Restrictive, Mixed Restrictive and Obstructive Lung Disease Flashcards

1
Q

AAFB

A

acid and alcohol fast bacilli

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2
Q

DPLD

A

Diffuse parenchymal lung disease

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3
Q

IPF

A

idiopathic pulmonary fibrosis

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4
Q

LDH

A

Lactate Dehydrogenase

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5
Q

NSIP

A

non-specific interstitial pneumonitis

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6
Q

SLE

A

systemic lupus erythematosus

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7
Q

Lung Function Test

A
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8
Q

Restrictive Lund disease diagram

A
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9
Q

Restriction diagram

A
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10
Q

Causes of restriction (4)(3)(5)(0)(4):

A
  • Parenchymal lung disease (DPLD):
  • Pulmonary fibrosis
  • Sarcoidosis
    -Pneumoconiosis
  • Pleural disease:
  • pleural effusion
  • pneumothorax
  • haemothorax
  • pleural calcification, thickening
  • mesothelioma
  • Obesity:
  • Chest wall disease:
  • neuromuscular disease
  • diaphragmatic palsy
  • kyphosis
  • scoliosis
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11
Q

Diffuse Parenchymal Lung Disease diagram:

A

Damage to lung parenchyma leads to inflammation and fibrosis
Injury causes release of chemicals that damage epithelium leading to inflammation and fibrosis (go over inflammatiton)

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12
Q

Fibrosis of the Lungs:

A
  • fibrosis of the lungs leads to scarring
  • primary site of injury of the interstitium: the space between the epithelial cells and endothelial basement membrane
  • lungs become thick and stiff
  • there is reduction transfer of oxygen from the alveolar space into the lung capillaries
  • patients become breathless
  • oxygen saturation will drop
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13
Q

History in suspected diffuse parenchymal lung disease:

A
  • Occupation: asbestos, pneumoconiosis
  • Pets: birds (pigeons, budgies, parakeets),cats, dogs
  • Drugs: amiodaron, nitrofurantoin, chemotherapy
  • Exposure to Radiation, including radiotherapy
  • Systemic enquiry: autoimmune diseases
  • HIV
  • family history
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14
Q

Symptoms in patients with Diffuse Parenchymal Lung Disease:

A
  • breathlessness, progressively worsening
  • cough
  • fatigue
  • weight loss
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15
Q

Symptoms specific to autoimmune disease/collagen vascular disease:

A
  • difficulty swallowing
  • cold hands
  • joint pains
  • weight loss
  • skin rash
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16
Q

Clinical Examination in Diffuse Parenchymal Lung Disease:

A
  • high respiratory rate at rest (tachypnoea, dyspnoea)
  • clubbing
  • fine bibasal crackles
  • cardiovascular examination
  • Increased JVP, peripheral oedema, loud P2 heart sound
  • low oxygen saturation and desaturation on exertion
  • Features of autoimmune disease:
    • skin changes: telangiectasia, Raynaud’s phenomena
  • joint signs
  • eye signs
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17
Q

High respiratory rate, clubbing, fine bibasal crackles, breathless likely to be

A

pulmonary fibrosis (DPLD)

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18
Q

Idiopathic pulmonary fibrosis definition

A

IPF is a distinctive type of chronic fibrosing interstitial pneumonia of an unknown cause limited to the lungs

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19
Q

Prevalence of idiopathic pulmonary fibrosis

A

6-14.6/100,000

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20
Q

Prevalence of idiopathic pulmonary fibrosis in over 75 yrs.

A

175/100,000

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21
Q

Idiopathic Pulmonary Fibrosis usually presents in what age group?

A

Over 50 yrs

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22
Q

Which gender does idiopathic pulmonary fibrosis present in?

A

Male more than female

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23
Q

Does idiopathic pulmonary fibrosis have any occupational history of relevance?

A

No

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24
Q

Does idiopathic pulmonary fibrosis have any features of autoimmune disease?

