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Medicine - Year 2 > The respiratory system - Breathlessness: restrictive lung disease > Flashcards

The respiratory system - Breathlessness: restrictive lung disease Flashcards

1
Q

Name the 5 R’s of inflammation and repair

A
  1. Recognition - of the injurious agent
  2. Recruitment - of the leukocytes
  3. Removal - of the agent
  4. Regulation - of the response
  5. Resolution/repair
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2
Q

What is acute respiratory distress syndrome (ARDS)?

A

ARDS is defined as non-cardiogenic pulmonary oedema and diffuse lung inflammation, typically secondary to an underlying illness.

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3
Q

Describe the pathophysiology of ARDS

A

ARDS involves a diffuse bilateral alveolar injury, as a result of inflammation mediated by inflammatory mediators such as TNF-α, IL-1 and IL-8.

The resulting endothelial injury activates neutrophils in the pulmonary capillaries, releasing reactive oxygen species and proteases that damage the alveolar endothelium and type 2 alveolar cells.

As a result, the vascular permeability increases and the lung surfactant is lost.

Fluid accumulation in the alveoli causes pulmonary oedema and subsequently hypoxaemia.

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4
Q

Causes of acute respiratory distress syndrome

a) Direct lung injury

b) Indirect lung injury

A

a)
Common causes
- Pneumonia
- Aspiration of gastric contents

Uncommon causes
- Pulmonary confusion
- Near drowning
- Inhalation injury
- Fat emboli
- Repercussion injury

b)
Common causes
- Sepsis
- Severe trauma with shock
- multiple transfusion

Uncommon causes
- Post cardiac surgery
- Pancreatitis
- Drug overdose
- After massive transfusion

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5
Q

Clinical features of ARDS

a) Symptoms

b) Signs

A

a) Acute onset respiratory failure which fails to improve with supplemental oxygen, the symptoms of which include severe dyspnoea, tachypnoea, confusion, and presyncope

b) Fine bibasal crackles, but no other features of heart failure

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6
Q

CXR changes in ARDS

A

Bilateral alveolar infiltrates, without other features of heart failure (such as cardiomegaly and Kerley B lines).

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7
Q

What is the histological pattern of acute respiratory distress syndrome

A

Diffuse alveolar damage

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8
Q

Management of ARDS

A
  • Ventilatory support: a low tidal volume is associated with better outcomes.
  • Haemodynamic support to maintain mean arterial pressure >60 mmHg.
  • DVT prophylaxis.
  • Nutritional support with enteral/parenteral means if necessary.
  • Regular repositioning of patient for pressure ulcer prophylaxis.
  • Antibiotics need only be administered if an infectious cause for the ARDS is identified (such as pneumonia or sepsis).
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9
Q

What is Type 1 respiratory failure?

A

Hyperaemia (PaO2 < 8 kPa) and a normal or low CO2

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10
Q

What is type 2 respiratory failure?

A

Hyperaemia (paO2 < 8 kPa) and hypercapnia (PaCO2 > 6.5 kPa)

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11
Q

Name 5 causes of T1RF

A

Ventilation-perfusion mismatch

Decreased atmospheric pressure

Shunt

Pneumonia

ARDS

Pulmonary embolism

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12
Q

Why does T2RF occur?

A

The rise in PaCO2 is no longer matched by an increase in alveolar ventilation. This can be because:

  • Ventilatory drive is insufficient
  • The work of breathing is excessive
  • The lungs are unable to pump air in and out

As a result, patients are unable to ‘blow off’ the excess CO2 causing PaCO2 levels to rise

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13
Q

a) What can acute T2RF lead to?

b) 3 common causes of acute T2RF

A

a) Respiratory acidosis (as the excess CO2 is converted to carbonic acid in the blood stream)

b)
Exacerbations of COPD, severe asthma, CF and bronchiectasis

Respiratory depressants (e.g., opiate overdose)

