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

• Published in: American journal of clinical pathology Vol. 119; no. 5; pp. 709 - 714
• Main Authors: RICE, Alexandra J, WELLS, Athol U, BOUROS, Demos, DU BOIS, Roland M, HANSELL, David M, POLYCHRONOPOULOS, Vlasis, VASSILAKIS, Dimitris, KERR, Jonathan R, EVANS, Timothy W, NICHOLSON, Andrew G
• Format: Journal Article
• Language: English
• Published: Chicago, IL American Society of Clinical Pathologists 01.05.2003
• Subjects: Aged; Autopsy; Biological and medical sciences; Connective Tissue Diseases - pathology; Female; Humans; Lung Diseases, Interstitial - pathology; Male; Medical sciences; Middle Aged; Pneumology; Pulmonary Alveoli - pathology; Pulmonary Fibrosis - pathology; Respiratory system : syndromes and miscellaneous diseases; Diffuse; Human; Lung disease; Usual interstitial pneumonia; Respiratory disease; Pathogenesis; Acute interstitial pneumonia, Autopsy; Lung; Terminal stage; Diffuse alveolar damage; Pathology; Autopsy; Fibrosis; Interstitial pneumonitis
• ISSN: 0002-9173; 1943-7722
• DOI: 10.1309/UVARMDY8FE9FJDKU

Acute exacerbations of idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis (IPF/CFA) are rare and typically terminal events, but their relationship to underlying patterns of idiopathic interstitial pneumonias is unknown. We reviewed autopsy material from patients who died of diffuse alveolar damage in the clinical setting of pulmonary fibrosis, both idiopathic and with background fibrosing alveolitis with connective tissue disorders (FA-CTDs), and compared them with cases of acute interstitial pneumonia. Of 15 patients with acute exacerbations of IPF/CFA (n = 12) or FA-CTD (n = 3), 12 had a background pattern of usual interstitial pneumonia and 3 had fibrotic nonspecific interstitial pneumonia. All cases of fibrotic nonspecific interstitial pneumonia were seen in association with FA-CTD. The cause of acute exacerbations is unknown, but our data suggest that toxic effects of oxygen and triggering infection are unlikely causes. In patients with CTDs, it remains uncertain whether the acute exacerbation is related to the fibrosis, the associated CTD, or a combination of these factors. Acute exacerbations of IPF/CFA may be a more common terminal event than previously thought.