A

No

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25
Q

Can idiopathic pulmonary fibrosis be famial?

A

Yes

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26
Q

Presentation of Idiopathic Pulmonary Fibrosis

A
  • progressively worsenign shortness of breath over 2 years
  • dry cough
  • crackles (fine babasil)
  • clubbing
  • weight loss
  • hypoxic
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27
Q

Prognosis of idiopathic pulmonary fibrosis

A
  • gradual deterioration
  • median length of survival from diagnosis: 2.5-3.5 years
  • exacerbations and sudden decline
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28
Q
A

Idiopathic pulmonary fibrosis

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29
Q

HRCT of idiopathic pulmonary fibrosis

A

watch this part of lecture

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30
Q

Idiopathic pulmonary fibrosis: lung function:

A
  • FVC decreases
  • FEV1 normal or slightly low
  • FEV1/FVC increases
  • reduced transfer factor/diffusing capacity (TLCO)
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31
Q

Management of Idiopathic pulmonary fibrosis: 4 types of treatment:

A
  • Symptomatic Treatment
  • Antifibrotic Treatment
  • Lung Transplantation under the age of 60
  • Palliative Care
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32
Q

Is Idiopathic pulmonary fibrosis associated with smoking?

A

Yes

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33
Q

Management of Idiopathic pulmonary fibrosis: Symptomatic Treatment:

A
  • cough: mucolytics
  • hypoxia treated with long term oxygen therapy
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34
Q

Management of Idiopathic pulmonary fibrosis: Antifibrotic Therapy:

A
  • Pirfenidone
  • Nintedanib
    (not a cure but can stabilise)
    (limiting factors are side effects)
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35
Q

What is Non - Specific Interstitial Pneumonia (NSIP) associated with?

A
  • associated with autoimmune disease/ collagen vascular disease
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36
Q

Median age affected by non-specific interstitial pneumonia

A

40-50 yrs

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37
Q

Which gender does non - specific interstitial pneumonia affect more?

A

Affects both equally

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38
Q

Does non-specific interstitial pneumonia have an association with smoking?

A

No

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39
Q

Symptoms of non-specific interstitial pneumonia

A
  • progressively worsening breathlessness over 1 year
  • cough
  • crackles
  • clubbing
  • fatigue
  • weight loss
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40
Q

NSIP Associated with Collagen Vascular Disease

A
  • Scleroderma (CREST/Systemic Sclerosis)
  • Rheumatoid arthritis
  • SLE
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41
Q

Non-specific interstitial pneumonia has a worse prognosis than IPF.

True or False? Why?

A

False
Better prognosis as can treat underlying autoimmune disease, which ease symptoms

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42
Q

Non-specific interstitial pneumonia responds better to anti-inflammatory drugs than IPF.

True or False?

A

True

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43
Q

Chest x-ray of NSIP

A

Small lungs with reticulo-nodular changes

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44
Q

CT NSIP

A

Ground glass changes

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45
Q

Management of NSIP

A
  • treatment of underlying condition
  • immunosuppression
  • long term oxygen therapy
  • palliative care
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46
Q

Prognosis of IPF and NSIP graph

A
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47
Q

Sarcoidosis (5) :

A
  • multisystem granulomatous disease
  • unknown cause/aetiology
  • characterised by non-caseating granulomas, often in multiple organs
  • sarcoidosis primarily affects the lungs
  • may resolve spontaneously, can recur and may progress to pulmonary fibrosis
48
Q

Aetiology of sarcoidosis:

A
  • infection:
    • remnants of microbial organisms
    • organic dusts, metals, minerals, solvents, pesticides, wood stoves
    • beryllium results in pulmonary granulomas
  • can run in families
49
Q

Clinical Presentation of Sarcoidosis: gender:

A
  • slightly higher in women
50
Q

Clinical Presentation of Sarcoidosis: age:

