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11 Cards in this Set
- Front
- Back
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Definition |
Rare, chronic, idiopathic. Scar tissue within the lungs and progressive dyspnoea.
Most common form of the group of interstitial (the tissue and space around the air sacs of the lungs) lung diseases |
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Epidemeology and aeitology |
Rare. Men > women. Increasing age.
Unknown but possible theory is that an unidentified insult causes damage to the alveolar epithelium, endothelium, and basement membrane.
Familial form (younger presentation) |
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Interstitial lung diseases |
Idiopathic Interstitial Pneumonias (IIP) = [1] Idiopathic Pulmonary Fibrosis (IPF) |
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Pathophysiology |
Pro-inflammatory and pro-fibrotic response that includes an influx of macrophages, fibroblasts, and other inflammatory cells >>> fibroblastic foci
Primary pathology involves deposition of ECM proteins by fibroblasts
Alveolar destruction, infiltration of the interstitial space with fibrosis
Honeycombing and bronchiectasis |
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Signs and symptoms |
Dyspnoea, cough and crackles. |
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Investigations |
Spirometry - Restrictive FEV1 and FEC < 80% but ration > 0.7 (maybe 0.8)
CXR - reticular opacities
HRCT - Honey combing and traction bronchiectasis ( pulmonary fibrosis pulls or distorts airways in ways that simulate bronchiectasis on imaging)
Biopsy - honeycombing |
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Treatment |
Ongoing - 1st line: antifibrotic therapy (perfenidone) >>> lung transplant
Acute: Hospitalisation + steroid
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Paracetamol |
COX 2 inhibitor. Reduced prostaglandins >>> raises pain threshold |
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DDx |
Lung cancer, TB, Bronchitis, other forms of fibrotic lung disease |
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Prognosis |
2-5 years but high variance. Can also lead to pulmonary hypertension and GORD |
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Anatomy |
Trachea > Bronchi > Lobar/2ndry bronchi > tertiraty/segmental which divide into bronchioles > terminal bronchioles > respiratory bronchioles |