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14
Q

a) How is chronic T2RF evidenced on ABGs

A
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15
Q

a) How is chronic T2RF evidenced on ABGs

b) 4 common causes

A

a) Normal pH, elevated PaCO2 and elevated bicarbonate levels

b)
- COPD
- Asthma
- Chronic neurological disorders (e.g.m motor neurone disease)
- Chronic neuromuscular disorder (e.g., myopathies)
- Chest wall diseases
- Obesity hypoventilation syndrome

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16
Q

Clinical features of hypoxia (T1RFF and T2RF)

A
  • Dyspnoea
  • Restlessness and agitation
  • Confusion
  • Cyanosis
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17
Q

Clinical features of hypercapnia (T2RF only)

A
  • Headaches
  • Drowsiness
  • Confusion
  • Tachycardia with a bounding pulse
  • CO2 retention hand flap
  • Peripheral vasodilation
  • Papilloedema
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18
Q

Describe the investigations for respiratory failure

A

Definitive diagnosis – ABG sampling

Oxygen saturation monitoring

Further investigations to find underlying cause:
- Blood tests and blood cultures
- Chest radiograph
- Sputum culture
- Bedside spirometry testing
- ECG: look for cardiac arrhythmias secondary to hypoxaemia and acidosis

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19
Q

What is the defenitive diagnostic investigation for respiratory failure?

A

ABG sampling

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20
Q

Describe the management for respiratory failure

A
  1. Treat the underlying cause
  2. Adequate oxygenation and respiratory support (type of support required depends on the type and severity of respiratory failure):
  • Oxygen delivery: via nasal canula, a face mask, a venturi mask, or a non-rebreather mask
  • Non-invasive ventilation: e.g., continuous Positive Airway Pressure (CPAP) ventilation or a Bilevel Positive Airway Pressure (BiPAP) ventilation
  • Invasive ventilation: via an endotracheal tube or a tracheostomy
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21
Q

a) Why must you take special care in oxygenation in patients at risk of T2RF e.g., severe COPD patients

b) What is the target

A

Respiratory drive in these patients has become relatively insensitive to high PaCO2 and so their drive to breathe is stimulated by low PaO2 (hypoxic drive to breath).

Over oxygenation leads to suppression of ventilation and PaCO2 may rise rather than fall (oxygen-induced hypercapnia)

This can result in respiratory acidosis which can be fatal if not recognized and treated aka decompensated T2RF

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22
Q

Name 3 estrictive extra-pulmonary disease affecting:

a) neuromuscular

b) Chest wall

A

a)
- Diaphragmatic paralysis
- Cervical spine injury
- Myasthenia Gravis
- Guillain-Barre
- muscular dystrophies

b)
- Kyphoscoliosis
- Obesity
- Ankylosing spondylitis

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23
Q

What is interstitial lung disease?

A

Refers to a family of conditions with shared characteristics of interstitial inflammation, fibrosis and/or cellular changes in the absence of infection of malignancy

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24
Q

ILD can be divided into main classifications. What are they?

A
  1. Known cause association
  2. Idiopathic interstitial pneumonias (IIP)
  3. Granulomatous
  4. Other ILDs (cystic)
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25
Q

Provide the subtypes of ILD due to known cause association

A
  • Drug side-effects
  • Connective tissue disease
  • Occupational ILD
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26
Q

Provide the subtypes of ILD due to idiopathic interstitial pneumonias

A

Idiopathic pulmonary fibrosis

Other IIPs
- Respiratory bronchiolitis (RBILD)
- Non-specific pneumonia (NSIP)
- Desquamative interstitial pneumonia (DIP)
- Lymphocytic intersiital pneumonia (LIP)
- Cryptogenic organising pneumonia (COP)
- Acute intersitital pneumonia

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27
Q

Provide the subtypes of granulomatous ILD

A
  • Sarcoidosis
  • Hypersensitivity pneumonitis (Extrinsic allergic alveolitis)
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28
Q

