Restrictive Lung Disease Flashcards
What are the major Sx’s of restrictive lung diseases?
Dyspnea, Tachypnea
End-inspiratory (velcro) crackles
Cyanosis
What sorts of things would be seen in CXR?
B/l nodules
Kerley B lines
Ground-glass shadows
What severe disease are associated/caused by restrictive lung diseases?
Pulmonary HTN
Cor pulmonale
R-sided HF
What is the origination of the pathogenesis of Idiopathic Pulmonary Fibrosis?
Aberrant repair of recurrent alveolar epithelial cell injuries caused by environmental exposures
Mutations in which genes are associated with IPF?
TERT
TERC
What is the age range for IPF and what is the prognosis with no Tx?
55-75 yo
3 years
What is the only curative Tx?
Transplant
If a female, non-smoker around 60 yo presents with b/l, symmetric, lower lobe opacities on radiograph and dyspnea and cough, what is your Dx?
Non-specific Interstitial Pneumonia
What is the pathogenesis of Pneumoconiosis?
Macrophages engulf inhaled particles –> activation of innate immune cells –> lung destruction
What are the severe complications of Coal Workers’ Pneumoconiosis?
Pulmonary HTN
Cor pulmonale
Which lobes does CWP mostly affect?
Upper lobes
Does CWP increase risk of cancer?
No
Which population is at an increased risk for Silicosis?
AA
What is the pathogenesis of Silicosis?
Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators
Pts with Silicosis have an increased risk for which two pathologies?
TB
Lung cancer
Which lobes does Silicosis mostly affect?
Upper lobes
Which form of asbestos is more virulent?
Serpentine/Chrysotile
What is the pathogenesis of Asbestos-related pneumoconiosis?
Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators
What is seen on CXR in pts with Asbestos-related pneumoconiosis?
Irregular linear densities in b/l lower lobes with pleural plaques
In which areas is Sarcoidosis most likely to present?
B/l hilar LNs
Lung
Eyes
Skin
Which populations are most susceptible?
Women < 40 yo
10x AA
SE U.S.
Which cells predominate in the pathogenesis of Sarcoidosis?
CD4 T cells
What is the pathogenesis of Hypersensitivity Pneumonitis?
Immunologically mediated interstitial lung disease due to prolonged exposure to inhaled Ags
Which type of Hypersensitivity Pneumonitis is due to dust from warm, newly harvested hay with thermophilic spores?
Farmers Pneumonitis
Which type of Hypersensitivity Pneumonitis is due to proteins from serum, excreta, or feathers of birds?
Pigeon Breeder’s Pneumonitis
Which type of Hypersensitivity Pneumonitis is due to thermophilic bacteria in heated water reservoirs?
Humidifier/AC Pneumonitis
Non-caseating granulomas suggesting a T cell-mediated type 4 rxn
Hypersensitivity Pneumonitis
What are the major Sx’s and CXR findings of Hypersensitivity Pneumonitis?
Fever, dyspnea, cough, leukocytosis
Micronodular interstitial infiltrates
Pulmonary eosinophilia is characterized by elevated alveolar levels of ___
IL-5 (eosinophil attractant)
Dyspnea, dry cough, and clubbing in a 40-50 yo smoker with presence of Smokers’ Macrophages is characteristic of what disease?
Desquamative Interstitial Pneumonia
What type of pt will characteristically have Pulmonary Langerhans Cell Histiocytosis?
Young smokers (get better with cessation)
Accumulation of surfactant in alveoli due to GM-CSF dysfunction is characteristic of what pathology?
Pulmonary Alveolar Proteinosis
What will be seen on CXR for Pulmonary Alveolar Proteinosis?
B/l patchy, asymmetrical pulmonary opacifications
What is the characteristic Sx of adults with Pulmonary Alveolar Proteinosis?
Chunky, gelatinous sputum
AR inheritance of the ABCA3 gene leading to dysfunction of surfactant trafficking/secretion is characteristic of what pathology?
Surfactant Dysfunction Disorder
