Restrictive, Mixed Restrictive and Obstructive Lung Disease Flashcards

1
Q

What is restrictive lung disease?

A
  • inability to inhale - ⬇️ compliance of lungs
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2
Q

When doing a spirometry test in patients with restrictive lung disease what would you expect to see in FVC, FEV1 and the FEV1/FVC ratio?

A
  • ⬇️ FVC
  • normal or ⬇️ FEV1 (relative to their own FVC
  • ⬆️ FEV1/FVC ratio (due to FVC)
  • <70% FEV1/FVC ratio is diagnostic
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3
Q

In patients with respiratory lung disease we would expect to see reductions in: - inspiratory reserve volume - tidal volume - expiratory reserve volume - residual volume Why is this the case?

A
  • inability to inflate lungs (reduced compliance)
  • lungs appear smaller (seen on volume flow loops)
  • observable on X-ray
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4
Q

What is parenchymal lung disease?

A
  • disease affecting the functioning cells of the lungs
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5
Q

Specifically, where in the lungs does parenchymal lung disease generally affect?

A
  • interstitium - space around alveoli
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6
Q

Pulmonary fibrosis is a restrictive lung disease, specifically parenchymal lung disease, what does this do to the interstitium?

A
  • interstitium becomes inflamed and swollen
  • interstitium becomes fibrotic
  • ⬇️ compliance in interstitium
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7
Q

What is sarcoidosis as a parenchymal lung disease (restrictive lung disease)?

A
  • accumulation of inflammatory cells
  • accumulation of matrix proteins
  • formation of granulomas generally at hilar lymph nodes
  • multisystem
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8
Q

What is pneumoconiosis as a parenchymal lung disease (restrictive lung disease)?

A
  • dangerous particles enter and damage lungs
  • commonly called an occupational lung disease
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9
Q

What does pneumoconiosis mean?

A
  • name comes from greek
  • pneumo = wind or breathe (also pneumocytes)
  • cono = dust
  • isis = inflammation
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10
Q

Pleural effusions can turn into a pleural disease, which are forms of respiratory disease, what is a pleural effusion?

A
  • ⬆️ fluid in pleural space
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11
Q

Pneumothorax can turn into a pleural disease, which are forms of respiratory disease, what is a pneumothorax?

A
  • collapsed lung
  • caused by air leaking into pleural space
  • ⬇️ volume = ⬆️ pressure on lung
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12
Q

Haemothorax can turn into a pleural disease, which are forms of respiratory disease, what is a haemothorax?

A
  • accumulation of blood in pleural space
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13
Q

Pleural calcification/thickening can turn into a pleural disease, which are forms of respiratory disease, what is pleural calcification/thickening?

A
  • calcification of pleural walls
  • causes thickening and stiffening of pleura
  • can be caused by asbestos (pleural plaques)
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14
Q

Why can obesity be classified as a restrictive lung disease?

A
  • fat compressed thoracic walls
  • diaphragm and lungs cannot inhale
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15
Q

What are some common diseases that affect the chest wall and ultimately reduce total lung capacity that are classed as restrictive lung diseases?

A
  • neuromuscular
  • diaphragmatic palsy
  • kyphosis
  • scoliosis
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16
Q

If the parenchyme of the lungs is damaged, what does this commonly cause?

A
  • inflammation
  • fibrosis follows
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17
Q

In diffused parenchymal lung disease (restrictive lung disease), what happens to fibroblasts?

A
  • migration and proliferation
  • at site of damage
  • leads to fibrotic loci
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18
Q

What are the 3 main diffused parenchymal lung disease (restrictive lung disease) that we are required to know about?

A

1 - Sarcoidosis

2 - Idiopathic Interstitial Pneumonia

3 - Non-Specific Interstitial Pneumonia

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19
Q

If the interstitium becomes damaged, inflamed and fibrotic, what does that do to the lungs ability to perfuse O2?

