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Flashcards in Interstitial Lung Disease Deck (35):
1

interstitial lung disease

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

2

four categories of ILDs

ILDs of known cause or association

idiopathic interstitial pneumonias

granulomatous ILDs

other (rare) DPLDs

3

interstitial lung diseases with a known cause

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

4

proposed pathogenesis of ILD

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

5

categories of idiopathic interstitial pneumonias

chronic fibrosing IIP

smoking-related IIP

acute/subacute IIP

6

chornic fibrosing IIPs

idiopathic pulmonary fibrosis (IPF)

idiopathic nonspecific interstitial pneumonia (NSIP)

7

smoking-related IIPs

respiratory bronchiolitis - interstitial lung disease (RB-ILD)

desquamative interstitial pneumonia (DIP)

8

acute/subacute IIPs

cryptogenic organizing pneumonia (COP

acute interstitial pneumonia (AIP)

9

symptoms of ILD

exertional dyspnea, usually insiduous

cough, usually non-productive

10

physical findings of ILD

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)

11

findings on ILD chest x-ray

10% are normal at initial presentation

reticular, nodular, or reticulo-nodular interstitial pattern

diffuse and patchy alveolar pattern

12

reticular pattern

indicative of IPF

13

nodular pattern

indicative of sarcoidosis

14

alveolar pattern

much more homogeneous and confluent

15

common abnormalities of IL on HRCT

reticular opacities

nodular opacities

ground glass opacities

traction bronchiectasis

honeycomb cysts

16

traction bronchiectasis

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

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reticular opacities

indicative of scleroderma

18

nodular opacities

suggestive of sarcoidosis

19

ground glass opacities

suggests hypersensitivity pneumonitis

20

honeycomb cysts

suggestive of IPF

21

pulmonary function test results for ILD

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

arterial blood gas for ILD

increased A-a gradient, decreased PaO2

23

pneumoconiosis

ILD caused by inorganic dusts

silica -> silicosis

coal dust -> coal workers pneumoconiosis

asbestos -> asbestosis

hard metal alloys -> giant cell pneumonitis

berylium 0> berylliosis

24

hypersensitivity pneumonitis (HP)

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