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Restrictive lung diseases Flashcards

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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
Autoimmune diseases that can be associated with bronchiolitis
SLE RA Dermatomyositis/polymyositis
26
Autoimmune diseases that can be associated with bronchiectasis
RA
27
Autoimmune diseases that can be associated with acute DAD interstitial lung disease with or without hemorrhage
RA SLE Scleroderma Dermatomyositis/polymyositis
28
Autoimmune diseases that can be associated with subacute/organizing (OP) pattern of interstitial lung disease
RA SLE Scleroderma Dermatomyositis/polymyositis
29
Autoimmune diseases that can be associated with subacute cellular pattern of interstitial lung disease
RA SLE Scleroderma Dermatomyositis/polymyositis
30
Autoimmune diseases that can be associated with chronic cellular and fibrotic pattern of interstitial lung disease
RA SLE Scleroderma Dermatomyositis/polymyositis
31
Autoimmune diseases that can be associated with granulomatous interstitial pneumonia
RA SLE
32
Autoimmune diseases that can be associated with vascular disease, HTN, or vasculitis in interstitial lung disease
RA SLE Scleroderma Dermatomyositis/polymyositis
33
Caplan syndrome
Lung disease with RA and pneumoconiosis
34
Most dangerous particle size in pneumonoconiosis
1-5 mcm
35
Lung diseases associated with inhalation of coal dust
Anthracosis Macules Progressive massive fibrosis Caplan syndrome
36
Lung diseases associated with inhalation of silica
Silicosis Caplan syndrome
37
Lung diseases associated with asbestos
Asbestosis Pleural plaques Caplan syndrome Mesothelioma Carcinoma - lung, larynx, stomach, colon
38
Lung diseases associated with beryllium exposure
Acute berylliosis Beryllium granulomatosis Lung carcinoma
39
Lung disease associated with iron oxide exposure
Siderosis
40
Lung disease associated with barium sulfate exposure
Baritosis
41
Lung disease associated with tin oxide exposure
Stannosis
42
Lung diseases associated with inhalation of chemical fumes or vapors
Bronchitis Asthma Pulmonary edema ARDS Mucosal injury Fulminant poisoning
43
Lung findings in simple coal worker's pneumoconiosis
Coal macules Coal nodules --> carbon laden macrophages Centrilobular emphysema
44
Lung findings in complicated coal worker's pneumonconiosis
Multiple intense black scars Dense collagen and pigmentation with necrotic center
45
Most prevalent chronic occupational disease in the world
Silicosis
46
Occupations at risk for silicosis
Concrete workers Sandblasting Hard rock mining Stone cutting
47
Inflammatory mediators associated with silicosis
IL-1 and IL-18
48
Complication associated with silicosis
Increased susceptibility to TB due to impaired macrophage function Progressive pulmonary fibrosis Lung cancer (2x the risk)
49
Lung shows tiny, discrete, pale to black nodules that coalesce to form hard collagenous scars. Nodules can undergo softening and cavitation.
Silicosis
50
Cause of nodule softening and cavitation in silicosis
Underlying TB Ischemia
51
CXR finding in silicosis
Egg shell calcification --> thin sheet of calcification in the periphery lymph nodes
52
Microscopic hallmark of central area of whorled collagen fibers with peripheral zone of dust laden macrophages
Silicosis
53
Dust that can act as a tumor initiator and promoter
Asbestos
54
Dense collagen plaques that are often calcified. Form on the anterior and posterolateral aspects of parietal pleura and on domes of diaphragm
Localized fibrous plaques/pleural plaques in asbestos exposure
55
Asbestosis
Parenchymal interstitial fibrosis
56
Golden brown fusiform or beaded rods with a translucent center in lung biopsy
Asbestos rods/ferruginous bodies
57
Lung finding seen in berylliosis
Non-caseating granuloma
58
Test to differentiate berylliosis from sarcoidosis
Beryllium lymphocyte proliferation test
59
Pt presents with fever, out of proportion dyspnea, pleural effusion, and pulmonary infiltrations 1-6 mos after radiation therapy
Acute radiation pneumonitis
60
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.
Acute radiation pneumonitis
61
Features of chronic radiation pneumonitis
Pulmonary fibrosis with associated cyanosis Pulmonary HTN and cor pulmonale
62
Systemic granulomatous disease of unknown etiology with disordered immune regulation, seen in genetically predisposed
Sarcoidosis
63
Genetic factors of sarcoidosis
HLA association - A1 and B8
64
Morphology findings of sarcoidosis
Non-caseating granulomas Asteroid bodies Schaumann bodies
65
Non-caseating granuloma description
Aggregates of tightly clustered epithelioid macrophages, often with giant cells
66
Asteroid bodies description
Stellate inclusions in giant cells
67
Schaumann bodies descripiton
Laminated concretions of calcium and proteins
68
Lupus pernio
Violaceous nodules over nose, cheeks, and ears Seen in sarcoidosis
69
Skin lesions seen in sarcoidosis
Lupus pernio Erythema nodosum
70
Ocular findings in sarcoidosis
Iritis or iridiocyclitis Corneal opacities Glaucoma Total loss of vision
71
Mikulicz syndrome
Enlargement of lacrimal glands and salivary glands Seen in sarcoidosis
72
Cardiac findings in sarcoidosis
AV block Restrictive cardiomyopathy
73
One of the causes of metastatic calcification in sarcoidosis
1-alpha hydroxylase activity convers vit D to its active form, leading to hypercalcemia
74
Findings of BAL in sarcoidosis
Increased proportion of CD4 T cells
75
Pt presents with gradual onset of SOB, dry cough, fatigue, tightening of skin, exaggerated cold response, reflux, and difficulty swallowing.
Systemic sclerosis associated interstitial lung disease
76
Pathophysiology of hypersensitivity pneumonitis
Mixed type III (more common) and type IV hypersensitivity reactions
77
Findings of BAL in hypersensitivity pneumonitis
Increased lymphocytes Increased proinflammatory cytokines
78
Fever, dyspnea, cough, and leukocytosis start 4-6 hrs after exposure and last for couple days. CXR shows micronodular interstitial infiltrates.
Acute form of hypersensitivity pneumonitis
79
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.
Desquamative interstitial pneumonia (DIP)
80
Treatment for desquamative interstitial pneumonia
Steroid therapy and cessation of smoking
81
Marked chronic inflammation and peribronchiolar fibrosis. Presence of pigmented intraluminal macrophages within first and second order respiratory bronchioles.
Respiratory bronchiolitis
82
Caused by defects related to GM-CSF signaling causing pulmonary macrophage dysfunction leading to accumulation of surfactant in the intra-alveolar and bronchiolar spaces
Pulmonary alveolar proteinosis
83
Features of alveolar precipitate in pulmonary alveolar proteinosis
PAS positive Contains cholesterol clefts and surfactant proteins
84
Pt presents with fever and productive cough with sputum that has chunks of gelatinous material. BAL is PAS positive.
Pulmonary alveolar proteinosis

Respiratory II (17 decks)

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