9.5 Restrictive diseases Flashcards
sarcoidosis in lung:
-histology. what’s characteristic?
- noncaseating granulomas, made of epitheloid histiocytes
- characteristic ‘asteroid bodies’ seen inside giant cells of granulomas
“pigeon breeder’s lung”
ex of Hypersensitivity pneumonitis.
beryliosis
- how presents
- what populations
- increased risk for what complication
- pneumoconiosis caused by inhalation of beryllium–aerospace industry, beryllium miners
- noncaseating granulomas in lung, hilar lymph nodes, systemic organs (just like sarcoidosis!)
- increase risk of lung cancer
Asbestosis
-what population
-construction workers, plumbers, shipyard workers
sarcoidosis:
-general mech, etiology
- etiology unknown,
- probably a T-cell response to unknown antigen
- noncaseating granulomas in systemic organs
what 2 drugs are notorious for causing interstitial fibrosis as side effect
amiodarone
bleomycin
sarcoidosis in lung:
-clinical presentation (3)
- dyspnea, cough
- elevated serum ACE
- hypercalcemia (increased 1A OHlase)
sarcoidosis:
-what electrolyte concentration is altered in blood? why?
- hypercalcemia
- noncaseating granulomas: the epitheloid histiocytes have 1-alpha-OHlase activity to convert Vit D to active form.
- you see this in berylliosis too
sarcoidosis:
what enzyme is increased in blood?
elevated serum ACE
what is the only pneumoconioses that increases risk of TB?
silicosis
Hypersensitivity pneumonitis
- clinical presentation
- complication
- mech
- fever, cough, dyspnea hours after exposure to inhaled organic antigen. Resolves with removal, but chronic leads to interstitial fibrosis
- inhaled organic antigen causes granulomatous reaction. Eosinophils involved.
Berylliosis
-what electrolyte concentration is altered in blood? why?
- hypercalcemia
- noncaseating granulomas: the epitheloid histiocytes have 1-alpha-OHlase activity to convert Vit D to active form.
- you see this in sarcoidosis too
Coal miner pt with lung problems:
-what other problems to look for
- Rheumatoid arthritis
- (Caplan syndrome–“rheumatoid pneumoconiosis”)
idiopathic pulmonary fibrosis
- progression of disease
- tx
- initially fibrosis seen on CT in subpleural patches
- then, results in diffuse fibrosis with end-stage ‘honeycomb’ lung
- tx: lung transplant
pneumoconioses
- what is this
- mech of disease
- interstitial disease caused by long-term inhalation of small particles in occupational exposure
- small particles are not removed by mucus and enter alveoli, where macrophages eat them. Macrophages don’t know what to do with these foreign particles and induce fibrosis of lung.