A
  • 20-50 (smaller peak larger than 60 years??)
51
Q

Clinical Presentation of Sarcoidosis: ethnicity:

A
  • higher in Scandinavian, Afro-Caribbean and African American women
52
Q

Clinical Presentation of sarcoidosis: incidence:

A
  • overall, 5/100,000
53
Q

Clinical Presentation of Sarcoidosis: Prevalence:

A
  • most common ILD in the UK
54
Q

Clinical Presentation of sarcoidosis: association:

A
  • TB
  • lymphoma
55
Q

Symptoms of acute sarcoidosis are:

  • duration
  • self-limiting
A
  • short duration
  • self-limiting
56
Q

Symptoms of chronic sarcoidosis:

  • duration
  • self-limiting
  • changes
A
  • longer duration
  • not self-limiting
  • persistent radiological changes
57
Q

Asymptomatic sarcoidosis would be diagnosed by an

A

incidental finding

58
Q

Symptoms and Signs of acute sarcoidosis:

A
  • fever
  • arthralgia: aching joints
  • anterior uveitis: painful red eyes
  • breathlessness
  • reduced appetite
  • night sweats
  • myalgia
  • erythema nodosum: painful red lumps on lower legs
  • lymphadenopathy: swollen lymph nodes
  • weight loss
  • Loefgren’s syndrome: erythema nodosum and BHL (bilateral hilar lymphadenopathy)
  • responds to oral prednisone
  • good prognosis
59
Q

Erythema Nodosum

A

painful red lumps on lower legs

60
Q

Anterior Uveitis

A

painful red eyes

61
Q

Chronic Pulmonary Sarcoidosis symptoms (10)

A
  • progressively worsening breathlessness
  • reduced exercise tolerance
  • cough
  • fatigue
  • anorexia
  • weight loss

Neurological Symptoms, bone pain, skin lesions (EN, lupus pernio in 25%), renal stones

Differential diagnoses: wide, cancer
Lung: any diffuse parenchymal disease

62
Q

If a patient comes in with acute exacerbation of IPF, with an infection, can be given

A
  • steroids
  • obvs antibiotics
  • steroids are not a long term solution
63
Q

Organ involvement in Sarcoidosis: What % have pulmonary involvement? What % have evidence of only lung involvement?

A
  • 90% have pulmonary involvement
  • 50% have evidence of only lung involvement
64
Q

Which organs can be involved in sarcoidosis? (

A
  • Skin
  • Joints and Muscles
  • Heart
  • Lacrimal glands
  • GI system
  • Lymph nodes
  • Liver
  • Bone
  • Kidneys
  • Pancreas
  • Eyes
  • Spleen
  • Salivary Glands
  • Nasal
  • Brain
65
Q

Clinical Approach in Sarcoidosis:

A
  • History: occupational, family
  • Examination
  • Chest X-Ray
  • Full lung function test
  • Blood tests
  • Urinalysis
  • ECG
  • Ophthalmologic examination
  • Tuberculin skin test
  • no definition diagnostic test for sarcoidosis
  • diagnosis made with compatible clinical, radiological and histopathological features
66
Q

Radiological Staging of Sarcoidosis:

A
  • Stage 0: Normal chest X ray (5-0%)
  • Stage I: Bilateral Hilar Lymph nodes (45%-65%) most common presentation, no treat lymph nodes
  • Stage II: Nodes + upper zone parenchymal disease (25=30%)
  • Stage III: parenchymal disease upper zones
  • Stage IV: end stage pulmonary fibrosis

Treatment begins with steroids in stage 2 and other medication

67
Q

Lung Function In Sarcoidosis:

A
  • restrictive pattern
  • airflow obstruction
  • can have both mixed restrictive and obstructive lung function
68
Q

Other Investigations in Sarcoidosis:

