Desquamative interstitial pneumonia is a type of idiopathic interstitial pneumonia Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic... read more . The vast majority of adult patients with desquamative interstitial pneumonia are smokers, who tend to develop the disease in their 30s or 40s.
The disease tends to affect the lung parenchyma uniformly. The alveolar walls are lined with plump cuboidal pneumocytes; there is moderate infiltration of the alveolar septum by lymphocytes, plasma cells, and, occasionally, eosinophils. Alveolar septal fibrosis, if present, is mild.
The most striking feature is the presence of numerous pigmented macrophages within distal airspaces, mistaken as desquamated pneumocytes when the disease was first described. Honeycombing is rare. Similar but much less extensive findings occur in respiratory bronchiolitis–associated interstitial lung disease Respiratory Bronchiolitis–Associated Interstitial Lung Disease Respiratory bronchiolitis–associated interstitial lung disease (RBILD) is a syndrome of small airway inflammation and interstitial lung disease occurring in smokers. Symptoms include cough and... read more (RBILD), leading to the suggestion that desquamative interstitial pneumonia and RBILD are different manifestations of the same disease caused by cigarette smoking.
Desquamative interstitial pneumonia manifests with gradually increasing dyspnea and nonproductive cough.
Diagnosis of Desquamative Interstitial Pneumonia
High-resolution CT (HRCT)
Sometimes surgical lung biopsy
Chest x-ray can show bibasilar hazy opacities without honeycombing, but is normal in up to 20% of cases of desquamative interstitial pneumonia. HRCT shows multifocal or diffuse, basilar, subpleural ground-glass opacities. Cysts may be present, often in areas of ground-glass opacity. Irregular linear and reticular opacities are common but are not usually the dominant features. Honeycombing may be visible, occurs in the minority of patients, and is usually limited.
Surgical lung biopsy is sometimes necessary.
Treatment of Desquamative Interstitial Pneumonia
Sometimes corticosteroids or cytotoxic drugs
Smoking cessation Smoking Cessation Most smokers want to quit and have tried doing so with limited success. Effective interventions include cessation counseling and drug treatment, such as varenicline, bupropion, or a nicotine... read more results in clinical improvement in an estimated 75% of patients with desquamative interstitial pneumonia. Patients who do not improve may respond to corticosteroids or cytotoxic drugs.
Prognosis is good, with about 70% survival at 10 years.