The diagnosis and management of interstitial lung diseases

The bottom line

• The interstitial lung diseases are a complex group of disorders, but idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, connective tissue disease associated interstitial lung disease, and sarcoidosis make up the majority seen in clinical practice

• Patients present with non-specific signs and symptoms and so a high index of suspicion is required

• Typical findings of bi-basal crepitations can be mistaken for pulmonary oedema, but patients usually lack other features of this; a lack of response to treatment for oedema or infection should raise suspicion of interstitial lung disease

• Early referral to specialist services enables prompt diagnosis and management to optimise outcome, and this process can be facilitated by investigations in the community, including chest radiography and some serology

• Symptom control, occupational therapy, and palliative care are also important aspects of managing patients with interstitial lung disease, requiring input from multiple agencies and specialists

Sources and selection criteria

We carried out an electronic search of Medline and Embase for relevant original papers and systematic reviews using the search terms “pulmonary fibrosis”, “interstitial lung disease”, and “diffuse parenchymal lung disease”, prioritising those published from 2010. Relevant articles from the Cochrane databases and personal references were also included.