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Desquamative interstitial pneumonia
Last reviewed: 04.07.2025
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[ What causes desquamative interstitial pneumonia?
More than 90% of patients with desquamative interstitial pneumonia are smokers, who tend to develop the disease between the ages of 30 and 40 years. The disease tends to affect the lung parenchyma homogeneously. The alveolar walls are lined with distended, cuboidal pneumocytes; there is moderate infiltration of the alveolar septa by lymphocytes, plasma cells, and occasionally eosinophils; in more severe cases, moderate alveolar septal fibrosis develops. The most striking feature is the presence of numerous pigmented macrophages in the distal respiratory spaces, which were mistaken for desquamated pneumocytes when the disease was initially described. Honeycombing is rare. Similar but much less pronounced changes are observed in interstitial lung disease associated with respiratory bronchiolitis (IDLRB), which supports the assumption that desquamative interstitial pneumonia and IDLBP are different variants of the same disease caused by smoking.
Symptoms of desquamative interstitial pneumonia
The symptoms of desquamative interstitial pneumonia, pulmonary function test results, and diagnostic principles are identical to those of idiopathic pulmonary fibrosis.
Diagnosis of desquamative interstitial pneumonia
Chest radiographic changes are less dramatic than in idiopathic pulmonary fibrosis; findings may be normal in 20% of cases. HRCT shows focal subpleural ground-glass opacities, usually without enhancement of pulmonary markings.
Treatment of desquamative interstitial pneumonia
Treatment of desquamative interstitial pneumonia, along with smoking cessation, results in clinical recovery in approximately 75% of patients; those who do not improve may respond to glucocorticoid or cytotoxic therapy.
What is the prognosis for desquamative interstitial pneumonia?
Desquamative interstitial pneumonia has a favorable prognosis; 10-year survival is approximately 70%.