Restrictive Pulmonary Disorders Flashcards

1
Q

What are common causes of restrictive lung disease?

A

Restrictive lung disease can be caused by intrinsic impairments or extrinsic conditions that affect chest wall mobility, neuromuscular function, and obesity.

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2
Q

how does RLD affect lung expansion and lung volumes?

A

Reduced lung expansion, reduced lung volumes —> overall difficulty getting air into the lungs because the lung tissue does not expand fully.

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3
Q

what percentage of total Vo2 max does normal breathing take in someone with restrictive lung disease?

A

the normal work of breathing can be as much as 40% of Vo2 max in someone with RLD

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4
Q

what contributes to lung stiffness?

A

Scarring in the lung tissue is common in many conditions that cause inflammatory processes and fibrosis of lung tissue

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5
Q

what lung volumes are reduced in RLD? How does that impact air going into and out of the lungs?

A
  • all lung volumes are reduced
  • less air coming into the lungs = less coming out of the lung
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6
Q

In restrictive lung disease
- lung volumes are ___
- lung compliance is ____
- lung elastic recoil is ____ which means _____
- work of breathing is _____ which leads to ____

A
  • lung volumes are decreased
  • lung compliance is reduced
  • lung elastic recoil is normal meaning it is easy to get air out but it is hard to get air in
  • work of breathing is increased which increases HR which leads to higher resting HR
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7
Q

What is FVC?

A

Forced vital capacity is the amount of air that can be forcefully and quickly exhaled after a full inspiration

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8
Q

what is FEV1

A

FEV1 is the amount of air that you can forcibly exhale in 1 second after maximal inhalation

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9
Q
  • what is the normal FEV1/ FVC ratio?
  • what is decreased FVC associated with?
  • what is decreased FEV1 associated with?
A
  • the normal ratio is normally 80%
  • decreased FVC is associated with RLD
  • COPD has a greater reduction in FEV1 because of air trapping thus lowering the FEV1/FVC ratio
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10
Q

what is the typical FEV1/FVC ratio for RLD?

A
  • people with RLD cannot inhale as much air but do not have challenges in exhaling air so the ratio tends to be normal or increased
  • normal ratio is 75-80%
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11
Q

How does FEV1/FVC ratio differ for obstructive lung disease and RLD

A
  • obstructive lung disease affects the ratio because of air trapping. Both FEV1 and FVC are decreased and the ratio is decreased (<75-80%)
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12
Q

What are classic signs of RLD

A
  • tachypnea
    -hypoxemia (V/Q mismatch)
  • fatigue/ weight loss
  • decreased lung volumes
  • chronic dry cough
  • pulmonary HTN
  • digital clubbing, cyanosis, decreased chest wall expansion
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13
Q

what are hallmark symptoms of RLD

A

-dyspnea
-cough (dry)
- wasted emaciated appearance

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14
Q

what are common breath sounds in RLD

A

inspiratory crackles in bilateral posterior lower lobes

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15
Q

what are interstitial lung diseases?

A
  • a large group of diseases that cause scarring/fibrosis of the lungs
  • the interstitum (the tissue surrounding the alveolar sac) becomes scarred
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16
Q

describe exposure related interstitial lung disease

A

Can be drug induced through chemotherapy, methotrexate, amiodarone, or Macrobid.
Can be occupational or environmental from gas, dust, fumes, or radiation.

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17
Q

describe autoimmune related interstitial lung disease

A
  • RA
    -systemic lupus
  • scleroderma
    -polymyositis
  • dermatomyositis
    -sjogrens syndrome
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18
Q

describe idiopathic interstitial lung disease

A
  • idiopathic pulmonary fibrosis
    -acute interstitial pneumonia
  • non-specific interstitial pneumonia
  • cryptogenic organizing pneumonia
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19
Q

what is idiopathic pulmonary fibrosis?

A
  • etiology is unknown
  • inflammatory process of alveolar wall cause scarring which becomes fibrotic
  • the most common of >200 different pulmonary fibrosis lung diseases
  • a progressive disease
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20
Q

what are risk factors for idiopathic pulmonary fibrosis?

A
  • age
  • smoking
  • genetic predisposition
  • air pollution
  • viral infection
  • GERD
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21
Q

what is the typical clinical presentation of idiopathic pulmonary fibrosis?

A
  • dx between ages 50-70
  • more common in men
  • persistent dry cough, fatigue, weight loss, digital clubbing, LE edema
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22
Q

what happens to alveoli in idiopathic pulmonary fibrosis

A
  • there is enlarged airways and deformed alveoli
  • honeycombing clustered appearance in air spaces which leads to decreased gas exchange and less O2 in systemic circulation
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23
Q

What is sarcoidosis?

A
  • a inflammatory autoimmune disease
  • multisystem disease characterized by the presence of granulomas in many organs (tiny clumps of inflammatory cells that cause fibrosis = lungs affected and lymph)
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24
Q

what is the clinical presentation of sarcoidosis?

