REstrictive lung disease Flashcards
Define Diffuse interstitial disease
Define:
–> heterogeneous group of disorders characterized by INFLAMMATION AND FIBROSIS of pulmonary connective tissue
–> principally the MOST peripheral and delicate interstitium in alveolar walls
Hallmark:
–> REDUCED COMPLIANCE (stiff lungs) –> dyspnea –> hypoxia
Describe the clinical presentation of interstitial lung diseases
Clinical
–> decreased lung capacity; FEV1/FVC ratio is NOT REDUCED
Presentation
–> dyspnea, hypoxia, end inspiratory crackles and eventually cyanosis
X-ray
–> diffuse bilateral infiltrative lesion by nodules, irregular lines, or ground glass shadows
Clinical course
–> may lead to pulmonary HPT or cor pulmonale (HONEYCOMB LUNG)
Acute lung injury (ALI) vs Acute respiratory distress syndrome (ARDS)
- Acute lung injury = abrupt onset of sifnificant hypoxema and pulmonary infiltrates in absence of cardial failure
- Acute respiratory distress = refers to SEVERE ALI; greater hypoxema
- BOTH produce diffuse alveolar damage and acute interstitial pneumonia
describe ARDS pathogenesis
- Uncontrolled activation of acute inflammatory system
–> activation of neutrophils leads to diffuse damage to alveolar capilalry walls
–> increased vascular permeability and alveolar thickening
–> loss of diffusion capacity
describe the clinical course of ARDS
1) normal chest x-ray
2) Symptoms = 1st dyspnea and tachypnea, then cyanosis and hypoxemia
3) Bilateral lung infiltrates develop
4) inhalation of NITRIC OXIDE HELPS; decrease PA pressure and arterial resistance. functional abnormalities not homogeneously distributed
describe clincial course of Idiopathic pUlmonary fibrosis
- 2/3 pts are > 60 years old (more frequent in men than women)
- insidious onset of SOB; nonproductive (dry) cough and increase dyspnea
- Advanced disease: Hypoxemia, cyanosis, clubbing
**DIAGNOSIS OF EXCLUSION**
IPF pathogenesis
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