This video presents the introduction to the restrictive lung diseases We will discuss:
Definition
- Types
- Extrapulmonary restrictive lung diseases
- 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 Presentation
- 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 > 80% (normal or above normal)
Treatment
- Anti-inflammatory (may not be very effective)
- TGF Beta-blockers
- Oxygen
- Lung transplant
Faculty
In addition to the presenter, following authors may have helped with the content writing, review,
or approval:
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Faculty |
Disclosures |
Dr. Mobeen Syed |
Author declares no conflict of interest. |
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2 Comments
iy393121@*.com
Oct 18 2024, 7:21 pm
Thanks!
abdullahkiller12@*.com
Oct 27 2019, 12:12 pm
Is it respiratory alkalosis or acidosis. I am little confused