Interstitial Lung Disease
In interstitial lung diseases (ILDs) the alveolar walls become thickened by accumulation of cells (e.g. inflammatory cells or fibroblasts) and extracellular matrix proteins (e.g. collagen). Idiopathic pulmonary fibrosis is the commonest ILD and is associated with a characteristic usual interstitial pneumonia (UIP) pattern of fibrosis on chest high-resolution CT scan. If the diagnosis is uncertain radiologically, lung biopsy may be required to make a diagnosis by clinical/radiological/pathological multidisciplinary consensus. It is important to distinguish IPF from other idiopathic ILDs, or ILD occurring in the context of connective tissue disease (CTD), drug reactions, or occupational dust exposure. Treatment is targeted at the underlying pathology, which may be primarily fibrotic (in IPF) or inflammatory (in certain CTD-ILD). Supportive measures and symptom palliation are important in all progressive ILDs. Lung transplantation is an option for selected patients.
KeywordsPulmonary fibrosis Fibroblast Autoimmunity Bronchoalveolar lavage Corticosteroids Immunosuppression Occupational lung disease Clinical trials Pulmonary function tests Asbestosis Hypersensitivity pneumonitis Connective tissue disease
- 1.American Thoracic Society/American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277–304.Google Scholar
- 2.Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–48.CrossRefPubMedPubMedCentralGoogle Scholar
- 4.Conway R, Low C, Coughlan RJ, O’Donnell MJ, Carey JJ. Methotrexate use and risk of lung disease in psoriasis, psoriatic arthritis, and inflammatory bowel disease: systematic literature review and meta-analysis of randomised controlled trials. BMJ. 2015;350:h1269.CrossRefPubMedPubMedCentralGoogle Scholar
- 5.Meyer KC, Raghu G, Baughman RP, Brown KK, Costabel U, du Bois RM, et al. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2012;185(9):1004–14.CrossRefPubMedGoogle Scholar