Flashcards in Interstitial Lung Disease Deck (42):
What are idiopathic interstitial lung diseases?
Idiopathic pulmonary fibrosis
What are drug induced interstitial lung diseases?
What are pneumoconiosis interstitial lung diseases?
Coal Worker’s Lung
What are Connective tissue disease
interstitial lung diseases?
Define idiopathic pulmonary fibrosis. When does it occur and what is the survival rate?
1. Chronic inflammation of alveolar walls with progressive fibrosis
2. Most commonly occurs between 50-70 yr
3. Survival is 3-5 years after diagnosis
What are other names for idiopathic pulmonary fibrosis?
Interstitial lung disease (ILD)
Cryptogenic fibrosing alveolitis
Diffuse interstitial fibrosis
Idiopathic interstitial pneumonitis
What is the pathophys of idiopathic pulmonary fibrosis?
1. Accumulation of excessive fibrous or connective tissue in lung parenchyma
2. Result of inflammatory, immune and fibrotic processes
3. Inflammation is responsible for injury and fibrosis, distorts & impairs the structure and function of alveolar-capillary gas exchange surface
4. Lungs become stiff and difficult to ventilate
5. Diffusion capacity of alveoli-capillary membrane decreases hypoxemia
What causes need to be excluded to diagnose idiopathic pulmonary fibrosis?
1. Environmental or occupational exposure
A. Coal dust, asbestos, silica, iron
2. Connective tissue diseases
A. Rheumatoid arthritis, SLE
A. Amiodarone, methotrexate, cocaine, heroin, chemo drugs
What are the sxs of interstitial lung disease?
1. Dyspnea with rapid shallow breathing
2. Dry hacking cough
4. Inspiratory rales on auscultation
7. Pulmonary HTN
What are the PFT results for interstitial lung disease>
What are the CXR results for idiopathic pulmonary fibrosis?
1. Diffuse interstitial lung disease with fibrosis and honeycombing (cystic opacities)
2. Reticular/nodular opacities are hallmark
3. usually bilateral
What are the CT results for iinterstitial lung disease?
Patchy distribution of ground glass in lung parenchyma
What are the surgical lung biopsy results in interstitial lung disease?
Mixed areas of normal tissue, interstitial inflammation, fibrosis and honeycombing
What are the treatment options for idiopathic pulmonary fibrosis? What is definitive treatment?
1. O2 to improve hypoxemia
2. Corticosteroids to reduce inflammatory response
3. Pulmonary rehab
4. Lung transplantation is definitive treatment
A. 5 yr survival rate estimated at 50%
What si the prognosis for idiopathic pulmonary fibrosis? Who has a better prognosis?
Usually poor response to treatment
Best prognostic factors include:
Young female with less than average dyspnea and hypoxemia
No smoking history
Positive response to corticosteroids
What are the complications for idiopathic pulmonary fibrosis?
Chronic restrictive lung disease caused by the inhalation of dust or various inert, inorganic or silicate dusts
Clinically important diseases include coal worker’s lung, silicosis, asbestosis, siderosis
When does silicosis occur?
1. Follows long term inhalation of small particles of silica
2. Associated with:
Metal mining (Pb, Cu, Ag, Au),
Sandstone & granite cutting
What is the pathophys of silicosis?
Alveolar macrophages engulf inhaled particles enter lymphatics and interstitial tissue
Macrophages cause cytotoxic enzymes to be released fibrosis of lung parenchyma
When macrophage dies, particles are released and engulfed by other macrophages process keeps repeating
What is the clinical presntation of simple cases of silicosis?
20-30 years exposure necessary before disease becomes apparent
What is the clinical presntation of complicated cases of silicosis?
Dyspnea, rales, clubbing, cyanosis
Related to amount of exposure
What are the cxr results of silicosis?
Small nodular opacities at upper lung fields
Calcified hilar lymph nodes
“eggshell” calcification strongly suggests silicosis
What is the treatment for silicosis?
No effective treatment
What is the prophylaxis for silicosis?
Effective dust control
External hoods & venting of dust
Surveillance of exposed workers with CXR
Frequency depends on type & amt of exposure
What are the complications of silicosis?
Increased risk of TB
Function of alveolar macrophages can be permanently compromised
Define coal workers lung
Diffuse nodular deposition of dust in lungs
Results from long term exposure to coal dust in coal mining
What is the patho phys of coal miners disease?
Coal dust is widely distributed throughout lung development of coal macules around bronchioles
Differentiate between simple and complicated coal miners disease
Mild dilation of bronchioles
Not associated with airflow obstruction
PFT’s not impressive
Progressive massive fibrosis develops as amorphous black mass that compromises and destroys airways and vascular beds
Differentiate between the sxs of simple and complicated coal miners disease
Underground exposure of 20 years before sx’s start to develop
Dyspnea on exertion
What is the treatment for coal miners disease?
No effective treatment
What is the prophylaxis for coal miners disease?
Suppress coal dust for prevention
What are the complications of coal miners disease?
Progressive massive fibrosis
Diffuse interstitial lung disease that results from long term inhalation of asbestos dust
Removal of asbestos products
Asbestosis is a promoter of carcinogenesis
What is the pathophys of asbestosis?
1. Asbestos fibers are inhaled deep into lung parenchyma
2. Deposits retained in lung tissue → alveolar and interstitial fibrosis
Slowly progressive, diffuse pulmonary fibrosis
What are the effects of asbestosis?
Asbestosis causes reduction in
Differentiate between the early and late presentation of asbestosis
1. Insidious onset
↓ exercise tolerance
When do most ppl seek treatment for asbestosis?
Most seek medical attention 15 or > years after exposure
What are the CXR results for asbestosis?
Diffusely distributed linear opacities/streaking at lung bases
Calcified plaques on diaphragm or lateral chest wall (pleural plaques)
Pleural involvement is Hallmark
What is the prophylaxis for asbestosis?
Dust suppression in work environment / Abatement teams
What is the supportive treatment for asbestosis?
Prompt tx of resp infections
What increases the risk of complications from asbestosis?