Provide the subtype of other ILDs (cystic)

A

Lymphangio-leimyomatosis (LAM)

Langerhans cell histiocytosis X (LCH)

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29
Q

What is the most common form of idiopathic interstitial pneumonia

A

Idiopathic pulmonary fibrosis

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30
Q

Clinical features of idiopathic pulmonary fibrosis

a) Symptoms

b) Signs

A

a)
- Exertion dyspnoea
- Dry cough
- Fatigue
- Arthralgia

b)
- Fine bi-basal end inspiratory crackles
- Clubbing
- Reduced chest expansion
- Acrocyanosis ( bluish discolouration of the extremities due to decreased amount of oxygen delivered to the peripheral part)

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31
Q

Describe the conservative, medical and surgical management of IPF

A

Conservative
- supportive care
- Smoking cessation
- Pulmonary rehabilitation

Medical
- Oxygen therapy: LTOT and if symptoms persist at rest, low dose opiates or benzodiazepines may be commenced
- DMARDS: Pirfenidone and nintedanib

Surgical
- lung transplant

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32
Q

Complications of IPF

A
  • T2RF
  • Increased risk of lung cancer
  • Cor pulmonale
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33
Q

Prognosis of IPF

A

Disease progression and prognosis varies greatly between individuals and is difficult to predict.

There is a median survival of 3 years after diagnosis

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34
Q

Only 2 DMARDs are linseed to treat IPF. What are they?

A

Pirfenidone

Nintedanib

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35
Q

Pirfenidone

a) MOA

b) Side-effetcs

A

a) anti-inflammatory and anti-fibrotic

b) Nausea and photosensitivity rash

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36
Q

Nintedanib

a) MOA

b) Side-effetcs

A

a) Acts on 3 growth receptor factors implicated in the development of iPF

b) Nausea

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37
Q

Describe the CXR changes in IPF

A

Bilateral lower zone reticule-nodular shadowing may be seen

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38
Q

Describe the HRCT changes in IPF

A

Honeycombing

Reticular opacities

Traction bronchiectasis

Emphysema

Loss of lung volume

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39
Q

Non-specific interstitial pneumonia (NSIP)

a) Epidemiology

b) What may it be associated with

c) HRCT changes

A

a) 40-50 years, M=F

b) may be associated with CTDs

c) Ground-glass, reticular shadowing

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40
Q

Respiratory bronchiolitis ILD (RBILD)

a) Why does it occur?

b) HRCT changes

A

a) Exaggerated bronchiolitis response to smoking

b) patchy ground glass, centrilobular micro nodules, regional attenuation

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41
Q

Desquamative intersiitital penumonia

a) Who does it occur in?

b) HRCT changes

A

a) Heavy smokers

b) Ground glass opacities, bronchial thickening

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42
Q

Hypersensitivity pneumonitits

a) What is it?

b) Breath sounds on examination

c) What is seen on biopsy?

d) Treatment

A

a) Type III and type IV hypersensitivity reaction to antigens, causing inflammation of the alveoli, bronchioles, peri-bronchioles

b) Crackles, wheeze, squeaks

c) Granulomas in alveolar space and bronchioles

d) Removal froma antigen, steroids

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43
Q

Give the causes of hypersensitivity pneumonitis and also the corresponding disease name

A

Actinomycetes in hay and or straw - farmers lung

Avian proteins fro feathers and droppings - bird fanciers lung

Mouldy barley - malt workers lung

Aspergillus in mushroom compost - mushroom workers lung

Actinomycetes n water reservoirs (air conditioning units) - ventilation pneumonitis

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44
Q

Lymphagioleimyomatosis (LAM)

a) What is its?

b) Epidemiology

c) What does it also cause

d) Treatment

A

a) Abnormal growth of smooth muscle cells, especially in the lungs and lymphatic system

b) Females, mid 30s

c) chylothorax (lymph formed in the digestive system (chyle) accumulates in your chest cavity)

