Pathoma - Restrictive Diseases Flashcards
Restricted Filling of the lung causes what spirometry values to change?
TLC goes down (can’t fill the total lung capacity)
FVC goes way down (amount of air you can blow out after filling all the way, makes sense cause you can’t bring that much in in the first place)
FE1 goes down (amount in first second, also makes sense)
FEV1:FVC ratio is increased (opposite of airway obstruction. This makes sense because our FVC goes way down, we are reducing the denominator more than the numerator)
Hallmark of these disorders that restrict filling
FEV1:FVC increases by >80% due to elastic recoil increasing
Most common cause of restricted filling?
Interstitial disease
May also arise with chest abnormalities like obesity
Interstitial fibrosis
Fibrosis of the elastic walls of the alveolar sac
Idiopathic Pulmonary fibrosis
Fibrosis of the lung interstitium
Histology of IPF
Random thickwalled, fibrotic alveolar sacs
Etiology of IPF?
Related to cyclical injury of unknow origin
Mechanism behind IPF?
TGF-B from injured pneumocytes induces fibrosis
Secondary causes of IPF?
Drugs (Bleomycin and amioderone)
Radiation therapy
Clinical features of IPF
Progressive Dyspnea and cough
Fibrosis on Lung CT
Treatment is lung transplantation
Honeycomb lung?
End stage IPF
Pneumoconioses and what causes it specifically
Interstitial fibrosis due to occupational exposure
Requires exposure to small particles that are fibrogenic.
The lung freaks out when it sees them and walls them off with fibrosis in an attempt to protect itself
Exposure that leads to coal workers’ pneumoconiosis
Carbon dust, seen in coal miners
Exposure that leads to silicosis
Silica, seen in sand blasters and silica miners
Exposure that leads to Berylliosis
Beryllium - seen in berryllium miners and workers in the aerospace industry
Exposure that leads to Asbestosis
- Asbestos fibers seen in construction workers, plumbers, shipyard workers
When do we see “black lung”? Discuss it
In Coal Workers pneumoconiosis - Associated with rheumatoid arthritis and is a massive exposure that leads to diffuse fibrosis
Pathologic findings for Berrylliosis?
Noncaseating granulomas in the lung, hilar lymph nodes and systemic organs
____ presents with fibrosis of lungs and pleura (plaques) with increased risk for _______ and ______.
Asbestois
Lung Cancer
Mesothelioma
Silicosis presents how?
Fibrotic nodules in upper lobes of the lung
Besides Asbestois, this condition also increases our chance for lung cancer
Berrylliosis
This condition increases our chances of TB. Why?
Silicosis - Silica impairs phagolysosome formation by macrophages
Mild exposure to carbon, like in pollution, results in this condition that is not all that clinically significant
Anthracosis - Cllection of carbon laden macrophages
How do we confirm exposure to asbestos?
Lesions may contain long, golden-brown fibers with associated iron (asbestos bodies) which confirm exposure to asbestos
Sarcoidosis and who tends to get it?
Granulomas in the interstitial of the lung (noncaseating granulomas) and other organs
African American women mostly
Cause of sarcoidosis
Unknown but likely due to CD4+ helper T-cell response to unknown antigen
What does noncaseating mean?
No death! Nuclei all still there
Defining histology of sarcoid granulomas?
Epithelioid histiocytes surrounding a giant cell granuloma that is non caseating
Common finding in a granuloma of sarcoidosis
Asteroid body in the center!
Besides lungs, what else can sarcoid hit?
- Eye - Uvea - Uveitis
- Skin - Nodules
- Salivary or lacrimal glands (mimics sjogren’s)
Clinical features of sarcoidosis?
Cough
SOB
Labs for sarcoidosis?
Elevated serum ACE and hypercalcemia, caused by granulomas have 1 alpha hydroxylase activity and can activate Vitamin D (we see this in all noncaseating granulomas)
Treatment for sarcoidosis?
Steroids, often resolves spontaneously without treatment
Hypersentitivity pneumonitis
Patient exposed to some inhaled organic antigens (bird poop for example) that results in a granulomatous reaction
Presentation and treatment for Hypersensitivity pneumonitis
Fever, cough, dyspnea hours after exposure. Resolves with removal of the exposure
Chronic exposure of whatever is causing the hypersensitivity pneumonitis leads to what?
Interstitial fibrosis
What is important to note about the granulomas involved with Hypersensitivity pneumonitis?
Remember this is hypersensitivity, so we will have a lot of eosinophils
