Restrictive Lung Disease Flashcards

1
Q

What are the major Sx’s of restrictive lung diseases?

A

Dyspnea, Tachypnea
End-inspiratory (velcro) crackles
Cyanosis

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2
Q

What sorts of things would be seen in CXR?

A

B/l nodules
Kerley B lines
Ground-glass shadows

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3
Q

What severe disease are associated/caused by restrictive lung diseases?

A

Pulmonary HTN
Cor pulmonale
R-sided HF

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4
Q

What is the origination of the pathogenesis of Idiopathic Pulmonary Fibrosis?

A

Aberrant repair of recurrent alveolar epithelial cell injuries caused by environmental exposures

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5
Q

Mutations in which genes are associated with IPF?

A

TERT

TERC

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6
Q

What is the age range for IPF and what is the prognosis with no Tx?

A

55-75 yo

3 years

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7
Q

What is the only curative Tx?

A

Transplant

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8
Q

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?

A

Non-specific Interstitial Pneumonia

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9
Q

What is the pathogenesis of Pneumoconiosis?

A

Macrophages engulf inhaled particles –> activation of innate immune cells –> lung destruction

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10
Q

What are the severe complications of Coal Workers’ Pneumoconiosis?

A

Pulmonary HTN

Cor pulmonale

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11
Q

Which lobes does CWP mostly affect?

A

Upper lobes

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12
Q

Does CWP increase risk of cancer?

A

No

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13
Q

Which population is at an increased risk for Silicosis?

A

AA

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14
Q

What is the pathogenesis of Silicosis?

A

Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators

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15
Q

Pts with Silicosis have an increased risk for which two pathologies?

A

TB

Lung cancer

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16
Q

Which lobes does Silicosis mostly affect?

A

Upper lobes

17
Q

Which form of asbestos is more virulent?

A

Serpentine/Chrysotile

18
Q

What is the pathogenesis of Asbestos-related pneumoconiosis?

A

Macrophages engulf inhaled particles –> inflammasome activation –> release of IL-1/IL-18 proinflammatory mediators

19
Q

What is seen on CXR in pts with Asbestos-related pneumoconiosis?

A

Irregular linear densities in b/l lower lobes with pleural plaques

20
Q

In which areas is Sarcoidosis most likely to present?

A

B/l hilar LNs
Lung
Eyes
Skin

21
Q

Which populations are most susceptible?

A

Women < 40 yo
10x AA
SE U.S.

22
Q

Which cells predominate in the pathogenesis of Sarcoidosis?

A

CD4 T cells

23
Q

What is the pathogenesis of Hypersensitivity Pneumonitis?

A

Immunologically mediated interstitial lung disease due to prolonged exposure to inhaled Ags

24
Q

Which type of Hypersensitivity Pneumonitis is due to dust from warm, newly harvested hay with thermophilic spores?

A

Farmers Pneumonitis

25
Q

Which type of Hypersensitivity Pneumonitis is due to proteins from serum, excreta, or feathers of birds?

A

Pigeon Breeder’s Pneumonitis

26
Q

Which type of Hypersensitivity Pneumonitis is due to thermophilic bacteria in heated water reservoirs?

A

Humidifier/AC Pneumonitis

27
Q

Non-caseating granulomas suggesting a T cell-mediated type 4 rxn

A

Hypersensitivity Pneumonitis

28
Q

What are the major Sx’s and CXR findings of Hypersensitivity Pneumonitis?

A

Fever, dyspnea, cough, leukocytosis

Micronodular interstitial infiltrates

29
Q

Pulmonary eosinophilia is characterized by elevated alveolar levels of ___

A

IL-5 (eosinophil attractant)

30
Q

Dyspnea, dry cough, and clubbing in a 40-50 yo smoker with presence of Smokers’ Macrophages is characteristic of what disease?

A

Desquamative Interstitial Pneumonia

31
Q

What type of pt will characteristically have Pulmonary Langerhans Cell Histiocytosis?

A

Young smokers (get better with cessation)

32
Q

Accumulation of surfactant in alveoli due to GM-CSF dysfunction is characteristic of what pathology?

A

Pulmonary Alveolar Proteinosis

33
Q

What will be seen on CXR for Pulmonary Alveolar Proteinosis?

A

B/l patchy, asymmetrical pulmonary opacifications

34
Q

What is the characteristic Sx of adults with Pulmonary Alveolar Proteinosis?

A

Chunky, gelatinous sputum

35
Q

AR inheritance of the ABCA3 gene leading to dysfunction of surfactant trafficking/secretion is characteristic of what pathology?

A

Surfactant Dysfunction Disorder