Interstitial lung disease
Background
- General group of diseases that involve the lung parenchyma
- Grouped together because of similar clinical, physiologic, radiographic, or pathologic findings
- Specific diseases separated into ones with known causes and idiopathic
- Treatment and prognosis differs between specific diseases
- Examples of specific diseases: interstitial pneumonia, hypersensitivity pneumonitis, cryptogenic organizing pneumonia, acute interstitial pneumonitis, sarcoidosis, idiopathic pulmonary fibrosis
Clinical Features
- Symptoms: progressive exertional dyspnea, persistent nonproductive cough, hemoptysis, pleuritic chest pain
- History of occupational exposure: asbestosis, silicosis
- Abnormal chest imaging or pulmonary function tests
- Take detailed history making sure to focus on past medical history (connective tissue disease, inflammatory bowel disease, malignancy), smoking history, family history, medications, occupational and environmental exposures
- Physical exam is generally nonspecific
Differential Diagnosis
- Pneumonia
- Asbestosis
- Berylliosis
- Pulmonary edema
- Coal worker's pneumoconiosis
- Cryptogenic organizing pneumonia
- Drug induced pulmonary toxicity
- Farmer's lung
- Hypersensitivity pneumonitis
- Interstitial pulmonary fibrosis
- Collagen-vascular disease
- Lung malignancy
- Restrictive lung disease
- Sarcoidosis
- Silicosis
Evaluation
- Work up and test results differ between types of interstitial lung diseases. Below are common studies obtained to determine disease. See page for specific disease for specific labs/imaging and findings.
- CBC/CMP
- Serologic studies: ANA, Rheumatoid factor
- Imaging: Chest x-ray, chest CT
- Pulmonary function tests
- Cardiac ECHO
- Bronchoalveolar lavage
- Lung biopsy
Management
- Management differs between specific types of interstitial lung diseases. This is why it is important to determine specific cause of disease. See specific disease page for management.
Disposition
- Depending on how stable the patient is, patient can be admitted for work up or discharged for outpatient work up.
- Important to determine acute vs chronic symptoms.
See Also
- Sarcoidosis
- Idiopathic pulmonary fibrosis
References
- <Bradley B, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 Suppl 5:v1./>
- <Pipavath S, Godwin JD. Imaging of interstitial lung disease. Clin Chest Med 2004; 25:455./>
- <Martinez FJ, Flaherty K. Pulmonary function testing in idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3:315./>
- <Schwartz M, King Jr TE. Interstitial Lung disease, 5th ed, People's Medical Clearing House, Shelton, CT 2011./>
- <Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183:788./>
- <Garcia CK, Raghu G. Inherited interstitial lung disease. Clin Chest Med 2004; 25:421./>
