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restrictive lung disease Flashcards

1
Q

definition of restrictive lung disease

A

characterized by a reduction in lung volume, alteration in lung parenchyma, and a disease of pleural, chest wall, and neuromuscular status

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2
Q

what values are reduced in restrictive lung disease

A

total lung capacity
vital capacity
resting lung volume

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3
Q

what two groups can restrictive lung disease be divided into

A 
lung parenchyma (intrinsic)
extraparenchyma (extrinsic)
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4
Q

what does intrinsic lung parenchyma result in?

A

inflammation or scarring of lung tissue

filling of air spaces with exudates and debris

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5
Q

what does extraparenchyma restrictive lung disease result in?

A

diseases of the chest wall, pleura and respiratory muscles

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6
Q

what condition causes extrinsic restrictive lung disease with external pressure?

A

obesity

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7
Q

types of intrinsic lung disease

A

sarcoidosis
indiopathic pulmonary fibrosis (IPF)
interstitial lung disease
ARDS, IRDS

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8
Q

causes of interstitial lung disease

A

asbestosis, silicosis, pneumoconiosis
medication, drug, radiation
hypersensitivity pneumonitis
rheumatoid arthritis, lupus, scleroderma

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9
Q

causes of extrinsic restrictive lung disease

A

myasthenia gravis
guillain barre
kyphosis and chest wall deformities
dz that restricts lower thoracic or abdomen volume (obesity, hernia,ascities)

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10
Q

what values are reduced in restrictive lung disease?

A

TLC or VC

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11
Q

what values are preserved in restrictive lung disease

A

airflow
resting lung volume
(measured by functional residual capacity (FRC))

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12
Q

what muscles have to contract in order for the lungs to inflate?

A

respiratory muscles
diaphragm
external costal

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13
Q

is inflation or deflation passive?

A

deflation

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14
Q

volume of air in the lungs when respiratory muscles are fully relaxed and no airflow is present

A

FRC (functional residual capacity)

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15
Q

what balances inward elastic recoil of lungs and outward elastic recoil of the chest wall?

A

FRC

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16
Q

what is a multi-system inflammatory disease of unknown etiology (especially lungs, lymph nodes, and skin) that causes intrinsic restrictive lung disease?

A

sarcoidosis

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17
Q

what manifests as non-necrotizing granulomas predominantly in the lungs and intrathoracic lymph nodes and causes an exaggerated immune response

A

sarcoidosis

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18
Q

what cells are part of the inflammatory immune process in sarcoidosis?

A

T cells-WBCs lymphocytes

which start the immune reaction, identify the illness, and share info with other immune cells

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19
Q

what do T cells cause?

A

an exaggerated immune response

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20
Q

who gets sarcoidosis commonly?

A

african americans

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21
Q

age onset of sarcoidosis

A

25-45

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22
Q

how is sarcoidosis in african americans different?

A

more severe and chronic

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23
Q

multiorgan inflammation of sarcoidosis presents on

A

lung (90%)
lymphadenopathy (33%)
skin (25%)

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24
Q

what percent of sarcoidosis is asymptomatic?

A

5%

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25
Q

non-specific symptoms of sarcoidosis?

A 
fever
weight loss
fatigue
arthraliga
nagging cough
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26
Q

main complaints of sarcoidosis

A 
pulmonary complaints
DOE
cough
chest pain
hemoptysis (rare)
crackles (pulm infiltrates)
mediastinal/hilar nodes
27
Q

other signs of sarcoidosis

A 
erythema nodosum
skin lesions
lupus pernio
uveitis
lacrimal gland granduloma
28
Q

CXR staging for sarcoidosis

A 
0-nml
1-b/l hilar lymphadenopathy
2-BHL + infiltrates
3-infiltrates
4-fibrosis
29
Q

what do non-caseating grandulomas secrete

A

vitamin-D like hormone

angiotensin converting enzyme

30
Q

what does the vitamin D like hormone in sarcoidosis cause?

A

hypercalcemia

hypercalcuria (more common)

31
Q

what is the best imaging for sarcoidosis?

A

CXR

32
Q

what other imaging could you do for sarcoidosis workup

A

bronchoscopy
transbronchial/transesophageal US guided bx of mediastinal nodes
Bx of skin, lymph nodes, lacrimal glands, conjunctiva

33
Q

relative level of VC in pulm function tests in sarcoidosis patient?

A

down

34
Q

DLCO levels in sarcoidosis pts?

A

down (<60%)

35
Q

O2 sat for sarcoidosis patients?

A

<90% with 6 minute walk test

36
Q

what is one of the monst clinically valuable tests for lung function?

A

DLCO

37
Q

what does DLCO measure

A

the ability of lungs to transfer gas from inhaled air to the RBC in pulmonary capillaries

38
Q

what can >75% of sarcoidosis patients be treated with?

A

NSAIDs

39
Q

what is the mainstay of therapy for sarcoidosis?

A

corticosteroids

40
Q

how do you dose prednisone for sarcoidosis?

A

QD then tapered over 6 months

41
Q

what are sarcoidosis patients initiated on?

A

NSAIDS and steroids

42
Q

what do you prescribe for a sarcoidosis patient if they cannot tolerate steroids?

A

methotrexate

43
Q

what adverse effects does chloroquine have?

A

cutaneous lesions
hypercalcemia
neurological
bone lesions

44
Q

what stage sarcoidosis patients get lung transplants? and what are their relative VC and FEV1 levels?

A

stage 4

<40% FEV1

45
Q

followup for sarcoidosis patients

A

CXR and PFT q6-12 mo

annual slit lamp eye exam

46
Q

a specific form of chronic, progressive interstitial scarring of unknown cause

A

IPF (idiopathic pulmonary fibrosis)

47
Q

what sex is more prone to getting IPF

A

men

48
Q

what age group is most prone to IPF

A

> 50

49
Q

what is the prognosis for IPF

A

2-5 years post diagnosis

50
Q

risk factors for IPF

A 
genetics
viruses
dust exposure
GERD
smoking
51
Q

what disease is IPF thought to be?

A

epithelial-fibroblastic disease

52
Q

what does some unknown insult disrupt in IPF

A

alveolar epithelial cells causing diffuse epithelial cell disorganization

53
Q

why isn’t IPF caught sooner?

A

most DOE patients are referred to a cardilogist
5% are asymptomatic
weak history
must exclude any other causes
obstructive sleep apnea in 88% of patients

54
Q

non-specific IPF symptoms

A 
exertional dyspnea (insidious and progressive)
non-productive cough
55
Q

systemic IPF symptoms

A

weight loss
low-grade fever
fatigue
arthralgia/myalgia

56
Q

IPF PE findings

A

fine inspiratory crackles
digital clubbing
PH

57
Q

symptoms of PH

A

right ventricular heave
Increased JVP
pedal edema
tricuspid regurgitation

58
Q

IPF diagnostic testing

A 
lung Bx
decreased DLCO
elevated ANA, RF, CRP, ESR
CXR
CT
59
Q

what is seen on a CXR of an IPF pt?

A

reticular opacities at lung bases

honeycombing

60
Q

what is seen on a high-res CT of an IPF pt?

A

patchy reticular opacities

61
Q

comorbid conditions with IPF

A 
COPD
OSA
GERD
CAD
smoking
62
Q

what vaccines should IPF patients get?

A

influenza

pneumococcal

63
Q

when should an IPF patient get O2 therapy?

A

when pO2 <88%

CMPP (17 decks)

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