Pathoma: Restrictive Diseases Flashcards
What is pneumoconiosis?
interstitial fibrosis due to chronic exposure to small, fibrogenic particles
Why do sarcoid pts have hypercalcemia?
the granulomas have 1-alpha-hydroxylase activity, which can activate vitamin D
What other tissues, besides the lungs, are commonly affected by sarcoidosis?
- uvea (Uveitis)
- skin (cutaneous nodules or erythema nodosum)
- salivary and lacrimal glands
- any other tissue
What is hypersensitivity pneumonitis?
a granulomatous reaction to inhaled organic antigens, esp from birds
Where do sarcoid granulomas occur in the lung?
in the hilar lymph nodes and throughout the lung tissue
What cells are assoc. with a granulomatous reaction?
eosinophils
What happens in idiopathic pulmonary fibrosis?
fibrosis of the lung interstitium
Pts exposed to asbestos are much more likely to get ____ than _____.
lung CA; mesothelioma
This is a systemic disease characterized by noncaseating granulomas in multiple organs.
sarcoidosis
What are the s/s of hypersensitivity pneumonitis?
- hours after exposure: fever, cough, dyspnea
- chronic: interstitial fibrosis
What workers are at risk of silicosis pneumoconiosis?
sandblasters and silica miners
Granulomas in the lung cause _____.
restrictive lung dz
What does non-caseating mean?
all the cells are alive; no necrosis
In sarcoid patients, granulomas have 1-alpha-hydroxylase activity, which can activate vitamin D. What does this cause?
hypercalcemia
What doe TGF-beta do?
induces a healing response and fibrosis
What are the clinical features of sarcoidosis?
cough, SOB, elevated serum ACE, hypercalcemia
What lung function values will be indicative of restrictive lung disease?
- decreased TLC
- decreased FVC
- decreased FEV1
- increased FEV1:FVC ratio (>80%)
This is a giant cell in sarcoidosis with an internal star-like pattern.
an asteroid body
Restrictive lung diseases cause _____ compliance.
decreased
What workers are at increased risk of asbestosis pneumoconiosis?
construction workers, plumbers, and shipyard workers
This is a granulomatous reaction to inhaled organic antigens, esp from birds.
hypersensitivity pneumonitis
Asbestosis pneumoconiosis pts are at increased risk of?
lung carcinoma and mesothelioma
What is an asbestos body?
a long, brown rod with iron depositions found in lung samples
What are the clinical features of idiopathic pulmonary fibrosis?
- progressive dyspnea and cough
- fibrosis on lung CT beginning at the subpleura
Pts with silicosis pneumoconiosis are at increased risk of _____.
TB
What disease can sarcoidosis mimic?
Sjogren syndrome
Where is TB found in the lung?
in the upper lobes
What cytokine induces a healing response and fibrosis?
TGF-beta
What are the pathologic findings of berylliosis pneumoconiosis?
- noncaseating granulomas in the lung
- hilar lymph nodes
- systemic organs
- DX?
- progressive dyspnea and cough
- fibrosis on lung CT beginning at the subpleura
idiopathic pulmonary fibrosis
This is interstitial fibrosis due to chronic exposure to small, fibrogenic particles.
pneumoconiosis
What is the tx for sarcoidosis?
- steroids
- none- self limiting and resolves spontaneously
Fibrosis in pneumoconiosis is mediated by _____.
macs
This is a long, brown rod with iron depositions found in certain lung histology.
asbestos bodies
What is the cause of idiopathic pulmonary fibrosis?
unknown (that’s why it’s idiopathic)
What is anthracosis?
carbon in the lung (black spots)- a little is normal
Silica in the macs _____.
impair the phagolysosome formation
What are the occupations most at risk for berylliosis pneumoconiosis?
miners, aerospace workers
What disease is often associated with coal workers’ pneumoconiosis?
RA
What are the pathologic findings of silicosis pneumoconiosis?
fibrotic nodules in the upper lobes
What is an asteroid body?
a giant cell in sarcoidosis with an internal star-like pattern
What are the interstitial diseases of the lung?
- idiopathic pulmonary fibrosis
- pneumoconiosis
- sarcoidosis
- hypersensitivity pneumonitis
Pts with berylliosis pneumoconiosis are at increased risk for?
lung CA
What causes pneumoconiosis?
occupational exposure
Name 2 drugs that can induce interstitial fibrosis.
- bleomycin
- amiodarone
What is sarcoidosis?
a systemic disease characterized by noncaseating granulomas in multiple organs
Which pneumoconiosis is similar to sarcoidosis?
Berylliosis
What is the defining cell of a granuloma?
an epitheliod hystiocyte
What pneumoconiosis increases your risk of TB?
pneumoconiosis
What is the interstitium?
the thin, delicate alveolar wall
What are the pathologic findings in asbestosis pneumoconiosis?
fibrosis of the lung and pleura in plaques- may have long, golden brown fibers containing iron, which are asbestos bodies (diagnostic)
What is the most common cause of restrictive lung disease?
interstitial fibrosis
What is the treatment for idiopathic pulmonary fibrosis?
lung transplant
What are the pathologic findins in Coal Workers’ pneumoconiosis?
diffuse anthracosis and fibrosis
Name 4 exposures that can cause pneumoconiosis.
- coal/carbon
- silica
- beryllium
- asbestos
