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Published on: March 6, 2019
Pulmonary Manifestations of Rheumatic Diseases in Children
Mary M Buckley1, C Egla Rabinovich1
1Department of Pediatrics, Division of Rheumatology, Duke University Medical Center, Box 3212 DUMC, Durham, NC 27710, USA.
Insights
Children with rheumatic diseases need screening for lung issues, as they are often asymptomatic. Early detection of pulmonary involvement, especially in systemic-onset juvenile idiopathic arthritis, can improve outcomes and reduce mortality.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Clinical Medicine
Background:
- Rheumatic diseases in children can lead to rare but severe pulmonary manifestations.
- Pulmonary involvement is often asymptomatic in early stages, delaying diagnosis and treatment.
- Systemic-onset juvenile idiopathic arthritis is associated with a recognized high mortality lung disease.
Purpose of the Study:
- To highlight the importance of screening children with rheumatic diseases for pulmonary involvement.
- To identify risk factors for severe lung disease in systemic-onset juvenile idiopathic arthritis.
- To emphasize the need for early recognition and treatment of pediatric lung disease in rheumatic conditions.
Main Methods:
- Review of existing literature on pulmonary manifestations in pediatric rheumatic diseases.
- Analysis of identified risk factors associated with high mortality lung disease in systemic-onset juvenile idiopathic arthritis.
- Clinical observation and case study analysis (implied).
Main Results:
- Pulmonary manifestations in children with rheumatic diseases can cause significant morbidity and mortality.
- Key risk factors for fatal lung disease in systemic-onset juvenile idiopathic arthritis include early onset (<2 years), macrophage activation syndrome, trisomy 21, and anaphylaxis to biologics.
- Asymptomatic presentation is common, underscoring the need for proactive screening.
Conclusions:
- Screening for pulmonary involvement is crucial for children with rheumatic diseases.
- Early identification and management of lung disease in this population can improve patient outcomes.
- Understanding specific risk factors aids in targeted monitoring and intervention.
Abstract:
Children with rheumatic disease have rare pulmonary manifestations that may cause significant morbidity and mortality. These children are often clinically asymptomatic until disease has significantly progressed, so they should be screened for pulmonary involvement. There has been recent recognition of a high mortality-related lung disease in systemic-onset juvenile idiopathic arthritis; risk factors include onset of juvenile idiopathic arthritis less than 2 years of age, history of macrophage activation syndrome, presence of trisomy 21, and history of anaphylactic reaction to biologic therapy. Early recognition and treatment of lung disease in children with rheumatic diseases may improve outcomes.
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