A
  • Serum ACE: produced by activated macrophages in granulomas
  • Hypercalcaemia due to increased Vitamin D by macrophages in granulomas
  • Hypercalciuria (high calcium in urine)
  • ACE = angiotensin converting enzyme
69
Q

Natural History of Sarcoidosis:

A
  • 2/3: remission within 10 years; stage 1 60% complete resolution within 2 years
  • 1/3: progression with significant organ damage
  • 1-5%: die secondary to respiratory failure, cardiac arrhythmia, neurosarcoidosis
69
Q

Natural History of Sarcoidosis:

A
  • 2/3: remission within 10 years; stage 1 60% complete resolution within 2 years
  • 1/3: progression with significant organ damage
  • 1-5%: die secondary to respiratory failure, cardiac arrhythmia, neurosarcoidosis
70
Q

Management of Acute Sarcoidosis:

A

Oral Prednisolone

71
Q

Management of Asymptomatic or Stage I Sarcoidosis:

A

observation

72
Q

Management of Stage II, III, IV Sarcoidosis:

A

prednisolone (steroid)

73
Q

Management of progressive Sarcoidosis:

A
  • immunosuppressive drugs
  • methotrexate
  • mycophenolate mofetil
74
Q

Main differential diagnosis for Stage I and Stage II Sarcoidosis is

A

differential diagnosis

75
Q

Differential diagnoses for Stage III and Stage IV include

A

other DPLD

76
Q

Pleural Disease (5):

A
  • Pleural Effusion
  • Pneumothorax
  • Haemothorax
  • Pleural Thickening
  • Mesothelioma
77
Q

Pleural Disease: Pleural Effusion:

A
  • fluid in the pleural space
  • exudate = thick pleural effusion
  • transudate = thin pleural effusion
  • sharp pain, which is worse on inspiration; pleural chest pain
78
Q

Pleural Disease: Pneumothorax:

A
  • air in pleural space, damage to parenchyma pops (anything could be emphysema)
  • spontaneous
  • traumatic: procedural, penetrating trauma, rib fracture, barotrauma
  • tall, skinny male (white)
79
Q

Pleural Disease: Haemothorax:

A
  • blood in the pleural space
  • traumatic injury
80
Q

Pleural Disease: Pleural Thickening:

A

pleura thicken

81
Q

Pleural Disease: Mesothelioma:

A

cancer of the pleura

82
Q

Pleural Effusion diagram

A
83
Q

Purpose of pleural fluid

A

lubricates pleural space, avoids friction when lungs move

84
Q

What is the amount of pleural fluid produced and cycled through the pleural space?

A

1L

85
Q
A
86
Q
A
87
Q

Pleural Aspiration (Thoracocentesis)

A
  • pleural fluid can be taken at the bedside with ultrasound guidance, local anaesthetic and a needle.
  • Pleural Drainage if patient is symptomatic
88
Q

Pleural Effusion: when examining a patient, what would you see?

A
  • complaining of chest pain maybe
  • breathless
  • not moving equally or symmetrically; eg left PE, left side of chest will not rise as much as the right side
  • reduced chest wall movement on the side of the effusion
  • dullness on percussion on the side of the effusion: this is the most reliable clinical finding
  • decreased tactile vocal fremitus and vocal resonance on the side of the effusion
  • bronchial breathing above the effusion
  • tracheal deviation away from side of a large effusion
89
Q

Name procedure

A

Pleural aspiration

90
Q

Diagnostic sample of pleural effusion:

A
  • Biochemistry: protein and lactate dehydrogenase (LDH)
  • Microbiology: microscopy, culture and sensitivity, acid and alcohol fast bacilli (AAFB) looking for infections (TB)
  • Cytology: abnormal cells (cancer)
91
Q

Types of pleural effusion:

A
  • exudate
  • transudate
92
Q

Type of pleural effusion is determined by:

A
  • protein
  • lactate dehydrogenase (LDH)
  • serum protein (ACE)
  • serum LDH
93
Q

What is used to differentiate between an exudate PE and transudate PE?

