Restrictive pulm diseasesw Flashcards
Restrictive pulmonary disease
- reduced expansion of lung parenchyma
- decreased lung capacity
- decreased compliance
- reduction of both fev and fvc
- fvc is normal
Idiopathic Pulmonary fibrosis
- Chronic, progrressive fibrosing of interlobular, subpleural lung, cobblestone pleural
- Cyclical lung injury
- 55 to 70
- rales with unknown onset
- tgfBETA
- transplant is only treatment
- Bleomycin and amidarone cause it as well as radiation therapy.
Cryotogenic Organizing pneumonia
- Polyploid plugs of loose organizing connective tissue in the small airways, alveolar ducts
- surrounded by inflammation
- Treat with roids
Pneumonconiosis: Asbestos
M* eats asbestos but cant degrade –>pleural plaques and effusions with parenchymal fibrosis
-Has golden brown beaded rods with translucent centers and iron coating
Crystal tile: more common less pathgoenic,
Amphibole: stiff and straight go into lungs.
-Greece or other exposure
Pneumonconiosis: silicosis
Berilium is also a thing for aerospace people
- Inhaled silicon dioxide activates m*
- AA stone cutters
- Fibrotic nodule with increased susceptibility to TB
Sarcoidosis :
- Cell mediated immune response to unknown insult
- Becomes non caseating granuloma with asteroid /Schaumann body especially in AA women.
- High ACE and calcium
- Eye and skin lesions occur
- CXR shows bilateral lymphadenopathy
Pulmonary Langerhans cells histiocytosis/Eosinphilic granuloma
- Proliferation of Langerhans cell with eosinophils
- Tennis racket shaped birbeck granule
- Nodules on imaging
- Smoking adult
- Pneumothorax
Pulmonary Alveolar Proteinosis: Cause
Accumulation of acellular surfactant in intra alveolar and bronchial spaces
-Autoimmine to anti GM-CSF –> loss of alveoli to catabolize surfactant
Pulmonary Alveolar Proteinosis: Presentation
Granular material and histiocytes with alveolar
-Bilateral pathy asymmetric pulmonary OPACITY
-30-50 year old men
Treat with lung lavage
Respiratory Bronchiolitis
- Accumlation of intra-alveolar M* leading to chronic inflammation of bronchioles in 30-40 year old smokers.
- Treat with roids
- CXR bilateral reticulonodular infiltrates with ground glass appearance