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.
Fibrosis of lung, pleura,
cancer of lung, pleura.
-what pneumoconiosis?
Asbestosis
Pt has noncaseating granulomas in lung, hilar lymph nodes, systemic organs, and works in aerospace industry
-suspect what?
- Beryliosis!
- sounds just like sarcoidosis
anthracosis
collections of macrophages filled with carbon from inhalation. Very common in hilar lymph nodes, but mostly asymptomatic. Can be found in people that live in polluted cities.
Asbestosis
- complications, which is more common
- how to diagnose?
-“fibrosis of lung, pleura,
cancer of lung, pleura”
- lung cancer much more common than mesothelioma (pleura cancer)
- dx with histology: asbestos bodies in lung (long fibers)
sarcoidosis:
-how does it affect lung
- noncaseating granulomas in lung and hilar lymph nodes
- restrictive lung disease
idiopathic pulmonary fibrosis
-what is considered most likely mech?
-cyclical lung injury induces TGF-beta healing response from injured pneumocytes. This induces fibrosis.
what is the syndrome assoc with Coal worker’s pneumoconiosis?
Caplan syndrome:
combo of:
- diffuse interstitial fibrosis (‘black lung’)
- rhematoid arthritis
idiopathic pulmonary fibrosis
-make sure to rule out what things?
- drugs: (bleomycin, amiodarone)
- radiation therapy
sarcoidosis:
- common clinical presentations (4)
- progression of disease, tx
noncaseating granulomas throughout systemic organs. Common ones:
- lung fibrosis
- uveitis
- skin (cutaneous nodules, erythema nodosum)
- salivary, lacrimal glands (mimics Sjogrens)
- Tx with steroids. often resolves spontaneously w/o tx
restrictive lung disease:
2 major categories of mechanism
restricted filling of lung:
- interstitial disease
- chest wall abnormalities (eg obesity), so lungs cannot fill
Pt with lung granulomas and eosinophils
-what is this
Hypersensitivity pneumonitis
-granulomatous reaction to inhaled organic antigen (eg pigeon breeder’s lung)
Name the pneumoconioses:
- Coal workers’ pneumonconiosis
- silicosis
- berylliosis
- asbestosis
silicosis
- mech
- how presents
- silica impairs phagolysosome formation in macrophages. only pneumoconiosis that increases risk of TB infection
- fibrotic nodules in upper lobes of lung
sarcoidosis:
-classic population
black females
