Restrictive Lung Disease Flashcards

1
Q

What is the pulmonary interstitium

A

network of tissue that extends throughout both lungs that provides support to the alveoli and capillary beds for gas exchange

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2
Q

PFTs

A

non invasive tests that measure how well the lings are expanding and contractions and how efficient gas exchange is

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3
Q

Tidal volume

A

volume of air moved in and out during each breath

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4
Q

Vital capacity

A

maximum volume of air that can be exhaled after a maximal inspiration

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5
Q

Residual volume

A

volume of air remaining in the lungs after maximal inspiration

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6
Q

Total lung capacity

A

volume of air in the lungs after maximal inspiration

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7
Q

What does spirometry measure

A

the volume of air exhaled at specific time points during a forceful and complete exhalation

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8
Q

What are the three values spirometry generate

A

FVC, FEV1 and the FEV1/FVC ratio

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9
Q

What does spirometry help differentiate between

A

obstructive and restrictive lung disease

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10
Q

What is FVC

A

maximum amount of air exhaled after a maximal inhalation

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11
Q

What is FEV1

A

amount of air exhaled in the first second

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12
Q

What is normal FEV1

A

about 70% or above

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13
Q

What does the FEV1/FVC ratio show

A

air flow obstruction (<70%)

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14
Q

When would you do flow volume loops

A

when patient has stridor or unexplained dyspnea

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15
Q

Obstructive flow volume loop

A

concave appearance

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16
Q

Restrictive flow pattern loop

A

smaller loop

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17
Q

FEV1, FVC and FEV1/FVC ratio in obstructive lung disease

A

FEV1- normal (mild disease) or decreased (mod/severe disease)

FVC- normal (mild/mod disease) or decreased (severe disease)

FEV1/FVC ratio- <70%

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18
Q

FEV1, FVC and FEV1/FVC ratio in restrictive lung disease

A

FEV1- normal or decreased

FVC- decreased

FEV1/FVC ratio- normal or increased (greater than or equal to 70)

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19
Q

What is the benefit of DLCO

A

differentiate the etiology of restrictive lung disease

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20
Q

Low DLCO

A

interstitial lung disease

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21
Q

What does DLCO measure

A

the overall function of the alveolar capillary membrane

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22
Q

Normal DLCO

A

extrathoracic cause or restriction (obesity, chest wall disorder, neuromuscular disorder)

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23
Q

What is restrictive disease associated with

A

decreased total lung capacity

24
Q

Intrinsic restrictive pulmonary disease

A

diseases of the lung parenchyma, inflammation or scarring of the lung tissue

(idiopathic fibrotic disease, pneumoconioses, sarcoidosis)

25
Q

Extrinsic restrictive pulmonary disease

A

extra-pulmonary diseases involving the chest wall, pleura and respiratory muscles

(obesity, myasthenia gravis, ALS, kyphoscoliosis)

26
Q

What medications induce interstitial lung disease

A

amiodarone, methotrexate, nitrofuranotoin

27
Q

Risk factors for idiopathic fibrosing interstitial pneumonia

A
  • smoking
  • occupational exposure (stone, metal, wood, organic dust)
  • GERD (micro-aspiration)
28
Q

Symptoms of idiopathic fibrosing interstitial pneumonia

A
  • insidious dry cough
  • exertional dyspnea
  • fatigue
  • tachypnea
29
Q

Exam characteristics for idiopathic fibrosing interstiital pneumonia

A
  • clubbing

- inspiratory rales

30
Q

PFTs in idiopathic fibrosing interstitial pneumonia

  • FVC, FEV1/FVC ratio
  • DLCO
A
  • reduced FVC
  • normal or elevated FEV1/FVC ration
  • Reduced DCLO
  • impaired 6 minute walk
31
Q

Idiopathic firbosing interstitial pneumonia on CXR?

CT?

A

CXR- increase in reticular markings

CT- diffuse patchy fibrosis with pleural based honeycombing

32
Q

Definitive diagnosis of idiopathic fibrosing interstitial pneumonia

A

lung biopsy, can also help rule out other causes

33
Q

Treatment options for idiopathic fibrosing interstitial pneumonia

A
  • supportive care (supplemental o2, vaccinations, pulm rehab)
  • medications
  • surgery (lung transplant)
34
Q

What medications are used for idiopathic fibrosing interstitial pneumonia

A

Nintedanib (tyrosine kinase inhibitor)

Pirfenidone (anti-fibrotic drug)

35
Q

Qualifications for lung transplant

A
  • age <65
  • free of stubstance abuse
  • acceptable BMI range of 20 to 29
36
Q

Pneumoconioses

A

interstitial lung disease caused by the inhalation and deposition of inorganic particles and mineral dust with subsequent reaction of the lung

37
Q

Clinically important pneumoconioses

A
  • coal workers pneumoconiosis
  • silicosis
  • asbestosis
38
Q

What is the milder form of coal worker’s pneumoconioses

A

anthracosis

39
Q

When do symptoms of coal worker’s pneumoconioses begin to develop? What are they?

A

10-15 years after exposure

chronic cough, fever, DOE

40
Q

What shows up on radiography with coal worker’s pneumoconoises

A

smaller, rounded, nodular opacities with a preference with the upper lobe

41
Q

Clinical stages of silicosis

A

acute, chronic, accelerated

42
Q

Clinical presentation of silicosis

A
  • cough
  • dyspnea
  • possible fever or pleuritic chest pain
43
Q

PFTs with silicosis

A
  • decreased FEV1 and DCLO

- normal FEV1/FVC ratio

44
Q

Findings on CXR/CT with silicosis

A

acute- bilateral, diffuse, groundglass opacities

chronic- small, innumerable rounded densities

45
Q

What are the 3 key elements diagnosis of silicosis is based on

A
  • history of silica exposure
  • chest imaging consistant with silicosis
  • absence of any other diagnosis
46
Q

Treatment of silicosis

A
  • no proven specific therapy
  • avoid further exposures
  • supportive care
  • steroid therapy
  • lung transplant
47
Q

Associated complications with silicosis

A
  • mycobacterial infection
  • asergillosis
  • lung cancer
  • chronic kidney disease
48
Q

Clinical presentation of asbestosis

A
  • asymptomatic for 20-30 years after exposure
  • dyspnea on exertion
  • cough
  • weight loss
49
Q

PFTs in asbestosis

A
  • reduced vital capacity and total lung capacity

- low DCLO

50
Q

Complication in asbestosis

A

malignant mesothelioma

51
Q

Sarcoidosis

A

multisystem granulomatous disorder of unknown etiology

52
Q

What is sarcoidodsis characterized by

A

presence of non-caseating granuloma

53
Q

Clinical presentation of sarcoidosis

A
  • dry, hacking cough
  • progressive worsening dyspnea
  • atypical chest discomfort
  • fever/night sweats
  • weight loss
54
Q

Radiographic imaging for sarcoidosis?
CXR?
CT?

A

CXR- bilateral hilar adenopathy

CT- right paratracheal lymphadenopathy along with bilateral diffuse reticular infiltrates

55
Q

How to diagnose sarcoidosis

A
  • endobronchial US guided biopsy
  • cervical mediastinoscopy
  • VATS lung biopsy
56
Q

Treatment of sarcoidosis

A
  • observation is asymptomatic

- tapering course of abx over 4 to 6 weeks