Current UK practice in pulmonary screening in paediatric connective tissue disease: UK multi-centre audit and...

Area of Science:

• Pediatric Rheumatology
• Pulmonology
• Medical Auditing

Background:

• Pulmonary manifestations in connective tissue diseases (CTDs) significantly contribute to patient morbidity and mortality.
• Early identification of lung involvement through screening is crucial for timely monitoring and effective management.
• Current practices for pulmonary screening in pediatric CTDs require standardization.

Purpose of the Study:

• To establish benchmarks for pulmonary screening protocols in pediatric CTDs.
• To evaluate current pulmonary screening practices within UK tertiary pediatric rheumatology centers.

Main Methods:

• A comprehensive review of national and international guidelines was conducted to define audit standards.
• Data collection involved five UK tertiary centers, including patients diagnosed with juvenile systemic lupus erythematosus (jSLE), juvenile dermatomyositis (jDM), juvenile systemic sclerosis (jSSc), or juvenile mixed connective tissue disease (jMCTD) within the past year.
• A total of 128 patients were included in the audit.

Main Results:

• Pulmonary involvement prevalence varied across CTDs: 39.7% in jSLE, 12.2% in jDM, 57.1% in jSSc, and 25.0% in jMCTD.
• Less than 50% of patients received regular assessments for pulmonary symptoms and signs.
• Inconsistent utilization of lung function tests and imaging for interstitial lung disease (ILD) at diagnosis and during follow-up suggests underestimation of pulmonary involvement.

Conclusions:

• The absence of universally accepted standards for pulmonary screening in juvenile CTDs results in practice variability among centers and clinicians.
• Implementing the proposed screening standards has the potential to enhance the recognition, monitoring, and management of pulmonary complications.
• Standardized practices will contribute to improved future guidance and research in the field.