Intended for healthcare professionals

Clinical Review

The diagnosis and management of interstitial lung diseases

BMJ 2015; 350 doi: (Published 07 May 2015) Cite this as: BMJ 2015;350:h2072
  1. Adam Wallis, consultant thoracic radiologist,
  2. Katherine Spinks, consultant respiratory physician
  1. 1Queen Alexandra Hospital, Portsmouth PO6 3LY, UK
  1. Correspondence to: A Wallis adam.wallis{at}

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

The interstitial lung diseases comprise a complex group of pulmonary disorders principally affecting the pulmonary interstitium. The group includes idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, sarcoidosis, and connective tissue disease associated interstitial lung disease.

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.

Recent years have witnessed an expansion of research studies, large randomised controlled trials, and novel treatments in interstitial lung diseases with the potential to change the treatment landscape for patients. For some patients the aim will be to control symptoms, whereas for others it may be possible to stabilise disease or even achieve remission.

The aim of this review is to highlight the salient features in the history and examination that can suggest a specific interstitial lung disease, and …

View Full Text

Log in

Log in through your institution


* For online subscription