Interstitial Lung Disease Flashcards
What are idiopathic interstitial lung diseases?
Sarcoidosis
Idiopathic pulmonary fibrosis
What are drug induced interstitial lung diseases?
Methotrexate
Amiodarone
Bleomycin
What are pneumoconiosis interstitial lung diseases?
Asbestosis
Silicosis
Coal Worker’s Lung
What are Connective tissue disease
interstitial lung diseases?
Polymyositis
Dermatomyositis
SLE
Rheumatoid arthritis
Define idiopathic pulmonary fibrosis. When does it occur and what is the survival rate?
- Chronic inflammation of alveolar walls with progressive fibrosis
- Most commonly occurs between 50-70 yr
- 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?
- Accumulation of excessive fibrous or connective tissue in lung parenchyma
- Result of inflammatory, immune and fibrotic processes
- Inflammation is responsible for injury and fibrosis, distorts & impairs the structure and function of alveolar-capillary gas exchange surface
- Lungs become stiff and difficult to ventilate
- Diffusion capacity of alveoli-capillary membrane decreases hypoxemia
What causes need to be excluded to diagnose idiopathic pulmonary fibrosis?
- Environmental or occupational exposure
A. Coal dust, asbestos, silica, iron - Connective tissue diseases
A. Rheumatoid arthritis, SLE - Radiation-induced
- Drug-induced
A. Amiodarone, methotrexate, cocaine, heroin, chemo drugs
What are the sxs of interstitial lung disease?
- Dyspnea with rapid shallow breathing
- Dry hacking cough
- Fatigue
- Inspiratory rales on auscultation
- Clubbing
- Cyanosis
- Pulmonary HTN
What are the PFT results for interstitial lung disease>
↓ TLC
Normal FEV1/FVC
What are the CXR results for idiopathic pulmonary fibrosis?
- Diffuse interstitial lung disease with fibrosis and honeycombing (cystic opacities)
- Reticular/nodular opacities are hallmark
- 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?
- O2 to improve hypoxemia
- Corticosteroids to reduce inflammatory response
- Pulmonary rehab
- 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?
Respiratory failure Heart failure Pulmonary embolism Pneumonia Lung cancer