Interstitial Lung Disease Flashcards

(35 cards)

1
Q

interstitial lung disease

A

aka diffuse parenchymal lung disease

disease of the alveolar wall, including the interstitium, the potential space between the basement membranes of the alveolar epithelium and the capillary endothelium

may also involve the alvolar space and small airways

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2
Q

four categories of ILDs

A

ILDs of known cause or association

idiopathic interstitial pneumonias

granulomatous ILDs

other (rare) DPLDs

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3
Q

interstitial lung diseases with a known cause

A

pneumoconiosis - exposure to inorganic dusts

hypersensitivity pneumonitis - immunologic response to inhaled organic dusts

reactions to chemicals, drugs, radiation, and toxic gases

those associated with connective tissue disease

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4
Q

proposed pathogenesis of ILD

A

age related susceptible lung

wound clot

aberrant epithelial cell activation

fibroblast focus

epithelial-mesenchymal transition

hypercoagulable milieu

exaggerated ECM accumulation

progressive lung remodeling

honeycomb changes

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5
Q

categories of idiopathic interstitial pneumonias

A

chronic fibrosing IIP

smoking-related IIP

acute/subacute IIP

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6
Q

chornic fibrosing IIPs

A

idiopathic pulmonary fibrosis (IPF)

idiopathic nonspecific interstitial pneumonia (NSIP)

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7
Q

smoking-related IIPs

A

respiratory bronchiolitis - interstitial lung disease (RB-ILD)

desquamative interstitial pneumonia (DIP)

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8
Q

acute/subacute IIPs

A

cryptogenic organizing pneumonia (COP

acute interstitial pneumonia (AIP)

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9
Q

symptoms of ILD

A

exertional dyspnea, usually insiduous

cough, usually non-productive

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10
Q

physical findings of ILD

A

fine, end-inspiratory crackles (“Velcro rales)

digital clubbing (IPF)

cyanosis

signs of cor pulmonale increased P2, RV lift, R-sided S3, JVD, edema (advanced disease)

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11
Q

findings on ILD chest x-ray

A

10% are normal at initial presentation

reticular, nodular, or reticulo-nodular interstitial pattern

diffuse and patchy alveolar pattern

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12
Q

reticular pattern

A

indicative of IPF

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13
Q

nodular pattern

A

indicative of sarcoidosis

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14
Q

alveolar pattern

A

much more homogeneous and confluent

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15
Q

common abnormalities of IL on HRCT

A

reticular opacities

nodular opacities

ground glass opacities

traction bronchiectasis

honeycomb cysts

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16
Q

traction bronchiectasis

A

bronchi are bigger than expected for specific lung sizes due to airways being pulled open

17
Q

reticular opacities

A

indicative of scleroderma

18
Q

nodular opacities

A

suggestive of sarcoidosis

19
Q

ground glass opacities

A

suggests hypersensitivity pneumonitis

20
Q

honeycomb cysts

A

suggestive of IPF

21
Q

pulmonary function test results for ILD

A

restrictive defect most common

decreased lung volumes (TLC, FRC, RV)

normal or elevated FEV1/FVC

decreased lung compliance

reduced diffusion capacity

mixed restrictive and obstructive defect possible

22
Q

arterial blood gas for ILD

A

increased A-a gradient, decreased PaO2

23
Q

pneumoconiosis

A

ILD caused by inorganic dusts

silica -> silicosis

coal dust -> coal workers pneumoconiosis

asbestos -> asbestosis

hard metal alloys -> giant cell pneumonitis

berylium 0> berylliosis

24
Q

hypersensitivity pneumonitis (HP)

A

ILD caused by inhalation of organic dusts

moldy hay -> farmer’s lung

bird proteins -> bird fancier’s disease

25
drugs that can cause ILD
chemotherapeutic agents (bleomycin, busulfan, methotrexate) antibiotics (nitrofurantoin) drug-induced lupus (hydralazine, procainamide) miscellaneous (gold salts, amiodarone)
26
connective tissue diseases that can lead to ILD
rheumatoid arthritis systemic lupus erythematosis progressive systemic sclerosis (scleroderma) Sjogren's syndrome polymyositis and dermatomyositis mixed connective tissue disease anylosing spondylitis
27
idiopathic pulmonary fibrosis (IPF)
unknown cause, limited to the lungs more common in men, usually for people \> 60 yrs old risk factors include smoking and dust exposure histologial pattern of UIP no effective therapy median survival 3-3.5 years
28
histologic findings for IPF
heterogeneit, alternating zones of interstitial fibrosis, inflammation, honeycombing, and normal lung patchy thickening of alveolar septa fibroblastic foci with scant inflammation honeycomb lung, broad bands of firosis with little inflammation, no remaining alveoli - end stage disease
29
idiopathic nonspecific interstitial pneumonia (NSIP)
associated with auto-immune phenomena more common in women smoking not a risk factor usually diagnosed in middle age may respond to steroids and other immunosuppressive treatments prognosis better than IPF
30
histologic findings in NSIP
cellular and fibrotic variations a lot more inflammatory cells in cellular less so in fibrotic don't see honeycomb cysts
31
sarcoidosis
multi-system disease of unknown cause commonly affects young and middle aged adults, more common in African Americans most common presentatin is ILD, thoracic lymph node enlargement other organs also involved (skin, eyes, nose, liver, heart, CNS) non-caseating epithelioid granulomas responsive to steroids
32
Which diseases can be diagnosed with a bronchoscopic transbronchial biopsy?
granulomatous processes neoplasm infection most ILDs require surgical lung biopsy
33
treatment of ILD
removal of offending agent, if identified corticosteroids for sarcoidosis (not useful for IPF) cytotoxic/immunosuppressive agents antioxidants investigational anti-fibrotic agents supplemental oxygen influenza and pneumococcal vaccines pulmonary rehabilitation lung transplantation
34
cytotoxic/immunosuppresive agents for ILD
azathioprine cyclophosphamide mycophenolate mofetil tacrolimus
35
perfenidone
investigational anti-fibrotic agent