Angiomyolipomas (benign tumors formation in kidneys)

d) Sirolimus (aka rapamycin is a immunosuppressant)

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45
Q

Langerhans cells histiocytosis

a) What is it?

b) Epidemiology

c) Treatment

A

a) A multi-system disorder in which excess immune system cells called Langerhans cells build up in the body.

b) Young smokers, (20-40yrs), M:F

b) Smoking cessation, steroids

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46
Q

Langerhans cells histiocytosis

a) What is it?

b) Epidemiology

c) Treatment

A

a) A multi-system disorder in which excess immune system cells called Langerhans cells build up in the body.

b) Young smokers, (20-40yrs), M:F

b) Smoking cessation, steroids

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47
Q

Describe the investigations in idiopathic pulmonary fibrosis

A

Bloods - FBC, Renal function, lots, cap

ABG

Lung function tests - spirometry, diffusion capacity of the lung for carbon monoxide (DLCO)

Imaging
- CXR
- High-resolution CT

Biopsy and cytology
- Bronchoalveolar lavage and/or transbronchial biopsy
- Surgical lung biopsy

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48
Q

a) Describe the spirometry changes in ILDs e.g., IPF

b) Which IPF shows a variable pattern in spirometry

A

a) Associated with a restrictive pattern

FEV1 and FVC both reduced

FEV1/FVC > 0.7

b) Sarcoidosis

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49
Q

How does ILDs e.g, IIPF affect the diffusing capacity of the lung for carbon monoxide decreased (DLCO)

A

Decreases

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50
Q

Describe the HRCT changes in ILD

A
  • Ground glass
  • Consolidation
  • Non-specific
  • Airspace shadow
  • Fibrosis: Reticular shadowing, traction bronchiectasis, honeycombing
51
Q

Describe the conservative and pharmacological treatment of ILD

A

Conservative
- Education
- Patient support group
- Pulmonary rehabilitation
- Palliative care

Pharmacological
1. Fibrosis - anti-fibrotic for IPF
2. Inflammation - corticosteroids, methotrexate/hydoxychlroroquine, azathioprine/mycophenolate,cyclophosphamide

52
Q

Pulmonary fibrosis

a) Signs

b) Causes

A

a) Fine end-inspiratory crepitations

b)
Lung damage: Infarction, pneumonia, tuberculosis

Irritants: Coal dust, silica

Diffuse parenchymal lung disease: Idiopathic pulmonary fibrosis and extrinsic allergic alveolitis

Connective tissue disease: RhA, SLE Systemic Sclerosis and Sjögren’s syndrome

Medications: Amiodarone, Nitrofurantoin, Bleomycin, Methotrexate

Hypersensitivity pneumonitis: Exposure to birds or moulds

53
Q

Describe the differential diagnosis for chronic dyspnoea

A

Respiratory
- Airways: asthma, COPD
- Parenchymal: ILD
- Pleura and chest wall
- Pericardium: effusion/constriction

Cardiac
- Muscle: ischaemic heart diseases, cardiomyopathies
-Valve: stenosis, regurgitation
- Rhythm: tachyarrythmia, bradycardia
- Pericardium: effusion/constriction

Other
- Thyrotoxicosis
- Anaemia/haemorrhage
- Obesity
- Anxiety

54
Q

What is sarcoidosis?