A
  • young adults
  • unexplained persistent dry cough
  • SOB and chest tightness
  • women> men
  • age: 30-55yo
25
Q

Describe pulmonary staging related to sarcoidosis

A

Patient can move back and forth from stages 1-3
Stage 1: lymph nodes
Stage 2: lymph nodes and lungs
Stage 3: lungs
Stage 4: irreversible scarring in the lungs

26
Q

what is RA?

A
  • the chronic inflammation of peripheral joints resulting in progressive destruction of articular and periarticular structures
27
Q

what is the second most common symptom in RA?

A
  • pulmonary symptoms including pulmonary nodules, pleural effusion, scarring in the airways from long term inflammation, interstitial lung disease
28
Q

what is the clinical presentation of RA

A
  • progressive dyspnea
  • non productive cough
  • cyanosis
  • warm, swollen, painful joints
  • diminished breath sounds, rales
  • reduced vital capacity
  • nodules in the peripheral lung fields (upper lobes affected)
29
Q

What are the risk factors for people with RA to develop interstitial lung disease?

A
  • those with RA have 8X higher risk of developing interstitial lung disease
  • smokers
  • severe RA
  • > 60 years = increased risk
    -males
30
Q

what is systemic lupus erythematosus (SLE)?

A
  • a multi system autoimmune disease and chronic inflammatory connective tissue disorder which unknown cause
  • can involve the skin, joints, kidneys, lungs, Nervous tissue
31
Q

what is the most common lung dysfunction associated with SLE

A

pleuritis (diaphragmatic weakness)

32
Q

what is the pulmonary clinical presentation of SLE

A
  • decreased lung volumes
  • consolidation at the base of lungs
  • pleural friction rubs
  • crackles at the lung bases
  • pulmonary HTN
33
Q

what is the general clinical presentation of SLE?

A
  • articular pain and swelling
  • arthalgia (OA)
  • dyspnea
  • fatigue “flare ups”
  • cough
  • weight loss
  • raynouds
    -photosensitivity
  • fever
  • mouth ulcers
  • chest pain
    -hair loss
  • lupus nephritis
    -anemia
34
Q

what is scleroderma?

A
  • an autoimmune disorder with unknown cause
  • progressive fibrosis disorder that causes degenerative changes in the skin, small blood vessels, esophagus, intestinal tract, lung, heart, kidney, and articular surfaces
  • in the lungs appears as progressive diffuse interstitial fibrosis
35
Q

what population is affected most by scleroderma?

A

women are affected most
ages 30-50

36
Q

what is the clinical presentation of scleroderma?

A
  • skin thickening, swelling, tightening
  • enlarged blood vessels on hands and face
  • calcium deposits on skin
    -HTN from kidney dysfunction
  • GI involvement such as bloating, constipation, and issues with esophageal motility
  • weight loss
  • decreased lung volumes = increased work of breathing= progression of interstitial lung disease
  • raynouds phenomenon
  • exertional dyspnea
  • non productive cough and digital clubbing common
    -joint pain
37
Q

what is polymyositis and dermatomyositis?

A
  • an inflammatory immune condition with progressive muscle weakness and skin changes
38
Q

what are the pulmonary characteristics of polymyositis and dermatomyositis?

A
  • aspiration pneumonia due to weakness
  • weakness of neck flexors, laryngeal, and pharyngeal muscles
  • increasing respiratory muscle weakness
  • elevated diaphragm (if involved) which reduces lung volume
  • interstitial lung disease at the lung bases
  • SOB and non productive cough
39
Q

What are the pulmonary clinical manifestations of SCI

A
  • decreased TLC, VC, IC
  • increased RV
  • decreased flow rates
  • decreased peak inspiratory and expiratory pressures
  • increased RR
40
Q

what are symptoms associated with SCI

A
  • fatigue from inefficient breathing pattern and increased use of accessory muscles
  • SOB
  • inability to cough
  • poor voice volume
  • overnight hypoxia = morning headache
41
Q

Describe respiratory involvement dependent on level of SCI

A
  • CI-2 unable to breath on their own
  • C3-C4 = impaired ventilation due to diaphragm paralysis
  • C5-C8= retained diaphragm with impaired accessory muscles
42
Q

what is diaphragm paralysis? What is the most common cause? How does it affect the diaphragm and what is the result? How is it treated?

