Acute exacerbations of fibrotic interstitial lung disease

Andrew Churg, Joanne L. Wright, Henry D. Tazelaar

Research output: Contribution to journalArticle

49 Citations (Scopus)

Abstract

An acute exacerbation is the development of acute lung injury, usually resulting in acute respiratory distress syndrome, in a patient with a pre-existing fibrosing interstitial pneumonia. By definition, acute exacerbations are not caused by infection, heart failure, aspiration or drug reaction. Most patients with acute exacerbations have underlying usual interstitial pneumonia, either idiopathic or in association with a connective tissue disease, but the same process has been reported in patients with fibrotic non-specific interstitial pneumonia, fibrotic hypersensitivity pneumonitis, desquamative interstitial pneumonia and asbestosis. Occasionally an acute exacerbation is the initial manifestation of underlying interstitial lung disease. On biopsy, acute exacerbations appear as diffuse alveolar damage or bronchiolitis obliterans organizing pneumonia (BOOP) superimposed upon the fibrosing interstitial pneumonia. Biopsies may be extremely confusing, because the acute injury pattern can completely obscure the underlying disease; a useful clue is that diffuse alveolar damage and organizing pneumonia should not be associated with old dense fibrosis and peripheral honeycomb change. Consultation with radiology can also be extremely helpful, because the fibrosing disease may be evident on old or concurrent computed tomography scans. The aetiology of acute exacerbations is unknown, and the prognosis is poor; however, some patients survive with high-dose steroid therapy.

Original languageEnglish (US)
Pages (from-to)525-530
Number of pages6
JournalHistopathology
Volume58
Issue number4
DOIs
StatePublished - Mar 2011

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Interstitial Lung Diseases
Cryptogenic Organizing Pneumonia
Asbestosis
Extrinsic Allergic Alveolitis
Biopsy
Idiopathic Pulmonary Fibrosis
Connective Tissue Diseases
Acute Lung Injury
Adult Respiratory Distress Syndrome
Radiology
Pneumonia
Fibrosis
Referral and Consultation
Heart Failure
Steroids
Tomography
Wounds and Injuries
Infection
Pharmaceutical Preparations

Keywords

  • Acute exacerbation
  • Interstitial lung disease
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Histology
  • Pathology and Forensic Medicine

Cite this

Acute exacerbations of fibrotic interstitial lung disease. / Churg, Andrew; Wright, Joanne L.; Tazelaar, Henry D.

In: Histopathology, Vol. 58, No. 4, 03.2011, p. 525-530.

Research output: Contribution to journalArticle

Churg, Andrew ; Wright, Joanne L. ; Tazelaar, Henry D. / Acute exacerbations of fibrotic interstitial lung disease. In: Histopathology. 2011 ; Vol. 58, No. 4. pp. 525-530.
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