Diffuse Interstitial Disease Flashcards
A group of chronic disorders characterized by inflammation and fibrosis of the pulmonary interstitial tissue
diffuse interstitial disease
Known collectively as restrictive lung disease
diffuse interstitial disease
What are diffuse interstitial diseases caused by?
Many causes, poorly understood
Diffuse interstitial diseases are responsible for about __% of __________ pulmonary diseases.
Diffuse interstitial diseases are responsible for about 15% of noninfectious pulmonary diseases.
What are some fibrosing diseases? (7)
- Idiopathic pulmonary fibrosis
- Nonspecific interstitial pneumonia
- Cryptogenic organizing pneumonia
- Pulmonary fibrosis associated with connective tissue diseases
- Pneumoconiosis
- Drug reactions
- Radiation pneumonitis
What is a granumlomatous disease?
tuberculosis
What are types of diffuse interstitial disease?
- fibrosing dz
- granulomatous dz
- pulmonary eosinophilia
What is the pathogenesis of interstitial fibrosis?
- endothelial damage and activation
- fibroblast migration and proliferation, basement membrane disruption
- angiogenesis and myofibroblast foci formulation
- pulmonary fibrosis
chronic process of pulmonary fibrosis or scarring due to unknown causes
idiopathic pulmonary fibrosis
What is idiopathic pulmonary fibrosis cause by?
Caused by recurrent inflammation and repair in susceptible persons
With IPF, what is the cause the susceptible persons?
hereditary
What does IPF result in?
decreased lung compliance
What will IPF show with spirometry
decreased lung volume
What is IPF associated with?
- pulmonary HTN
- right HF
Does IPF occur abruptly or insidiously?
insidiously
What are the initial symptoms of IPF?
gradually increasing dyspnea on exertion and dry cough
What are the later symptoms of IPF?
- hypoxemia
- cyanosis
- clubbing
What is the typical age range for presentation with IPF?
40-70 yo
What are some general symptoms of IPF? (5)
- Shortness of breath
- Chronic dry, hacking coughing
- Fatigue and weakness
- Chest discomfort
- Loss of appetite and rapid weight loss
What are some CXR features of IPF?
- Reduced lung volume
- Reticular opacities
- Lower lobe and peripheral field predominance
- Honeycombing may be visible (fibrocystic appearance)
What imaging technique can you best se honeycombing?
better on CT versus xray
diseases that can involve the lung, resulting in different presentations including pulmonary interstitial fibrosis (other manifestations include vascular sclerosis, pneumonia, and bronchiolitis)
autoimmune connective tissue diseases
What are autoimmune connective tissue diseases associated with?
- pulmonary hypertension
- right HF
What are the causes of autoimmune connective tissues diseases? (3)
- Rheumatoid arthritis
- Systemic sclerosis (scleroderma)
- Lupus erythematosus
What percent of patients is rheumatoid arthritis the cause of autoimmune connective tissue diseases?
30-40%
Lung fibrosis in response to inhaled particles and aerosols
pneumoconiosis
With pneumoconiosis, ____________ ingest particles, die and release ________ contents and cytokines causing _______ without clearing ________ stimulus.
With pneumoconiosis, macrophages ingest particles, die and release lysosomal contents and cytokines causing fibrosis without clearing inflammatory stimulus.
What size are particles that are most likely to reach terminal alveoli?
1-5 microns
Pneumoconiosis has a similar pathology as which condition?
IPF
What are the causes of pneumoconiosis?
- mineral dust
- organic dust
- chemical fumes