A
  • O2 perfusion is ⬇️
  • SAO2 is ⬇️
  • patients become breathless
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20
Q

When taking a history of patients with suspected lung disease, what are some of the most common things that should be included?

A
  • occupational history
  • pets, specifically birds
  • drug use
  • radiation exposure
  • autoimmune disorders
  • HIV
  • family history
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21
Q

What are the 4 most common symptoms observed in patients with diffused parenchymal lung disease, a restrictive lung disease?

A
  • breathlessness (progressively worsens)
  • cough
  • fatigue (low Sa02)
  • weight loss
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22
Q

In specific autoimmune associated diffused parenchymal lung disease, a restrictive lung disease, what are some additional common symptoms that they may present with?

A
  • difficulty swallowing
  • cold hands
  • joint pain
  • weight loss
  • skin rash
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23
Q

When examining a patient with suspected diffused parenchymal lung disease, a restrictive lung disease, what may there respiratory rate be?

A
  • ⬆️ respiratory rate
  • tachypnoea or dysponea
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24
Q

What is tachypnoea?

A
  • rapid short breathing
  • normal respiratory rate is 12-16 breaths/minute
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25
Q

What is dysponea?

A
  • greek for disordered breathing
  • shortness of breathe
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26
Q

Clubbing can be common in patient with suspected diffused parenchymal lung disease, a restrictive lung disease, what is clubbing?

A
  • thickening of tips of toes and fingers
  • look at fingers side on
  • feel fingers for swelling
  • loss of schamroth angle between fingers
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27
Q

What lung sounds may be heard in patient with suspected diffused parenchymal lung disease, a restrictive lung disease?

A
  • fine crackles
  • bibasal = bottom of both lungs
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28
Q

What cardiovascular changes may occur in patients with suspected diffused parenchymal lung disease, a restrictive lung disease?

A
  • increased pressure in lungs back up into right side of heart
  • RA pressure seen in ⬆️ jugular venous pressure
  • ⬆️ peripheral oedema
  • loud P2 sound (PV closing - pulmonary hypertension)
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29
Q

In patients with suspected diffused parenchymal lung disease, a restrictive lung disease what happens to SaO2, especially during exertion?

A
  • ⬇️ SaO2
  • ⬇️ SaO2 upon exertion
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30
Q

In addition to all the common presentations associated with diffused parenchymal lung disease, a restrictive lung disease, what may patients with an autoimmune form of the disease also present with?

A
  • skin changes
  • eye signs
  • joint signs
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31
Q

What does idiopathic mean?

A
  • no known cause
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32
Q

What is idiopathic pulmonary fibrosis?

A
  • idiopathic = no known cause
  • pulmonary = lungs
  • fibrosis = excess collagen that becomes fibrotic
  • lungs become damaged and inflamed
  • lungs scar and become fibrotic, specifically in interstitium
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33
Q

What is the prevalence of idiopathic pulmonary fibrosis in the UK?

A
  • 6-14.6 per 100,000
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34
Q

What age does idiopathic pulmonary fibrosis increase significantly?

A
  • >75 year olds
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35
Q

Is idiopathic pulmonary fibrosis more common in patients above or below 50 years old?

A
  • >50 years old
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36
Q

Is idiopathic pulmonary fibrosis more common in males or females?

A
  • males
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37
Q

Why does idiopathic pulmonary fibrosis cause crackles on auscultation?

A
  • alvelor air sacs popping open and collapsing
  • sounds like when you dive and breathe
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38
Q

What is the prognosis in idiopathic pulmonary fibrosis?

A
  • poor
  • median survival is only 2.5-3.5 years
  • exacerbations can kill, such as infections
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39
Q

On an X-ray or Ct scan, what may be present in patients with idiopathic pulmonary fibrosis?

A
  • really small lungs
  • honeycomb appears
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40
Q

How is idiopathic pulmonary fibrosis treated?

A
  • no known cure
  • symptoms (cough and hypoxia) are treated
  • antifibrotic therapy
  • lung transplant
  • palliative care
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41
Q

What is non-specific interstitial pneumonia?

