Interstitial Lung Disease

  • Simon P. Hart


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.


Pulmonary fibrosis Fibroblast Autoimmunity Bronchoalveolar lavage Corticosteroids Immunosuppression Occupational lung disease Clinical trials Pulmonary function tests Asbestosis Hypersensitivity pneumonitis Connective tissue disease 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Respiratory Research GroupHull York Medical School, Castle Hill HospitalCottinghamUK

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