ArticleBMC pediatrics2022
A toddler with an unusually severe polyarticular arthritis and a lung involvement: a case report.
Article in BMC pediatrics, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
What it found
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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.
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Who cites it
8 citing papers in PubMed, 11 citations in OpenAlex.
- A Rare Case of COPA Syndrome: Multisystem Relapse and Fatal Septic Complication.Respirology case reports · 2026Article
- Mosaicism in Two Patients with COPA Syndrome.Journal of clinical immunology · 2025Article
- Significant Reduction of Lung Disease on Baricitinib Therapy in a Patient With COPA Syndrome.Pediatric pulmonology · 2025Article
- COPA Syndrome-From Pathogenesis to Treatment.Diagnostics (Basel, Switzerland) · 2024Review
- Review
- COPA Syndrome from Diagnosis to Treatment: A Clinician's Guide.Rheumatic diseases clinics of North America · 2023Review
- Clinical Presentation and Treatment of Juvenile Idiopathic Arthritis Combined with Lung Disease: A Narrative Review.Rheumatology and therapy · 2023Review
- Childhood-Onset COPA Syndrome Recognized Retrospectively in the Context of Polyarticular Juvenile Idiopathic Arthritis and Rheumatoid Arthritis.Case reports in rheumatology · 2023Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCOPA syndrome is a rare hereditary inflammatory disease caused by mutations in the gene encoding the coatomer protein subunit alpha, causing excessive production of type I interferon. This case is a reminder for the general paediatrician, highlighting the relevance of the association between arthritis and lung involvement in toddlers. CASE PRESENTATION: We report the case of a 2-year-old girl with intermittent limping and joint pain. Her family history was relevant for a Still disease with lung involvement in the mother. Physical examination showed moderate wrist swelling. Laboratory findings on admission showed an increase in inflammatory markers, positive rheumatoid factor, antibodies antinuclear antibody (ANA) and cyclic citrullinated peptide (anti-CCP). Wrists' ultrasound documented synovial thickening, and chest X-rays showed an unexpected severe interstitial pneumopathy. Genetic testing confirmed the diagnosis of a heterozygous mutation of the COPA gene in c.841C > T (p.R281W). Janus kinase treatment was started (baricitinib, 4 mg daily per os) with a remarkable improvement in limping and joint pain after two weeks.
conclusionsIn cases of recurrent arthritis with family history and multiple involvement organs, a genetic disorder should be suspected and genetic testing should be performed. Furthermore, this case suggests that therapy with jak inhibitors may be effective and safe in interferonopathies.
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