A
  • form of idiopathic pulmonary fibrosis
  • has been linked with autoimmune disease
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42
Q

What is collagen vascular disease?

A
  • form of autoimmune disease
  • body attacks its own collagen throughout the body
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43
Q

What is the median age of patients with non-specific interstitial pneumonia?

A
  • 40-50 years old
  • not linked to smoking
44
Q

What symptoms are common in non-specific interstitial pneumonia?

A
  • progressive worsening of shortness of breathe over 1 years
  • cough
  • crackles
  • weight loss
  • fatigue
45
Q

What 3 diseases is non-specific interstitial pneumonia associated with?

A

1 - scleroderma

2 - rheumatoid arthritis

3 - systemic lupus erythematosus

46
Q

Does non-specific interstitial pneumonia or idiopathic pulmonary fibrosis have a better prognosis?

A
  • non-specific interstitial pneumonia
  • better response to steroids
47
Q

How is non-specific interstitial pneumonia treated?

A
  • treat underlying cause
  • immunotherapy
  • long term O2 therapy
  • palliative care
48
Q

What causes sarcoidosis, a parenchymal lung disease (restrictive lung disease)?

A
  • no known aetiology
  • linked with autoimmune deficiency
  • mainly affect the lungs
  • may be genetic inheritance
49
Q

Is sarcoidosis, a parenchymal lung disease (restrictive lung disease) permanent?

A
  • no
  • can resolve itself
  • can remain latent and present at any time
  • remains latent in granulomas of T cells and macrophages
50
Q

Is sarcoidosis more common in men or women?

A
  • women
51
Q

What age group does sarcoidosis normally affect?

A
  • 20-50 years old
52
Q

Is there an ethnicity affect of sarcoidosis?

A
  • yes
  • Scandinavian, Afro-Caribbean and African American
53
Q

What is the incidence of Sarcoidosis in the UK?

A
  • 5 per 100,000
  • most common idiopathic pulmonary fibrosis disease in UK
54
Q

What infection of the lungs is Sarcoidosis commonly associated with?

A
  • tuberculosis which has similiar symptoms
  • also linked with lymphoma
55
Q

What are the acute symptoms of sarcoidosis?

A
  • presents similiar to the flu
  • fever/night sweats
  • arthralgia (joint pain)
  • myalgia (muscle pain)
  • anterior uveitis (inflammation of middle of the eye) - breathlessness
  • ⬇️ appetite
  • weight loss
  • erythema nodosum
  • lymphadenopathy
56
Q

What are the 2 most common acute symptoms of sarcoidosis?

A
  • erythema nodosum (painful red skin)
  • anterior uveitis (painful red eyes)
57
Q

How long do sarcoidosis symptoms last in an acute bout?

A
  • short duration
  • usually self limiting
58
Q

How long do sarcoidosis symptoms last in an chronic bout?

A
  • longer than acute
  • radiological changes become present
  • may see bilaterl lymphadenopathy
59
Q

How is sarcoidosis commonly identified?

A
  • generally an incidental finding
  • best on a CXR
60
Q

What might a patient with chronic sarcoidosis present with when sarcoidosis is active?

A
  • progressive worsening of breathlessness
  • ⬇️ exercise tolerance
  • cough
  • ⬇️ appetite/anorexia
  • weight loss
61
Q

Sarcoidosis can be multisystem, what organs can it affect?

A
  • neurological symptoms
  • bone pain
  • skin lesions (nodules erythema nodosum)
  • renal stones (due to hypercalcaemia)
62
Q

What % of patients with sarcoidosis have any lung involvement, or only lung involvement?

A
  • 50% have only lung involvement - 90% have some form of lung involvement
63
Q

How can sarcoidosis be diagnosed?

