Desquamative interstitial pneumonia
Last reviewed: 23.04.2024
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[What causes desquamative interstitial pneumonia?
More than 90% of patients with desquamative interstitial pneumonia are smokers who have a tendency to develop the disease at the age of 30 to 40 years. The disease tends to have a uniform lesion of the lung parenchyma. The walls of the alveoli are lined with swollen pneumocytes of cuboidal form; there is a moderate infiltration of alveolar septa with lymphocytes, plasma cells and, sometimes, eosinophils; in more severe cases, moderate alveolar septal fibrosis develops. The most striking feature is the presence of numerous pigmented macrophages in the distal parts of respiratory spaces mistakenly mistaken for depleted pneumocytes in the initial description of the disease. "Honeycomb" is rare. Similar but much less pronounced changes are noted in interstitial lung disease associated with respiratory bronchiolitis (IBLARB), which supports the assumption that desquamative interstitial pneumonia and IBLARB are different variants of the same disease caused by smoking.
Symptoms of desquamative interstitial pneumonia
Symptoms of desquamative interstitial pneumonia, the results of lung function tests and the principles of diagnosis are identical to those in idiopathic pulmonary fibrosis.
Diagnosis of desquamative interstitial pneumonia
Changes in the roentgenogram of the chest are less pronounced than in idiopathic pulmonary fibrosis; the results of the study can be normal in 20% of cases. HRCT demonstrates focal subpleural darkening by the type of opaque glass, usually without amplification of the pulmonary pattern.
Treatment of desquamative interstitial pneumonia
Treatment of desquamative interstitial pneumonia, along with cessation of smoking, leads to clinical recovery in approximately 75% of patients; those who do not experience improvement may respond to glucocorticoid therapy or cytotoxic agents.
What prognosis does desquamative interstitial pneumonia have?
Desquamative interstitial pneumonia has a favorable prognosis; The ten-year survival rate is approximately 70%.