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Respiratory II > Restrictive lung diseases > Flashcards

Restrictive lung diseases Flashcards

1
Q

Chest wall disorders that can cause restrictive lung disease

A

Polio
Obesity
Pleural disease
Kyphoscoliosis

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2
Q

Classic pulmonary function abnormalities in restrictive lung diseases

A

Decreases lung volumes
Decreased lung compliance
Decreased FEV1 and FVC
Normal to increased FEV1/FVC

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3
Q

General radiology finding in restrictive lung diseases

A

Bilateral lesions in the form of small nodules, irregular lines, or ground glass shadows

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4
Q

Restriction with normal DLCO

A

Extra-pulmonary cause

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5
Q

Restriction with increased DLCO

A

Pulmonary cause

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6
Q

Change in lung compliance curve in restrictive lung disease

A

Shifted down and to the right

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7
Q

Lung shows areas of contraction/scarring and formation of cystic spaces

A

Honeycomb lung

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8
Q

Clinicopathologic syndrome marked by progressive interstitial pulmonary fibrosis and respiratory failure. Has a usual interstitial pneumonia (UIP) histologic pattern

A

Idiopathic pulmonary fibrosis

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9
Q

Lung disease caused by repetitive alveolar epithelial micro-injury and aberrant wound repair

A

Idiopathic pulmonary fibrosis

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10
Q

Profibrotic mediators in idiopathic pulmonary fibrosis

A

TGF-beta and PDGF

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11
Q

Earliest microscopic lesions in idiopathic pulmonary fibrosis

A

Fibroblastic proliferation

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12
Q

Later microscopic lesion in idiopathic pulmonary fibrosis

A

Dense collagenous fibrosis with less cellular areas

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13
Q

Microscopy of lung shows cystic spaces lined by hyperplastic type II pneumocytes or bronchiolar epithelium

A

Honeycomb lung

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14
Q

Treatment for idiopathic pulmonary fibrosis

A

Immunosuppressives - slow disease

Steroids
Cyclophosphamide
Azathioprine

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15
Q

Targeted therapy for idiopathic pulmonary fibrosis

A

Tyrosine kinase inhibitor and TGF-beta antagonist

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16
Q

Only definitive therapy of idiopathic pulmonary fibrosis

A

Lung transplant

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17
Q

Diffuse interstitial lung disease, either idiopathic or with associated CT disease. Biopsies fail to show diagnostic features.

A

Non-specific interstitial pneumonia (NSIP)

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18
Q

Cellular pattern of non-specific interstitial pneumonia

A

Lymphocytes and few plasma cells
Uniform or patchy

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19
Q

Fibrosing pattern of non-specific interstitial pneumonia

A

Diffuse or patchy interstitial fibrotic lesions of roughly the same stage of development

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20
Q

Lung findings absent in non-specific interstitial pneumonia

A

Fibroblastic foci
Honeycombing
Hyaline membranes
Granulomas

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21
Q

Restrictive lung disease that develops in response to infection or inflammatory injury to the lungs. Effectively resolved with corticosteroid therapy.

A

Cryptogenic organizing pneumonia/bronchiolitis obliterans organizing pneumonia

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22
Q

CXR findings in cryptogenic organizing pneumonia

A

Localized opacities or bilateral interstitial infiltrates that may migrate over time

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23
Q

Microscopy of lung shows subpleural or peri-bronchial patchy areas of airspace consolidation, polypoid plugs of loose organizing CT of a similar age, and no honeycombing or interstitial fibrosis.