A
  • the loss or impairment of motor function of the diaphragm due to a lesion in the neurological or muscular system
  • commonly caused by injury to the phrenic nerve
  • paralysis leads to the diaphragm being pulled upward and anterior ribs pulled inward
  • will result in alveolar hypoventilation
  • treated with mechanical ventilation if there is bilateral involvement
43
Q

Describe the signs associated with diaphragmatic paralysis
1. pulmonary
2. arterial blood gases
3. breath sounds
4. Cardiovascular

A
  1. all lung capacity and dynamic lung volumes decreased; further decreased when in supine
  2. decreased PaO2 especially in supine
  3. decreased breath sounds in the involved side
  4. severe hypoxemia = pulmonary HTN = cor pulmonale
44
Q

how does amyotrophic lateral sclerosis (ALS) affect pulmonary function

A
  • reduced pulmonary function
  • RR reduced with weak ventilatory muscles
  • secretions and infiltrates = poor airway clearance
  • decreased breath sounds ( crackles and rhochi)
  • dyspnea with mild exertion
  • easily fatigue
  • poor activity endurance
45
Q

how does polymyelitis affect pulmonary function

A
  • reduced lung volumes
  • diminished lung sounds ( rhonchi)
  • weak cough
  • anxious with poor airway clearance
    -dyspnea
  • easily fatigued
  • poor activity endurance
46
Q

How does Guillain barre syndrome affect pulmonary function

A
  • reduced lung volumes
  • diminished breath sounds (rhonchi or crackles)
  • B LE weakness
  • dyspnea
  • poor cough and poor airway clearance
  • reduced endurance and increased fatigue
  • poor activity endurance
47
Q

what is myasthenia gravis and how does it affect pulmonary function?

A
  • a chronic neuromuscular disease characterized by progressive muscular weakness on exertion
  • autoimmune disease
  • all lung volumes are decreased with diminished breath sounds
  • poor airway clearance of secretion will cause crackles or rhonchi
  • weakness and fatigue, dyspnea, weak and ineffective cough
48
Q

what is ankylosing spondylitis? how does it affect pulmonary function? what is the treatment?

A
  • a chronic inflammatory disease of the spine characterized by immobility of the sacroiliac and vertebral joint and ossification of paravertebral ligaments
  • it is a inherited arthritic condition marked by decreased compliance of the chest wall
  • treatment includes good body alignment and thoracic mobility
49
Q

what are the signs associated with ankylosing spondylitis?
1. pulmonary
2. chest
3. arterial blood gases
4. breath sounds
5. cardiovascular

A
  1. decreased VC and IC leading to increased RV and FRC
  2. upper lobe fibrosis
  3. no abnormality
  4. normal breath sounds
  5. no abnormality
50
Q

what are the symptoms associated with ankylosing spondylitis?

A
  • dyspnea on exertion
  • pleuritic chest pain in 60% of patients
  • if upper lobe involved, productive cough
  • LBP, weight loss, and anorexia
51
Q

how does kyphosis or scoliosis affect pulmonary function

A
  • an angle less than 70 degree causes no pulmonary dysfunction
  • an angle from 70-120 degrees causes some pulmonary dysfunction
  • an angle greater than 120 degrees causes severe RLD and respiratory failure
52
Q

what are the signs associated with scoliosis?
1. pulmonary
2. chest
3. arterial blood gases
4. breath sounds
5. cardiovascular

A
  1. all dynamic lung volumes and capacities are decreased
  2. grossly abnormal because of the deformity (compressed lung tissue with increased vascular marking)
  3. decreased PaO2
  4. decreased breath sounds over the compressed lungs
  5. pulmonary HTN (thicken arteriolar walls, cor pulmonale)
53
Q

what are symptoms associated with scoliosis?

A
  • dyspnea on exertion
  • decrease exercise tolerance
  • muscle spasms
  • overuse of respiratory accesory muscles
54
Q

what is pectus excavatum? what is pectus carinatum?

A
  • pectus excavatum is funnel chest and sternal depression with decreased A/P diameter that will restrict lung volumed
  • pectus carinatum is pigeon breasts with the sternum protruding anteriorly associated with childhood asthma
55
Q

how does obesity affect pulmonary function?

A
  • the chest wall is restricted due to adiposity in the chest and abdomen regions making expansion difficult and movement of the diaphragm restricted
  • volumes are reduced and shallow breathing is observed
  • commonly presents with orthopnea, dyspnea with light activity, and wheezing
56
Q

what are supportive measures for the treatment of RLD

A
  • supplemental O2
  • antibiotic therapy for secondary infection
  • interventions to promote adequate ventilation
  • interventions to prevent accumulation of secretion
  • good nutritional support
57
Q

compare lung volume and FEV1/ FVC ratio for RLD and OLD

A

RLD: decreased volumes, increased or normal FEV1/ FVC ratio
OLD: increased volumes, decreased FEV1/FVC

58
Q

compare OLD and RLD
1. anatomy affected
2. breathing phase difficulty
3. pathophysiology
4. useful measurements

A
  1. OLD: airways; RLD: lung parenchyma and thoracic pump
  2. OLD: expiration; RLD: inspiration
  3. OLD: increased airway resistance; RLD: decreased lung or thoracic compliance
  4. OLD: flow rates; RLD: volumes or capacities