A

Light’s Criteria

94
Q

Exudate Pleural Effusion:
- pleural fluid protein/serum protein
- pleural fluid LDH/serum LDH
- pleural fluid LDH

A
  • > 0.5
  • > 0.6
  • > 2/3 of upper limit of normal serum LDH
  • more protein
95
Q

Transudate pleural effusion:
- pleural fluid protein/serum protein
- pleural fluid LDH/serum LDH
- pleural fluid LDH

A
  • < 0.5
  • < 0.6
  • < 2/3 of upper limit of normal serum LDH
  • less protein
96
Q

Causes of Pleural Effusion: Transudate:

A
  • low protein, reduced oncotic pressure, normal pleura
  • Congestive cardiac failure
  • Low albumin caused by nephrotic syndrome or liver failure
97
Q

Causes of Pleural Effusion: Exudate:

A
  • unhealthy pleura
  • malignancy
  • infection (pneumonia, tuberculosis, emphysema)
  • autoimmune disease
  • chylothorax (due to blocked lymphatics)
98
Q

Clinical Signs of Pleural Effusion

A
  • reduced chest wall movement on the side of the effusioni
99
Q
A

Pneumothorax

100
Q

Pneumothorax classification:

A
  • Primary: spontaneous
  • Secondary: due to underlying disease
101
Q

Large pneumothorax can cause mediastinum to be deviated.

True or False?

A

True

102
Q

Tension pneumothorax

A
  • tension builds up
  • can cause cardiac arrest
103
Q

Primary Pneumothorax Classification: Risk Factors:

A
  • tall and thin: to do with pressure gradients
  • asthma
  • collagen vascular disease
104
Q

Secondary Pneumothorax Classification: Risk Factors:

A
  • COPD
  • Pulmonary Fibrosis
  • Cystic Fibrosis
105
Q
A

Pleural Disease

106
Q

Chest Wall Disease

A
  • results in difficulty expanding the lungs
  • does not directly affect oxygenation, lung structure is normal but can’t ventilate
  • presents with progressively worsening breathlessness
107
Q

Chest Wall Disease Risk Factors:

A
  • obesity
  • neuromuscular: motor neuron disease, muscular dystrophy, poliomyelitis, diaphragmatic palsy
  • musculoskeletal: kyphosis, scoliosis
108
Q
A
109
Q

Combination of obstructive and restrictive diseases: smoking:

A
  • emphysema
  • pulmonary fibrosis
110
Q

Combination of obstructive and restrictive diseases: pulmonary sarcoidosis:

A
  • endobronchial sarcoidosis
  • fibrosis
111
Q

Combination of obstructive and restrictive diseases: Obesity in a smoker:

A
  • obstruction
  • restriction
112
Q

What will spirometry show in a restrictive lung disease?

1 = Increased FEV1
2 = Reduced FEV1
3 = Increased FVC
4 = Decreased FVC
5 = Decreased FEV1/FVC ratio

A

4

113
Q

Which of these statements about idiopathic pulmonary fibrosis is true?

1 = is a restrictive lung disease
2 = is a type of malignancy
3 = has an excellent prognosis
4 = can be treated with antibiotics
5 = is common in young females

A

1

114
Q

Which organ is most commonly affected by sarcoidosis?

1 = eyes
2 = kidneys
3 = lungs
4 = skin
5 = spleen

A

3

115
Q

Which of these statements about pleural effusion is true?

1 = can cause an obstructive lung disease
2 = can be caused by heart failure
3 = there is no pleural fluid in healthy lungs
4 = important to measure calcium in the fluid
5 = is always associated with a bad outcome

A

2

116
Q

Which of these conditions can cause a restrictive lung disease?

1 = kyphosis
2 = obesity
3 = pulmonary fibrosis
4 = sarcoidosis
5 = all of these

A

5