A

Sarcoidosis is a rare multi system granulomatous disorder of Unkown aetiology

55
Q

Describe the epidemiology of sarcoidosis

A
  • More common in women with a 2:1 female-male-ratio
  • Most often diagnosed between 25-40 years
56
Q

Describe the pathophysiology of sarcoidosis

A

Requires two things
- Genetically primed individual
- Exposure to susceptible antigen

Antigen and host proteins from a poorly soluble compound

APCs activate macrophages and dendritic cells producing cytokines

T-helper type 1 promoted by cytokines help form epithelium granulomas

57
Q

A combination of genetic predisposition and environmental can predispose patient sot sarcoidosis. Give an example for the following:

a) Genetic predispositions

b) Environmental exposures

A

a)
- Multiple areas of the HLA region
- BTNL2
- ANXA11

b)
- Beryllium, silica, other dusts
- Mycobacterium tuberculosis and Propionibacterium catalases
- Moulds

58
Q

Describe the presentation of acute sarcoidosis

A

Flu-like illness

Lofgren’s syndrome (Bilateral hilar lymphadenopathy, erythema nodosum, arthralgia)

59
Q

Give the different manifestations of sarcoidosis

A
  • Pulmonary
  • Ocular
  • Cutaneous
  • Constitutional
  • Musculoskeletal
  • Abdominal
  • Gi and genito-urinary
  • Cardiac
  • Hereford’s syndrome (aka uveoparotid fever)
  • Lofgren’s syndrome (acute sarcoidosis)
60
Q

Describe the symptoms of different manifestations of sarcoidosis

A

Ocular
- Eye pain
- Visual disturbance
- Vision loss

Pulmonary
- Breathlessness (exertion)
- Cough
- Wheeze

Skin
- Rashes (erythema nodosum, subcutaneous nodules, lupus pernio)

Joints
- Joint pain and swelling
- Joint stiffness
- Skin rashes
- Myalgia and weakness

Neurological
- Seizures
- Headaches
- Cranial nerve palsies

Cardiac
- Arrhythmias
- Ventricular failure
- Sudden cardiac death

GI/urogenital
- Abdominal pain
- Haematuria
- Deranged LFTs
- Change in bowel habit
- Infertility

Incidental Hypercalcaemia

Constitutional
- Fatigue

61
Q

Name the top 3 manifestations in sarcoidosis

A
  1. Pulmonary (90%)
  2. Ocular (30-60%)
  3. Cutaneous (25%)
62
Q

Pulmonary sarcoidosis

a) Manifestations

b) Symptoms

c) Signs

d) Complications

A

a)
- Lymphadenopathy (mediastinal and hilar): bilateral and symmetrical
- Parenchymal disease: ILD
- Airway disease: asthma-like symptoms/bronchial hyper-reactivity, airway stenosis

b) Progressive breathlessness

c)
- Fine inspiratory crackles
- Exertional desaturations
- Clubbing

d) Pulmonary artery hypertension, cor pulmonale

63
Q

Ocular sarcoidosis manifestations

A
  • Uveitis
  • Keratoconjunctivitis sicca
  • Adnexal granuloma
  • Secondary glaucoma
64
Q

Cutaneous sarcoidosis manifestations

A

Papular sarcoidosis: multiple papules develop, generally on the head and head areas

Erythema nodosum

Lupus pernio: a violaceous, nodular rash distributed over the nose and cheeks

65
Q

Describe the presentation (briefly) of chronic sarcoidosis

A

Progressive symptoms - e.g., dyspnoea, cough

Associated extra-pulmonary disease and organ dysfunction

66
Q

Describe the different manifestations of sarcoidosis

A

Constitutional - fatigue, weight loss, arthralgia and low grade fever. Lymphadenopathy and enlarged parotid glans

Calcium metabolism - Hypercalcaemia/vit D deficiency

Pulmonary -Llymphadenopathy, ILD, airway obstruction and stenosis

Ocular - uveitis, keratoconjuctivitis sicca, adnexal granuloma, secondary glaucoma

Cutaneous - papular sarcoidosis, erythema nodosum, lupus pernio

Musculoskeletal - bone cysts, arthralgia, arthritis and enthesitis

Renal disease - occurs secondary to hypercalcaemia which ay cause nephrocalcinosis

GI and genitourinary
Abdominal - hepatomegaly, splenomegaly, renal stones, IBD mimic, infertility

Neurological - mononeuropathy/polyneuropathy, meningitis, raised intracranial pressure, psychiatric problems

Cardiac - arrhythmias, cardiomyopathy and HF

Hereford’s syndrome (aka uveoparotid fever) - characterised by uveitis, parotid swelling, fever, and facial nerve palsy

Lofgren’s syndrome (acute sarcoidosis) - bilateral hilarity lymphadenopathy, erythema nodosum, arthralgia and fever

67
Q

What is Heerfordt’s syndrome (aka uveuoparotid fever) characterised by?

