, both of which were treated with anti-interleukin-5 therapy. CASE PRESENTATION: Case one is a 13-year-old male with asthma and allergies who presented with one month of cough and periorbital edema and subsequently developed submandibular swelling. Evaluation identified chronic sinusitis, weight loss, positive c-ANCA and anti-MPO IgG antibodies, peripheral blood eosinophilia, pulmonary eosinophilia, tracheal and pulmonary nodules, and eosinophilic infiltration of the submandibular salivary gland with granulomas and fibrosis fitting a diagnosis of EGPA. He improved with glucocorticoids and mepolizumab with a significant partial response, and eventually switched to benralizumab and mycophenolate mofetil with complete response. Case two presented at 19-months-old in acute respiratory distress with a history of reactive airway disease. EGPA diagnosis was confirmed on lung biopsy (eosinophilic capillaritis and interstitial expansion of eosinophils) in the setting of anti-MPO and p-ANCA positivity. He has done very well on mepolizumab for three years.

conclusionsTo our knowledge, this is the first reported case of submandibular salivary gland infiltrate in a child with EGPA and the youngest successfully treated patient with EGPA reported in the literature. These cases demonstrate the variation in age and disease manifestations seen in children with EGPA as well as positive responses to anti-interleukin-5 therapy. Children with EGPA may present with common or unusual complaints and require astute recognition to avoid delays in diagnosis and long-term damage.

Indexed as

Antibodies, Monoclonal, HumanizedChurg-Strauss SyndromeInterleukin-5Submandibular GlandAdolescentGlucocorticoidsHumansInfantMaleTreatment OutcomeAntibodies, Monoclonal, HumanizedGlucocorticoidsInterleukin-5mepolizumabANCA-associated vasculitisanti-IL5 therapyBenralizumabChurg StraussEGPAEosinophilic granulomatosis with polyangiitisMepolizumabSialadenitis

Identifiers

PMID40457436
PMCPMC12128228

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.