Education And Debate

Fortnightly Review: Diffuse lung disease: an approach to management

BMJ 1994; 309 doi: http://dx.doi.org/10.1136/bmj.309.6948.175 (Published 16 July 1994) Cite this as: BMJ 1994;309:175
  1. R M Du Bois
  1. Royal Brompton Hospital, London SW3 6NP.

    The term diffuse (interstitial) lung disease embraces a large number of disorders characterised by distinct cellular and extracellular infiltrates in the acinar regions of the lung (that is, distal to the terminal bronchiole). Some of these diseases present acutely whereas others have a subacute or chronic course: the infiltrate may result in tissue injury, as in cryptogenic fibrosing alveolitis, or cause little damage to the lung architecture, as in pulmonary eosinophilia. Because diffuse lung disease particularly affects the peripheral part of the lung, chest radiographs usually show widespread shadows of various types, and in clinical practice this is the commonest starting point for diagnostic evaluation: a clinician is consulted by a (usually breathless) patient with a chest radiograph that shows widespread pulmonary shadows.

    About one in every 3000-4000 of the population in Britain has diffuse lung disease, and about 3000 people die each year from it.1 Cryptogenic fibrosing alveolitis is the most common diffuse lung disease and carries the worst prognosis: in all studies half of patients are dead within five years of presentation despite treatment, and only a quarter show an apparent response to treatment.*RF 1a4* In the United Kingdom mortality from fibrosing alveolitis is increasing and approaching 1500 a year.5

    Diffuse (interstitial) lung disease

    Cause known
    • Organic dusts (extrinsic allergic alveolitis)

    • Inorganic dusts (pneumoconioses)

    • Gases or fumes

    • Drugs or radiation

    • Infection

    Cause unknown
    • Cryptogenic fibrosing alveolitis

    • Interstitial lung disease associated with rheumatological diseases (such as rheumatoid arthritis and systemic sclerosis)

    • Granulomatous diseases (such as sarcoidosis and Langerhans' cell histiocytosis)

    • Neoplasia (such as lymphoproliferative diseases, metastases, and lymphangitis carcinomatosa)

    • Miscellaneous (such as amyloidosis and alveolar proteinosis)

    • Vasculitis

    • Inherited diseases (such as neurofibromatosis)

    Despite these disturbing figures, a recent survey of the management of fibrosing alveolitis in three regions in Britain revealed that few patients underwent investigations to confirm the diagnosis.6 Transbronchial biopsy …

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