Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study

Alexandra J Rice¹, Athol U Wells, Demos Bouros

¹Department of Histopathology, Royal Brompton Hospital, London, England.

|May 23, 2003

Summary

Acute exacerbations of idiopathic pulmonary fibrosis (IPF) are rare terminal events. This study found IPF exacerbations often occur with usual interstitial pneumonia patterns, suggesting it may be a more common outcome than previously recognized.

Area of Science:

Pulmonary Medicine
Pathology
Interstitial Lung Diseases

Background:

Acute exacerbations of idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis (IPF/CFA) are rare, terminal events with unknown underlying causes.
The relationship between IPF exacerbations and specific patterns of idiopathic interstitial pneumonias (IIPs) is not well understood.

Purpose of the Study:

To investigate the underlying histological patterns associated with acute exacerbations in patients with idiopathic pulmonary fibrosis and fibrosing alveolitis with connective tissue disorders (FA-CTDs).
To explore potential causes and frequency of acute exacerbations in IPF/CFA.

Main Methods:

Retrospective review of autopsy materials from patients who died with diffuse alveolar damage in the context of pulmonary fibrosis.
Comparison of cases with idiopathic interstitial pneumonias and acute interstitial pneumonia.

Main Results:

Of 15 patients with acute exacerbations (12 IPF/CFA, 3 FA-CTD), 12 showed usual interstitial pneumonia (UIP) and 3 showed fibrotic nonspecific interstitial pneumonia (NSIP).
All fibrotic NSIP cases were associated with FA-CTDs.
Toxic oxygen effects and infection were deemed unlikely causes for exacerbations.

Conclusions:

Acute exacerbations of IPF/CFA may be a more frequent terminal event than previously recognized.
Usual interstitial pneumonia is a common background pattern in IPF exacerbations.
The exact cause of exacerbations in FA-CTD patients remains uncertain, potentially linked to fibrosis, CTD, or both.

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Background:

Purpose of the Study:

Main Methods:

Main Results:

Conclusions:

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