Churg-Strauss Syndrome in Childhood

Albahri, Ziad; Minxová, Lenka; Lukeš, A.; Mawiri, Abdul Al; Štefáčková, Š

doi:10.1177/0883073813506492

Cited by 5 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Literature on pediatric EGPA is scarce, and the specificities of pediatric cases remain poorly described. To the best of our knowledge, only 38 articles describing pediatric cases of EGPA are listed in the PubMed database, reporting 92 cases (mostly individual case reports) . The two largest reported pediatric EGPA series described, respectively, 8 and 13 pediatric cases.…”

Section: Introductionmentioning

confidence: 99%

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

Fina

Dubus

Tran

et al. 2018

Pediatric Pulmonology

View full text Add to dashboard Cite

This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation.

show abstract

Section: Introductionmentioning

confidence: 99%

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

Fina

Dubus

Tran

et al. 2018

Pediatric Pulmonology

View full text Add to dashboard Cite

show abstract

“…1−4,6−8 Albahri et al described a 17-year-old girl with myocardial fibrosis and mitral regurgitation whose cardiac findings recovered on follow-up, unlike our patient where endomyocardial fibrosis and severe mitral regurgitation were persistent until death. 8 Intracardiac thrombus is a rare manifestation in paediatric eosinophilic granulomatosis polyangiitis, to our knowledge, having been reported in only one case by Eleftheriou et al 2 Moreover, intracardiac thrombus, causing systolic obliteration of left ventricular mid-cavity, has not been previously reported in children. A similar case has been reported in an adult where there was systolic obliteration of left ventricular cavity with high pressure gradient.…”

Section: Discussionmentioning

confidence: 87%

Rare presentation of eosinophilic granulomatosis polyangiitis with left ventricular endomyocardial fibrosis in a child

2022

View full text Add to dashboard Cite

Eosinophilic granulomatosis polyangiitis represents less than 2% of vasculitis cases in childhood. Children have worse long-term outcomes and higher mortality. Cardiac involvement portends a worse prognosis. We describe here an adolescent girl who presented with heart failure and stroke. Her blood investigations showed eosinophilia and high IgE levels. Cardiac evaluation revealed myocarditis, intracardiac thrombus, and endomyocardial fibrosis, a rare presentation of this disease in childhood.

show abstract

Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review

et al. 2017

View full text Add to dashboard Cite

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain. He had eosinophilia, and electroneuromyography demonstrated sensorimotor polyneuropathy. His skin biopsy revealed necrotizing eosinophilic vasculitis and eosinophilic panniculitis. Although he had no respiratory symptoms or history of asthma, prominent pulmonary involvement was evident on thoracic MRI. After treatment, his complaints of pain improved but mild neuropathy persisted. After 4 years of follow-up, he had minimal hypoesthesia in his right hand but had not experienced any relapses. This case highlights the fact that in cases suspected of EGPA, even without respiratory symptoms or asthma, detailed imaging should be performed for a definitive diagnosis. In addition, mild neurological findings may persist despite treatment in EGPA. The relevant literature on EGPA, with specific reference to pediatric cases, is reviewed.

show abstract

Churg-Strauss Syndrome in Childhood

Cited by 5 publications

References 10 publications

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort

Rare presentation of eosinophilic granulomatosis polyangiitis with left ventricular endomyocardial fibrosis in a child

Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review

Contact Info

Product

Resources

About