Diffuse Parenchymal Lung Disease


Diffuse parenchymal lung diseases predominately affect the acinar regions of the lung. Some types of the disease are relatively common, while others are extremely rare, but taken together they affect roughly 1 in 3–4000 of the UK population and in many cases there is irreversible scarring.

Keywords: diffuse lung disease; diagnostic algorithm; bronchoalveolar lavage; high‐resolution computed tomography; radionuclide imaging; therapy

Figure 1.

Structures within the lung affected by diffuse parenchymal lung diseases. (a) A terminal respiratory unit – the acinus. (b) The alveolar–endothelial interface and ‘the interstitium’.

Figure 2.

Protocol for the investigation of diffuse parenchymal lung disease.



Agusti C, Xaubet A, Agusti AG et al. (1994) Clinical and functional assessment of patients with idiopathic pulmonary fibrosis: results of a 3 year follow‐up. European Respiratory Journal 7: 643–650.

Baughman RP and Fernandez M (1996) Radionuclide imaging in interstitial lung disease. Current Opinion in Pulmonary Medicine 2: 376–379.

Cooper JAD, White DA and Matthay RA (1986) Drug‐induced pulmonary disease. American Review of Respiratory Disease 133: 321–340, 488–505.

Drent M, van Nierop MA, Gerritsen FA, Wouters EF and Mulder PG (1996) A computer program using BALF‐analysis results as a diagnostic tool in interstitial lung diseases. American Journal of Respiratory and Critical Care Medicine 153: 736–741.

Foucher P, Camus P and the Groupe d’Etudes de la Pathologie Pulmonaire Iatrogène (1999) Pneumotox Online. Dijon, France: University Hospital.


Katzenstein AL and Myers JL (1998) Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. American Journal of Respiratory and Critical Care Medicine 157: 1301–1315.

Lynch JP and McCune WJ (1997) Immunosuppressive and cytotoxic pharmacotherapy for pulmonary disorders. American Journal of Respiratory and Critical Care Medicine 155: 395–420.

Muller NL and Miller RR (1990) Computed tomography of chronic diffuse lung disease. American Review of Respiratory Disease 142: 1206–1215, 1440–1448.

Raghu G (1995) Interstitial lung disease: a diagnostic approach. Are CT scan and lung biopsy indicated in every patient? American Journal of Respiratory and Critical Care Medicine 151: 909–914.

Rennard SI, Aalbers R, Bleeker E et al. (1998) Bronchoalveolar lavage: performance, sampling procedure, processing and assessment. European Respiratory Journal. Supplement 26: 13S–15S.

Venn GE, Kay PH, Midwood CJ and Goldstraw PJ (1985) Open biopsy in patients with diffuse pulmonary shadowing. Thorax 40: 931–935.

Wells AU, du Bois RM and Hansell DM (1994) Clinical utility of thin‐section CT in the diffuse interstitial lung diseases. Clinical Pulmonary Medicine 1: 154–164.

Wells AU, Hansell DM, Rubens MB et al. (1997) Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison. American Journal of Respiratory and Critical Care Medicine 155: 1657–1665.

Further Reading

Black CM and du Bois RM (1995) Organ involvement: pulmonary. In: Clements PJ and Furst DE (eds) Systemic Sclerosis, pp. 299–332. Baltimore: Williams & Wilkins.

du Bois RM (1994) Diffuse lung disease: an approach to management. British Medical Journal 309: 175–179.

du Bois RM (guest ed.) (1998) Pulmonary vascular disease. Seminars in Respiratory and Critical Care Medicine 19: 1–96.

du Bois RM and Turner‐Warwick M (1996) Diffuse parenchymal lung disease. In: Brewis RA, Corrin B, Geddes DM and Gibson GJ (eds) Respiratory Medicine, pp. 1365–1375. London: WB Saunders.

Kalassian KG, Doyle R, Kao P, Ruoss S and Raffin T (1997) Lymphangioleiomyomatosis: new insights. American Journal of Respiratory and Critical Care Medicine 155: 1183–1186.

King TE Jr (1998) Idiopathic pulmonary fibrosis. In: Schwartz MI and King TE Jr (eds) Interstitial Lung Diseases, pp. 597–644. Hamilton, ON, Canada: BC Decker.

Lynch JP and DeRemee RA (eds) (1991) Immunologically Mediated Pulmonary Diseases. Philadelphia, PA: JB Lippincott.

Newman‐Taylor AJ (1996) Extrinsic allergic alveolitis. In: Brewis RA, Corrin B, Geddes DM and Gibson GJ (eds) Respiratory Medicine, pp. 1394–1402. London: WB Saunders.

Tazi A and Hance AJ (1995) Pulmonary histiocytosis X. In: Walters EH and du Bois RM (eds) Immunology and Management of Interstitial Lung Diseases, pp. 305–318. London: Chapman and Hall.

Contact Editor close
Submit a note to the editor about this article by filling in the form below.

* Required Field

How to Cite close
Ranasinha, CD, and Bois, RM du(Apr 2001) Diffuse Parenchymal Lung Disease. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1038/npg.els.0002132]