A

Uveitis, parotid swelling, fever, and facial nerve palsy

68
Q

What is Lofgren’s syndrome characterised by?

A

Bilateral hilar lymphadenopathy, erythema nodosum, arthralgia and fever.

69
Q

What important extra-pulmonary manifeststations require treatment?

A

Cardiac
- Dyspnoea
- Collapse
- Cardiomyopathy
- Sudden cardiac death/arrhythmias
- Pulmonary hypertension

Calcium metabolism
- Hypercalcaemia
- Hypercalciuria/renal calculi

Ophthalmic
- Uveitis
- Visual loss

Neurological
- Seizures
- Aseptic meningitis
- Cranial nerve palsies

70
Q

Give the investigations for sarcoidosis and there indications

A

Bedside
- Observations
- Mantoux test: To distinguish from TB, as TB and sarcoidosis cause cavitating lesions

Bloods
- FBC: lymphopenia, anaemia
- Renal function: assess for renal disease
- LFTs: assess for liver disease
- ESR/CRP
- Bone profile: assess for hypercalcaemia
- Serum ACE: raised in around 70%. However, it is NOT specific
- Immunoglobulins: associated with Ig deficiencies (particular IgA)

ECG
- assess for arrhythmias

Imaging
- CXR
- HRCT

Special
- Spirometry
- Tissue biopsy: to confirm diagnosis
- Lumbar puncture: if CNS disease suspected

71
Q

Describe the CXR changes in sarcoidosis

A
  • Bilateral hilar and mediastinal lymphadenopathy
  • Reticulonodular opacites
  • Airspace opacities
  • Pulmonary fibrosis
72
Q

Describe the HRCT changes in sarcoidosis

A
  • Lymphadenopathy
  • Diffuse nodularity
  • Ground glass opacification
  • Fibrosis (upper lobe predominance)
73
Q

Scaffold staging may be used to stage pulmonary sarcoidosis based upon chest radiograph findings.

Describe the chest radiograph findings for the following stages.

a) Stage 0

b) Stage I

c) Stage II

d) Stage III

e) Stage Iv

A

a) Normal

b) Bilateral hilar lymphadenopathy (BHL)

c) BHL with pulmonary infiltrates/parenchymal changes

d) pulmonary infiltrates/parenchymal changes without BHL

e) Advance pulmonary fibrosis

74
Q

a) What investigation confirms the absolute diagnosis of sarcoidosis

b) Is it always required?

A

a) Tissue biopsy

b) No

75
Q

In what instant may a tissue biopsy for sarcoidosis not be required?

A

If Lofgren’s syndrome or chronic stable CT changes typical of sarcoidosis and after monitoring patient is well

76
Q

What type of lung biopsy is best for predominantly nodal disease (Stage I/II) sarcoidosis

A

Endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA)

+/- bronchoalveolar lavage (BAL)

77
Q

What type of lung biopsy is best for predominantly parenchymal disease sarcoidosis

A

Transbronchial biopsy

+/- bronchoalveolar lavage (BAL)

78
Q

What type of lung biopsy is best for mixed nodal and parenchymal sarcoidosis

A

Endobronchial ultrasound - Transbronchial (EBUS-TBNA)

+/- Bronchoalveolar lavage (BAL)