A
  • no definitive test
  • combination of clinical experience and tests
  • bilateral lymphadenopathy on CXR
  • hypercalcaemia (created by macrophages)
  • high T cells and ACE (produced by T cells)
  • biopsy
64
Q

What are some common tests used to help diagnose sarcoidosis?

A
  • family and occupational history
  • radiography (CXR)
  • lung function
  • ECG (arrythmias)
  • blood and urine analysis
  • eye examination
  • skin test (rule out TB)
  • angiotensin converting enzymes (ACE)
  • hypercalcaemia
65
Q

How many different stages of sarcoidosis are there?

A
  • 5 stages - 0 - IV
66
Q

Is sarcoidosis only a restrictive lung disease?

A
  • no - can be obstructive and restrictive
67
Q

Why is angiotensin converting enzymes (ACE) measured in patients with suspected sarcoidosis?

A
  • T cells and macrophages are increased and can form granulomas
  • both can secrete ACE
  • so if sarcoidosis is active ACE wil be higher
68
Q

What is the prognosis of sarcoidosis?

A
  • 2/3 in remission within 10 years - those in stage 1, 60% in remission in 2 years - 1/3 progress to significant organ damage
69
Q

What is the mortality rate in patients with sarcoidosis?

A
  • 1-5% die - respiratory failure - cardiac arrhythmia - neurosarcoidosis
70
Q

How are patients with sarcoidosis generally treated?

A
  • oral prednisolone (steroid) - stage 2-4
  • sarcoidosis and TB present similarly
  • steroid worsen TB, so correct diagnosis is key!
71
Q

Do asymptomatic or stage 1 sarcoidosis patients receive any specific treatment?

A
  • no generally observation only
72
Q

What is an exudate?

A
  • think E for Exit incorrectly
  • fluid from circulatory system
  • generally due to inflammation
  • high protein and lactate dehydrogenase content (similiar to blood)
73
Q

What is the protein and lactate dehydrogenase (LDH) content of exudate?

A
  • ⬆️ protein >0.5
  • ⬆️ LDH >0.6
  • 2/3 of serum levels (higher than serum)
74
Q

What is lactate dehydrogenase?

A
  • enzyme in all cells
  • converts pyruvate to lactate
75
Q

What is transudate?

A
  • think T = transversing the membrane
  • thinner fluid than exudate
  • extravascular fluid
  • generally due to high hydrostatic or low osmotic pressures
76
Q

What is the protein and lactate dehydrogenase (LDH) content of transudate?

A
  • ⬇️ protein <0.5
  • ⬇️ LDH <0.6
  • 2/3 of serum levels (higher than serum)
77
Q

When could a pneunomthorax (air in pleural space) occur?

A
  • generally due to trauma
  • spontaneous symptoms
78
Q

When could a haemomthorax (blood in pleural space) occur?

A
  • generally due to trauma
  • spontaneous symptoms
79
Q

What are mesothelioma cells?

A
  • simple squamous cells originating from mesoderm
  • cells lining pleural walls
  • secrete pleural fluid
80
Q

What is a common cause of thickening of the pleura and mesothelioma?

A
  • exposure to chemicals and toxins
  • specifically asbestos
81
Q

What is the only way to test if fluid in the pleural space is an exudate or transudate?

A
  • pleural aspiration guided by ultrasound
  • pleural drainage can be used if lots of fluid
82
Q

When analysing pleural fluid what are the 3 types of tests that are required?

A
  • biochemistry (protein and lactate dehydrogenase)
  • microbiology (rule out TB)
  • cytology (rule out cancer)
83
Q

When investigating pleural aspirations what else is required to compare this with?

A
  • serum blood sample
84
Q

What is Lights Criteria?

A
  • accurate diagnosis of pleural effusions
  • determines if effusion is transudate or exudate
85
Q

What are the 2 most common causes of transudate pleural effusions?

A

1 - increase hydrostatic pressure

  • pulmonary hypertension and congestive cardiac failure

2 - ⬇️ albumin

  • liver failure or nephrotic syndrome
86
Q

What are some common causes of exudate pleural effusions?

