ReviewDiagnostics (Basel, Switzerland)2022
Lung Involvement in Systemic Juvenile Idiopathic Arthritis: A Narrative Review.
Review in Diagnostics (Basel, Switzerland), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 18 citations in OpenAlex.
- Utilizing a chest CT interstitial lung disease scoring system for commonly encountered pediatric rheumatologic diseases with associated interstitial lung disease with clinical correlation.Pediatric radiology · 2026Article
- Radiological Change in Interstitial Lung Abnormalities and Interstitial Lung Disease in Patients With Rheumatoid Arthritis at 1 Year.International journal of rheumatic diseases · 2026Article
- Distinct cytokine and chemokine alterations in bronchoalveolar fluid from patients with systemic juvenile idiopathic arthritis associated lung disease (SJIA-LD).Arthritis research & therapy · 2026Article
- Methotrexate Therapy in Juvenile Idiopathic Arthritis: No Clinically Relevant Pulmonary Impairment but Frequent Transient Liver Enzyme Elevations in a Longitudinal Single-Center Pediatric Cohort of 274 Children Over 30 Years.ACR open rheumatology · 2026Article
- Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease.ACR open rheumatology · 2026Article
- Case Report: Treatment of refractory lung disease in systemic juvenile idiopathic arthritis with cyclophosphamide and rituximab combination therapy.Frontiers in immunology · 2026Article
- Assessing Lung Involvement in Paediatric Rheumatic Diseases: A Retrospective Evaluation of the Limitations of Spirometry.Mediterranean journal of rheumatology · 2025Article
- Unmet needs and research gaps in Still's disease across ages: proceedings from a pediatric and adult joint expert panel.Pediatric rheumatology online journal · 2025Article
- Assessment of Pulmonary Function in Children with Juvenile Idiopathic Arthritis: A Cross-Sectional Study.Children (Basel, Switzerland) · 2025Article
- Advancing multidisciplinary management of pediatric hyperinflammatory disorders.Frontiers in pediatrics · 2025Review
- Worldwide evaluation of Clinical Practice Strategies (CliPS) for lung involvement in Still's disease within the JIR-CliPS network: a COST action.Rheumatology advances in practice · 2025Article
- Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study.Pediatric rheumatology online journal · 2024Observational
- Initial Respiratory System Involvement in Juvenile Idiopathic Arthritis with Systemic Onset Is a Marker of Interstitial Lung Disease: The Results of Retrospective Cohort Study Analysis.Journal of clinical medicine · 2024Article
- Review
- Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study.Medicina (Kaunas, Lithuania) · 2023Article
- Systemic juvenile idiopathic arthritis: The Great Ormond Street Hospital experience (2005-2021).Frontiers in pediatrics · 2023Article
- Imaging Findings of Pediatric Rheumatic Disorders: JIA in the PRINTO Era and Autoimmune Interferonopathies.Pediatrics international : official journal of the Japan Pediatric SocietyReview
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Systemic juvenile idiopathic arthritis associated with lung disorders (sJIA-LD) is a subtype of sJIA characterized by the presence of chronic life-threatening pulmonary disorders, such as pulmonary hypertension, interstitial lung disease, pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia, which were exceptionally rare before 2013. Clinically, these children show a striking dissociation between the relatively mild clinical manifestations (tachypnoea, clubbing and chronic cough) and the severity of the pulmonary inflammatory process. Our review describes sJIA-LD as having a reported prevalence of approximately 6.8%, with a mortality rate of between 37% and 68%. It is often associated with an early onset (<2 years of age), macrophage activation syndrome and high interleukin (IL)-18 circulating levels. Other risk factors may be trisomy 21 and a predisposition to adverse reactions to biological drugs. The most popular hypothesis is that the increase in the number of sJIA-LD cases can be attributed to the increased use of IL-1 and IL-6 blockers. Two possible explanations have been proposed, named the “DRESS hypothesis” and the “cytokine plasticity hypothesis”. Lung ultrasounds and the intercellular-adhesion-molecule-5 assay seem to be promising tools for the early diagnosis of sJIA-LD, although high resolution computed tomography remains the gold standard. In this review, we also summarize the treatment options for sJIA-LD, focusing on JAK inhibitors.
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