Harrison's Manual of Medicine
Chapter 136: Interstitial Lung Disease
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Interstitial lung diseases (ILDs) are a group of >200 disease entities characterized by diffuse lung parenchymal abnormalities. ILDs can be classified into two major groups: (1) diseases associated with predominant inflammation and fibrosis, and (2) diseases with predominantly granulomatous reaction in interstitial or vascular areas (Table 136-1). ILDs are nonmalignant and noninfectious, and they are typically chronic. The differential diagnosis of ILDs often includes infections (e.g., atypical mycobacteria, fungi), congestive heart failure, and malignancy (e.g., bronchoalveolar cell carcinoma). One of the most common ILDs associated with a granulomatous reaction, sarcoidosis, is discussed in Chap. 169: Sarcoidosis. Other common ILDs include idiopathic pulmonary fibrosis (IPF) and ILDs related to connective tissue diseases. Many ILDs are of unknown etiology; however, some ILDs are known to be associated with specific environmental exposures including asbestos, radiation therapy, and organic dusts.
| Lung response: Alveolitis, interstitial inflammation, and fibrosis | |
|---|---|
| Known Cause | |
| Asbestos | Radiation |
| Aspiration pneumonia | |
| Drugs (e.g., nitrofurantoin, amiodarone, methotrexate, rituximab) and chemotherapy drugs | Residual of adult respiratory distress syndrome |
Smoking-related Desquamative interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung disease Langerhans cell granulomatosis (eosinophilic granulomatosis of the lung) | |
| Unknown Cause | |
| Idiopathic interstitial pneumonias | Pulmonary alveolar proteinosis |
| Idiopathic pulmonary fibrosis (usual interstitial pneumonia) | Lymphocytic infiltrative disorders (lymphocytic interstitial pneumonitis associated with connective tissue disease) |
| Eosinophilic pneumonias | |
| Acute interstitial pneumonia (diffuse alveolar damage) | Lymphangioleiomyomatosis
Amyloidosis |
Cryptogenic organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) Nonspecific interstitial pneumonia | Inherited diseases Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher disease, Hermansky-Pudlak syndrome |
Connective tissue diseases Systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren syndrome, polymyositis-dermatomyositis | Gastrointestinal or liver diseases (Crohn disease, primary biliary cirrhosis, chronic active hepatitis, ulcerative colitis)
Graft-versus-host disease (bone marrow transplantation; solid organ transplantation) |
Pulmonary hemorrhage syndromes Goodpasture syndrome, idiopathic pulmonary hemosiderosis, isolated pulmonary capillaritis | |
| Lung response: Granulomatous | |
| Known Cause | |
| Hypersensitivity pneumonitis (organic dusts) | Inorganic dusts: beryllium, silica |
| Unknown Cause | |
| Sarcoidosis | Bronchocentric granulomatosis |
| Granulomatous vasculitides | Lymphomatoid granulomatosis |
| Granulomatosis with polyangiitis (Wegener’s), allergic granulomatosis of Churg-Strauss | |
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Interstitial lung diseases (ILDs) are a group of >200 disease entities characterized by diffuse lung parenchymal abnormalities. ILDs can be classified into two major groups: (1) diseases associated with predominant inflammation and fibrosis, and (2) diseases with predominantly granulomatous reaction in interstitial or vascular areas (Table 136-1). ILDs are nonmalignant and noninfectious, and they are typically chronic. The differential diagnosis of ILDs often includes infections (e.g., atypical mycobacteria, fungi), congestive heart failure, and malignancy (e.g., bronchoalveolar cell carcinoma). One of the most common ILDs associated with a granulomatous reaction, sarcoidosis, is discussed in Chap. 169: Sarcoidosis. Other common ILDs include idiopathic pulmonary fibrosis (IPF) and ILDs related to connective tissue diseases. Many ILDs are of unknown etiology; however, some ILDs are known to be associated with specific environmental exposures including asbestos, radiation therapy, and organic dusts.
| Lung response: Alveolitis, interstitial inflammation, and fibrosis | |
|---|---|
| Known Cause | |
| Asbestos | Radiation |
| Aspiration pneumonia | |
| Drugs (e.g., nitrofurantoin, amiodarone, methotrexate, rituximab) and chemotherapy drugs | Residual of adult respiratory distress syndrome |
Smoking-related Desquamative interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung disease Langerhans cell granulomatosis (eosinophilic granulomatosis of the lung) | |
| Unknown Cause | |
| Idiopathic interstitial pneumonias | Pulmonary alveolar proteinosis |
| Idiopathic pulmonary fibrosis (usual interstitial pneumonia) | Lymphocytic infiltrative disorders (lymphocytic interstitial pneumonitis associated with connective tissue disease) |
| Eosinophilic pneumonias | |
| Acute interstitial pneumonia (diffuse alveolar damage) | Lymphangioleiomyomatosis
Amyloidosis |
Cryptogenic organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) Nonspecific interstitial pneumonia | Inherited diseases Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher disease, Hermansky-Pudlak syndrome |
Connective tissue diseases Systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren syndrome, polymyositis-dermatomyositis | Gastrointestinal or liver diseases (Crohn disease, primary biliary cirrhosis, chronic active hepatitis, ulcerative colitis)
Graft-versus-host disease (bone marrow transplantation; solid organ transplantation) |
Pulmonary hemorrhage syndromes Goodpasture syndrome, idiopathic pulmonary hemosiderosis, isolated pulmonary capillaritis | |
| Lung response: Granulomatous | |
| Known Cause | |
| Hypersensitivity pneumonitis (organic dusts) | Inorganic dusts: beryllium, silica |
| Unknown Cause | |
| Sarcoidosis | Bronchocentric granulomatosis |
| Granulomatous vasculitides | Lymphomatoid granulomatosis |
| Granulomatosis with polyangiitis (Wegener’s), allergic granulomatosis of Churg-Strauss | |
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