Restrictive Lung Disease Flashcards

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
Q

pathophys of IPF

A

cell injury and death in the lungs causes fibroblast proliferation and coagulation, eventual collagen deposition

mediated by TGF beta, KGF, CTGF, PDGF

26
Q

3 cells involved in sarcoidosis

A

histiocytes, dendritic cells w/i non caseating granuloma

lymphs surrounding, Th1 CD4 interact w/ APCs via MHC complexes

27
Q

cytokine profile in sarcoidosis

A

INF-gamma, IL 2, IL12

similar to bacteria, fungi, viral

28
Q

two possible antigens that provoke sarcoid

A

mycobacteria, propionibactera

29
Q

lofgrens syndrome

A

combo of hilar adenopathy and erythema nodosum seen in sarcoid pts, usually self resolves

30
Q

stage 1 of pulm sarcoidosis

A

adenopathy, normal lungs

31
Q

stage 2 pulm sarcoid

A

abnormal nodes and lung

32
Q

stage 3 sarcoid

A

abnormal lung, normal nodes

33
Q

stage 4 sarcoid

A

fibrosis of lung parenchyma, usually upper lobe w/ retraction of the hila

34
Q

needed for sarcoid Dx

A

granulomas (non caseating), exclude other causes (TB, fungi, cancer)

35
Q

lupus pernio

A

cutaneous sarcoid, associated w/ chronic fibrotic lung disease

36
Q

enzymes produced by sarcoid granulomas

A

1 alpha hydroxylase (increases serum calcium via calcitriol, more absorption and reabsorption)

ACE

37
Q

Tx for sarcoid

A

either let self resolve

corticosteroids