Restrictive Lung Diseases - Interstitial Pulmonary Fibrosis. Pathology, Clinical, Tests, Treatment
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http://bit.ly/2QsIwQ5This video presents the introduction to restrictive lung diseases. We will discuss:
Definition
Types
Extrapulmonary restrictive lung diseases
* Obesity
* Scoliosis
Pulmonary parenchymal restrictive lung diseases
* Idiopathic
* Autoimmune
* Granulomatous (Wagner's granulomatosis, Sarcoidosis)
* Occupational (pneumoconiosis, silicosis, asbestosis, coal, etc.)
* Other irritants
* Chronic infections (Tuberculosis)
Neuromuscular restrictive lung diseases
* Myasthenia gravis
* Diaphragm issues
* Polio
Cellular events/pathogenesis
* Damaged Type I Pneumocytes
* Hypertrophy of Type II Pneumocytes
* Irritated epithelium
* Irritated macrophages
* Apoptosis of epithelium
* Tissue damage leading to local inflammation and lung destruction/scaring.
* Neutrophils releasing cytokines, proteases, tumor growth factors (TGF)
* Macrophages releasing transforming growth factor beta, tumor necrosis factor (TNF), platelet activation factor.
* Fibroblasts overactivity resulting in more extracellular matrix and hardening.
* Epithelial-mesenchymal transformation.
* Smooth muscle proliferation.
* Respiratory interface thickening. Disrupted gaseous exchange.
Clinical:
Dry hacking cough
* Dyspnea
* Hypoxia
* Respiratory alkalosis
* Late inspiratory crackles
Abnormal chest X-Ray
* Reduced volume in chest X-Ray
* Lower lobes show more involvement and the reason why.
* Right heart failure/cor pulmonale
Lung Function Changes
* FEV1/FVC ratio increases above 80% (normal or above normal)
Treatment
* Anti-inflammatory (may not be very effective)
* TGF Beta-blockers
* Oxygen
* Lung transplant
...
https://www.youtube.com/watch?v=b9slTXwYMKw