A

Cryptogenic organizing pneumonia

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24
Q

Masson bodies

A

Polypoid plugs of loose organizing CT

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25
Q

Autoimmune diseases that can be associated with bronchiolitis

A

SLE
RA
Dermatomyositis/polymyositis

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26
Q

Autoimmune diseases that can be associated with bronchiectasis

A

RA

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27
Q

Autoimmune diseases that can be associated with acute DAD interstitial lung disease with or without hemorrhage

A

RA
SLE
Scleroderma
Dermatomyositis/polymyositis

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28
Q

Autoimmune diseases that can be associated with subacute/organizing (OP) pattern of interstitial lung disease

A

RA
SLE
Scleroderma
Dermatomyositis/polymyositis

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29
Q

Autoimmune diseases that can be associated with subacute cellular pattern of interstitial lung disease

A

RA
SLE
Scleroderma
Dermatomyositis/polymyositis

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30
Q

Autoimmune diseases that can be associated with chronic cellular and fibrotic pattern of interstitial lung disease

A

RA
SLE
Scleroderma
Dermatomyositis/polymyositis

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31
Q

Autoimmune diseases that can be associated with granulomatous interstitial pneumonia

A

RA
SLE

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32
Q

Autoimmune diseases that can be associated with vascular disease, HTN, or vasculitis in interstitial lung disease

A

RA
SLE
Scleroderma
Dermatomyositis/polymyositis

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33
Q

Caplan syndrome

A

Lung disease with RA and pneumoconiosis

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34
Q

Most dangerous particle size in pneumonoconiosis

35
Q

Lung diseases associated with inhalation of coal dust

A

Anthracosis
Macules
Progressive massive fibrosis
Caplan syndrome

36
Q

Lung diseases associated with inhalation of silica

A

Silicosis
Caplan syndrome

37
Q

Lung diseases associated with asbestos

A

Asbestosis
Pleural plaques
Caplan syndrome
Mesothelioma
Carcinoma - lung, larynx, stomach, colon

38
Q

Lung diseases associated with beryllium exposure

A

Acute berylliosis
Beryllium granulomatosis
Lung carcinoma

39
Q

Lung disease associated with iron oxide exposure

40
Q

Lung disease associated with barium sulfate exposure

41
Q

Lung disease associated with tin oxide exposure

42
Q

Lung diseases associated with inhalation of chemical fumes or vapors

A

Bronchitis
Asthma
Pulmonary edema
ARDS
Mucosal injury
Fulminant poisoning

43
Q

Lung findings in simple coal worker’s pneumoconiosis

A

Coal macules
Coal nodules –> carbon laden macrophages
Centrilobular emphysema

44
Q

Lung findings in complicated coal worker’s pneumonconiosis

A

Multiple intense black scars
Dense collagen and pigmentation with necrotic center

45
Q

Most prevalent chronic occupational disease in the world

46
Q

Occupations at risk for silicosis

A

Concrete workers
Sandblasting
Hard rock mining
Stone cutting

47
Q

Inflammatory mediators associated with silicosis

A

IL-1 and IL-18

48
Q

Complication associated with silicosis

A

Increased susceptibility to TB due to impaired macrophage function

Progressive pulmonary fibrosis
Lung cancer (2x the risk)

49
Q

Lung shows tiny, discrete, pale to black nodules that coalesce to form hard collagenous scars. Nodules can undergo softening and cavitation.

50
Q

Cause of nodule softening and cavitation in silicosis

A

Underlying TB
Ischemia

51
Q

CXR finding in silicosis

A

Egg shell calcification –> thin sheet of calcification in the periphery lymph nodes

52
Q

Microscopic hallmark of central area of whorled collagen fibers with peripheral zone of dust laden macrophages

53
Q

Dust that can act as a tumor initiator and promoter

54
Q

Dense collagen plaques that are often calcified. Form on the anterior and posterolateral aspects of parietal pleura and on domes of diaphragm

A

Localized fibrous plaques/pleural plaques in asbestos exposure

55
Q

Asbestosis

A

Parenchymal interstitial fibrosis

56
Q

Golden brown fusiform or beaded rods with a translucent center in lung biopsy

A

Asbestos rods/ferruginous bodies

57
Q

Lung finding seen in berylliosis

A

Non-caseating granuloma

58
Q

Test to differentiate berylliosis from sarcoidosis

A

Beryllium lymphocyte proliferation test

59
Q

Pt presents with fever, out of proportion dyspnea, pleural effusion, and pulmonary infiltrations 1-6 mos after radiation therapy

A

Acute radiation pneumonitis

60
Q

Diffuse alveolar damage associated with atypical of hyperplastic type II pneumocytes and fibroblasts. Epithelial cell atypia and foam cells within vessel walls. History of lung cancer with radiation.

