Restrictive Lung Disease Flashcards

(37 cards)

1
Q

4 most common ILD in young adults

A

IPF, pulmonary langerhans cell histiocytosis, eosinophilic granulomatosis w/ polyangiitis, sarcoidosis

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2
Q

3 requirments for normal respiratory physiology (mechanics)

A

compliant chest wall, normal muscle strength, compliant lungs

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3
Q

3 mechanisms for RLDs (think about 3 requirements for normal physiology)

A

abnormalities of chest wall (obesity, kyphoscoliosis)

weakness of respiratory muscles (polio, myasthenia gravis)

abnormalities of lung parenchyma (sarcoidosis, IPF)

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4
Q

ILD effect on PFTs

A

TLC, FRC, RV all lowered- increased elastic recoil

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5
Q

muscle weakness effect on PFTs

A

lower TLC, normal FRC (no effect on chest expansion or lung recoil), RV elevated (cant expel as much air forcefully)

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6
Q

obesity effect on PFTs

A

low TLC, low FRC (increased chest expansion), normal RV

hallmark is lower FRC

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7
Q

two pathological types of ILD

A

cellular and fibrotic

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8
Q

ILD impact on spirometry

A

reduced FVC, reduced FEV1, normal to high FEV/FVC ratio (no obstruction)

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9
Q

ILD impact on Hb sat

A

increases the time required to saturate RBC Hb for a given amount of alveolar O2, uses up the reserve time

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10
Q

ILD and cor pulmonale

A

reduced compliance leads to higher resistance for RV, eventual right heart failure

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11
Q

common disease that mimics ILD- how is it different

A

CHF, different b/c the diffuse infiltrates are from increased hydrostatic pressure and pulm edema rather than cellular/fibrotic infiltrates in the alveolar interstitium

on CXR- infiltrates tend to be in lower lungs, cardiomegaly, kerley lines

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12
Q

3 possible known etiology ILDs

A

pneumoconiosis- inhaled dust

hypersensitivity pneumonities- inhaled organic antigens

iatrogenic- radiation or drugs (bleomycin, amiodarone, methotrexate)

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13
Q

risk factors for silicosis

A

occupational exposure: mining, masonry, pottery, jewelers, quarry, foundry

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14
Q

silicosis on CXR

A

upper lobe nodular infiltrate

eggshell calcification of hilar lymph nodes

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15
Q

risk factors for asbestosis

A

shipyard, roofing, factory

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16
Q

asbestos on CXR and histopath and gross

A

lower lobe diffuse infiltrate on CXR

ferruginous bodies on histology (rod like fibers coated w/ iron)

pleural plaques grossly

17
Q

CXR of coal workers pneumoconiosis

A

mainly upper lobes, nodular pattern that can progress to diffuse

18
Q

utility of high resolution chest CT

A

helps Dx ILDs when not detectable on CXR, specific Dx, distinguish b/w active inflammation and fibrosis

19
Q

most likely Dx w/ consolidation and air bronchograms

A

bacterial pneumonia

20
Q

differentiate ground glass and consolidation

A

ground glass- can see blood vessels through infiltrate, common w/ active inflammation ILDs, cardiogenic pulm edema

cant w/ consolidation, common w/ bacteiral pneumonia

21
Q

what does ground glass mean w/ suspected ILD

A

active inflammation vs fibrosis

most common cause of this is cardiogenic pulm edema

22
Q

5 possible causes of diffuses alveolar hemorrhage

A

goodpasture, microscopic polyangiitis, granulomatosis w/ polyangiitis, lupus, cocaine

23
Q

most common idiopathic interstitial pneumonia

A

IPF, presents w/ UIP

24
Q

physical exam finding and CXR for IPF

A

traction bronchiectasis- the fibrosis pulls the airways apart causing dilation

clubbing from polycythemia

25
pathophys of IPF
cell injury and death in the lungs causes fibroblast proliferation and coagulation, eventual collagen deposition mediated by TGF beta, KGF, CTGF, PDGF
26
3 cells involved in sarcoidosis
histiocytes, dendritic cells w/i non caseating granuloma lymphs surrounding, Th1 CD4 interact w/ APCs via MHC complexes
27
cytokine profile in sarcoidosis
INF-gamma, IL 2, IL12 similar to bacteria, fungi, viral
28
two possible antigens that provoke sarcoid
mycobacteria, propionibactera
29
lofgrens syndrome
combo of hilar adenopathy and erythema nodosum seen in sarcoid pts, usually self resolves
30
stage 1 of pulm sarcoidosis
adenopathy, normal lungs
31
stage 2 pulm sarcoid
abnormal nodes and lung
32
stage 3 sarcoid
abnormal lung, normal nodes
33
stage 4 sarcoid
fibrosis of lung parenchyma, usually upper lobe w/ retraction of the hila
34
needed for sarcoid Dx
granulomas (non caseating), exclude other causes (TB, fungi, cancer)
35
lupus pernio
cutaneous sarcoid, associated w/ chronic fibrotic lung disease
36
enzymes produced by sarcoid granulomas
1 alpha hydroxylase (increases serum calcium via calcitriol, more absorption and reabsorption) ACE
37
Tx for sarcoid
either let self resolve corticosteroids