Clinical care in interstitial lung disease: a critical appraisal of clinical guidance documents

Interstitial lung diseases (ILDs) are complex, requiring multifaceted care for optimal management of patients' symptoms and health outcomes. This systematic review evaluated the content coverage of currently available clinical guidance documents ILD.

A systematic search was performed to identify clinical guidance documents published between 2011 and March 2025 in the Embase, Ovid Medline and Trip databases. Document characteristics, quality and contents covered were independently assessed by two reviewers.

A total of 79 ILD clinical guidance documents were identified, with clinical practice guidelines (n=50) having superior quality based on the Institute of Medicine standards. The content of most documents (84%) focused on ILD aetiology, with connective tissue disease (44%) being the most discussed. Only 46% of documents covered pulmonary manifestations, which often encompassed pulmonary hypertension (30%) and hypoxaemia (28%). Extrapulmonary morbidities were covered in 28% of documents, with gastro-oesophageal reflux disease (23%) and obstructive sleep apnoea (10%) being commonly presented. Behavioural and lifestyle factors were covered in 34% of documents, with most addressing physical inactivity (30%). Additionally, 51% of documents covered overall diagnostic approach for ILD, 35% lung transplantation, 22% acute exacerbations and 19% palliative care.

Despite growing awareness of ILD, most clinical guidance documents have limited coverage for domains of patient care outside of diagnosis and pharmacotherapies. Future clinical guidance documents on ILD should address the content gaps to deliver comprehensive care for patients with ILD, with engagement of different stakeholders from various regions.