A

Acute radiation pneumonitis

61
Q

Features of chronic radiation pneumonitis

A

Pulmonary fibrosis with associated cyanosis
Pulmonary HTN and cor pulmonale

62
Q

Systemic granulomatous disease of unknown etiology with disordered immune regulation, seen in genetically predisposed

A

Sarcoidosis

63
Q

Genetic factors of sarcoidosis

A

HLA association - A1 and B8

64
Q

Morphology findings of sarcoidosis

A

Non-caseating granulomas
Asteroid bodies
Schaumann bodies

65
Q

Non-caseating granuloma description

A

Aggregates of tightly clustered epithelioid macrophages, often with giant cells

66
Q

Asteroid bodies description

A

Stellate inclusions in giant cells

67
Q

Schaumann bodies descripiton

A

Laminated concretions of calcium and proteins

68
Q

Lupus pernio

A

Violaceous nodules over nose, cheeks, and ears
Seen in sarcoidosis

69
Q

Skin lesions seen in sarcoidosis

A

Lupus pernio
Erythema nodosum

70
Q

Ocular findings in sarcoidosis

A

Iritis or iridiocyclitis
Corneal opacities
Glaucoma
Total loss of vision

71
Q

Mikulicz syndrome

A

Enlargement of lacrimal glands and salivary glands
Seen in sarcoidosis

72
Q

Cardiac findings in sarcoidosis

A

AV block
Restrictive cardiomyopathy

73
Q

One of the causes of metastatic calcification in sarcoidosis

A

1-alpha hydroxylase activity convers vit D to its active form, leading to hypercalcemia

74
Q

Findings of BAL in sarcoidosis

A

Increased proportion of CD4 T cells

75
Q

Pt presents with gradual onset of SOB, dry cough, fatigue, tightening of skin, exaggerated cold response, reflux, and difficulty swallowing.

A

Systemic sclerosis associated interstitial lung disease

76
Q

Pathophysiology of hypersensitivity pneumonitis

A

Mixed type III (more common) and type IV hypersensitivity reactions

77
Q

Findings of BAL in hypersensitivity pneumonitis

A

Increased lymphocytes
Increased proinflammatory cytokines

78
Q

Fever, dyspnea, cough, and leukocytosis start 4-6 hrs after exposure and last for couple days. CXR shows micronodular interstitial infiltrates.

A

Acute form of hypersensitivity pneumonitis

79
Q

Large collections of macrophages with abundant cytoplasm containing a dust brown pigment (smoker’s macrophages) in the airspaces of a current or former smoker. Thickened alveolar septa lined by plump cuboidal pneumocytes. Mild interstitial fibrosis.

A

Desquamative interstitial pneumonia (DIP)

80
Q

Treatment for desquamative interstitial pneumonia

A

Steroid therapy and cessation of smoking

81
Q

Marked chronic inflammation and peribronchiolar fibrosis. Presence of pigmented intraluminal macrophages within first and second order respiratory bronchioles.

A

Respiratory bronchiolitis

82
Q

Caused by defects related to GM-CSF signaling causing pulmonary macrophage dysfunction leading to accumulation of surfactant in the intra-alveolar and bronchiolar spaces

A

Pulmonary alveolar proteinosis

83
Q

Features of alveolar precipitate in pulmonary alveolar proteinosis

A

PAS positive
Contains cholesterol clefts and surfactant proteins

84
Q

Pt presents with fever and productive cough with sputum that has chunks of gelatinous material. BAL is PAS positive.

A

Pulmonary alveolar proteinosis

Respiratory II (17 decks)

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