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

Objectives Pulmonary manifestations of connective tissue diseases (CTDs) are associated with morbidity and mortality. Early recognition through screening ensures appropriate monitoring and management. The aim of this audit was to define standards for pulmonary screening in CTDs and assess practice in UK tertiary paediatric rheumatology centres. Methods Relevant national and international guidelines were identified and reviewed to inform audit standards. Data was collected from five UK centres. Patients with a diagnosis of juvenile systemic lupus erythematosus (jSLE), juvenile dermatomyositis (jDM), juvenile systemic sclerosis (jSSc) or juvenile mixed connective tissue disease (jMCTD) seen within the last 12 months were included. Results A total of 128 patients met inclusion criteria and identified pulmonary involvement varied between the CTDs; 27/68 (39.7%) in jSLE, 5/41 (12.2%) in jDM, 4/7 (57.1%) in jSSc and 3/12 (25.0%) in jMCTD. Pulmonary involvement is likely to be under-estimated due to inconsistent screening with ≤50% of patients having regular assessment of pulmonary symptoms and signs during follow-up and inconsistent use of lung function testing and imaging for interstitial lung disease (ILD) both at diagnosis and during follow-up. Conclusion There are no universally recognised standards on pulmonary screening in juvenile connective tissue diseases leading to inconsistency across centres and between practitioners. However, adoption of the proposed standards would likely improve recognition, monitoring and management of pulmonary manifestations, standardise practice and inform and improve future guidance and research.