A
  • malignancy
  • infection (pneumonia, TB, emphysema)
  • autoimmune disease
  • chylothorax (blocked lymphatics)
87
Q

What are some common signs of a pleural effusion in patients?

A
  • ⬇️ chest wall movement on side of effusion
  • ⬇️ ventilation on side of effusion
  • dullness on percussion (MOST reliable finding)
  • ⬇️ tactile vocal fremitus and vocal resonance on side of perfusion
  • bronchial breathing above perfusion
  • tracheal deviation away from perfusion
88
Q

In patients with a pneumothorax (collapsed lung), what may they present with?

A
  • sudden shortness of breathe
  • sudden chest pain
  • BOTH SPONTANEOUS as generally caused by trauma
  • WILL BE ON EXAM!!!!!!
89
Q

Is a pneumothorax (collapsed lung) dangerous?

A
  • yes, can be medical emergency
90
Q

Why is Boyles Law important in a pneumothorax (collapsed lung)?

A
  • ⬆️ pressure = ⬇️ volume - pressure changes will crush lung cavity
91
Q

How can a pneumothorax (collapsed lung) be treated?

A
  • lung aspiration using needle - chest drain if required
92
Q

What may be present on radiography in a patient with a pneumothorax?

A
  • large space
  • lung may have collapsed, difference size between lungs
  • may cause tracheal deviation
93
Q

What are some primary risk factors for a pneumothorax (collapsed lung)?

A
  • tall and thin - asthma - collagen vascular disease
94
Q

What are some secondary risk factors underlying to lung disease for a pneumothorax (collapsed lung)?

A
  • COPD - pulmonary fibrosis - cystic fibrosis
95
Q

What is the only thing that can cause calcium build up on the walls of the pleural (pleural plaques), not in lungs?

A
  • asbestos inhalation - nothing else can cause pleural plaques WILL BE ON EXAM!!!!!!!
96
Q

Do chest wall disease affect the lungs ability to perfuse O2?

A
  • no lung tissue is not normally affected
97
Q

What will a patient with chest wall disease present with in a respiratory clinic?

A
  • progressive worsening of breathlessness
  • not sudden though
98
Q

What are the 3 main groups that can cause chest wall disease in respiratory medicine?

A
  • obesity
  • neuromuscular (innervation of muscles of chest wall)
  • musculoskeletal
99
Q

Smoking can cause a combination of obstructive and restrictive lung disease, what is a common example?

A
  • emphysema = OBSTRUCTIVE
  • pulmonary fibrosis = RESTRICTIVE
100
Q

Pulmonary Sarcoidosis can cause a combination of obstructive and restrictive lung disease, what is a common example?

A
  • endobronchial sarcoidosis = OBSTRUCTIVE
  • fibrosis = RESTRICTIVE
101
Q

In an obese patient who smokes this can cause a combination of obstructive and restrictive lung disease, what is a common example?

A
  • smoking = OBSTRUCTIVE - obesity = RESTRICTIVE
102
Q

Some comon symptoms of a pneumothroax can include breathlessness, chest pain, tachycardia, coughing, tacyopnea and more. Do these symptoms present suddenly or progressively get worse?

A
  • sudden onset of symptoms
  • generall caused by trauma
103
Q

In idiopathic pulmonary fibrosis how long does shortness of breathe need to have been present for?

A
  • >2 years
104
Q

Why does idiopathic pulmonary fibrosis cause a dry cough?

A
  • no specific known reason
  • fibrosis stress on lungs may cause it
  • fibrosis may increase sensitivity to coughing triggers
105
Q

Why does idiopathic pulmonary fibrosis cause a clubbing and hypoxia?

A
  • clubbing = CO2 cannot be sufficiently perfuse out of lungs
  • hypoxia = O2 cannot perfuse sufficiently
106
Q

In idiopathic pulmonary fibrosis is weight loss and fatigue common?

A
  • yes