Restrictive Diseases - Pathoma Flashcards
What is the main problem in restrictive lung diseases?
restricted filling of the lung=
What are the PFT’s in restrictive lung disease?
- decreased TLC
- significantly decreased FVC, moderately decreased FEV1
- FEV1:FVC ratio is increased (>80%)
What are restrictive diseases most commonly due to?
Interstitial disease of the lung => fibrosis of the interstitium
(may also arise with chest wall abnormalities, e.g. obesity)
What is the interstitium of the lung?
Wall of the alveolar air sacs or gas barrier
What happens is Idiopathic Pulmonary Fibrosis?
fibrosis of lung interstitium
What is the etiology of Idiopathic Pulmonary Fibrosis related to?
Cyclical lung injury:
-TGF-beta from injured pneumocytes induces fibrosis (induces cyclical healing)
When diagnosing a patient with Idiopathic Pulmonary Fibrosis, what conditions must be exclued?
Other causes:
Secondary causes such as drugs (e.g. bleomycin and amiodarone) and radiation therapy
What are the clinical features of Idiopathic Pulmonary Fibrosis?
- Progressive dyspnea and cough
- Fibrosis on lung CT (entire lung => “honeycomb lung”)
- Treatment is lung transplantation
What are Pneumoconioses?
Disorders in which you get interstitial fibrosis due to some occupational exposure
=>activates alveolar macrophages to lay down fibrous tissue
What kind of exposure is required to develop Pneumoconioses?
Requires chronic exposure to small particles that are fibrogenic => get to bottom of the lung & induce fibrosis (mucus filters big particles out)
What are the four most common types of Pneumoconioses?
- Coal Workers’ Pneumoconioses
- Silicosis
- Berylliosis
- Asbestosis
What is the small particle in Coal Workers’ Pneumoconioses that induces fibrosis?
carbon dust
What pathological findings are seen in lungs with Coal Workers’ Pneumoconioses?
Diffuse fibrosis = “Black Lung”
Shrunken lungs
What joint disease is associated with Coal Workers’ Pneumoconioses?
Rhematoid Arthritis => Caplan Syndrome
What do you call carbon build up in alveolar macrophages due to mild carbon exposure?
Anthracosis
collection of carbon-laden macrophages
What kind of exposure causes Silicosis?
SILICA
-seen in sandblasters and silica miners
What pathological findings are seen in lungs with Silicosis?
***Impairs phagolysosomes formation by macrophages => causes fibrotic nodules in UPPER lobes of the lungs
What is the only type of Pneumoconioses that increases the risk for TB?
Silicosis
What type of occupation do you see Berylliosis?
- Beryllium miners
- Aerospace industry
What pathological findings are seen in Berylliosis?
Noncaseating granulomas in the lung, hilar lymph nodes, and systemic organs (similar to sarcoidosis)
Patient’s with Berylliosis have an increased risk for what condition?
Lung cancer :(
What type of occupations are exposed to asbestos?
- construction workers
- plumbers
- shipyard workers
What are the common pathological findings seen in Asbestosis?
Fibrosis of lung and pleura (plaques) with increased risk for lung carcinoma and mesothelioma
***Lung carcinoma is more common than mesothelioma (cancer of the pleura) in exposed individuals!
How do you confirm exposure to asbestos or Asbestosis?
lesions containing long, golden-brown fibers with associated iron => “asbestos bodies”
How does Sarcoidosis cause restrictive filling of the lung?
noncaseating granulomatous deposits in the interstitium of the lung => results in restricted filling
What is the etiology of Sarcoidosis?
Unknown:
-likely due to CD4+ helper T-cell response to unknown antigen
What race/gender is most commonly affected by Sarcoidosis?
Classically seen in African American females
What is the defining cell of a granuloma?
epitheliod histiocyte
What unique cell is found in Sarcoid granulomas?
Asteroid body
funny configuration of giant cells
Where do granulomas most commonly form in Sarcoidosis?
hilar lymph nodes & the lungs => leads to restrictive disease (less compliant)
What is the typical presentation in Sarcoidosis?
- Cough or dyspnea
- Elevated serum ACE
- Hypercalcemia (why? granulomas activate Vitamin D)
What causes Hypersensitivity Pneumonitis?
inhale some organic antigen (e.g. feces of bird/fethers) => granulomatous reaction to antigen => EOSINOPHILS
What initial symptoms do patients with Hypersensitivity Pneumonitis present with?
Hours after exposure:
- Fever
- Cough
- Dyspnea
***Resolves with removal of the exposure
What chronic symptoms do patients with Hypersensitivity Pneumonitis present with?
Interstitial fibrosis => restrictive lung disease