79
Q

Name the classic histopathological finding in sarcoidosis

A

Non-caseating granuloma

80
Q

Describe the conservative management of sarcoidosis

A
  • Offered support and counselling
  • Optimisation of lifestyle factors e.g., smoking cessation, weight loss, dietary advice
81
Q

Give the indications stated by the British Thoracic Society of when treatment should be started if sarcoidosis

A

Potential danger of a fatal outcome or permanent disability

OR

Unacceptable loss of QoL

82
Q

Describe the 1st, 2nd and 3rd line pharmacological management of pulmonary disease in sarcoidosis

A

1st line - steroids
- High dose, typically 20-40mg daily for 4-6 weeks which is tapered down over 6-18 months until a maintenance dose, typically 5-10mg a day is reached

2nd line - Classical immunosuppressants e.g., Methotrexate 10-15mg (+ Folic acid) (best evidenced 2nd line), Azathioprine, Hydroxychloroquine, Leflunomide and Mycophenolate mofetil

3rd line - Biologics e.g., anti-TNF such as infliximab

83
Q

What are the indications for second line (immunosuppressant therapy) treatment of pulmonary sarcoidosis

A
  • Significant side-effects from steroids
  • Co-morbidities putting patients at greater risk of steroid-related side effects

Progressive pulmonary disease / unacceptable ongoing symptoms

Inability to taper steroids to an acceptable maintenance dose

Patient aversion to steroid therapy - may be used as initial therapy

84
Q

Describe the surgical options for pulmonary sarcoidosis and the indications

A

Lung transplant - indicated in those with:
- Advanced pulmonary fibrosis
- Pulmonary hypertension

85
Q

Describe the prognosis of sarcoidosis

A

Acute and incidental findings have better outcomes

Severe manifestations are associated with higher mortality

86
Q

Name the 5 most common occupational respiratory diseases

A
  • Occupational asthma
  • COPD
  • Asbestos related
  • Silicosis
  • Pneumoconiosis
  • Extrinsic Allergic Alveolitis (EAA)
87
Q

Name 5 asbestos related diseases

A
  • Pleural plaques
  • Diffuse pleural thickening
  • Malignant mesothelioma
  • Asbestosis
  • Lung cancer
  • Laryngeal cancer
88
Q

a) What is asbestos?

b) Describe how the different size and length of asbestos play a role in asbestosis

A

a) Asbestos is an industrial term for naturally occurring fibre silicates

b)

  • Particles < 10µm may penetrate alveoli
  • Long straight fibres can reach the alveoli despite their length being > than 10µm
  • Fibres > 5 µm more resistant to clearance by phagocytosis
89
Q

a) What is asbestosis and what is characterised by?

b) When do symptoms of asbestosis develop?

A

a) Asbestosis is a form of pneumoconiosis caused by inhalation of asbestos fibres, which is characterised by scarring and inflammation

b) Symptoms typically start to develop decades following exposure to asbestos

90
Q

Clinical features of asbestosis

A
  • Dyspnoea
  • Non-productive cough
  • Weight loss
  • Fine inspiratory crackles
  • Finger clubbing
  • Susceptibility and deterioration associated with smoking
91
Q

Describe the investigations for asbestosis

A
  • Spirometry
  • Transfer factor
  • CXR
  • HRCT
  • Lung biopsy
92
Q

Describe the findings for following investigations in asbestosis

a) Spirometry

b) Transfer factor

c) CXR

d) HRCT

e) Lung biopsy

A

a) Usually a restrictive pattern

b) Reduced

c) Fine nodular shadowing

d) Fibrosis

e) Interstitial fibrosis and asbestos bodies

93
Q

Describe the management of asbestosis

A
  • Supportive
  • Smoking cessation
94
Q

Mesothelioma

a) What does it have a strong association with?

b) When do symptoms develop?

c) Symptoms

d) Name 3 industries that increase risk of mesothelioma

e) Prognosis

A

a) Asbestos exposure

b) > 20 years after asbestos exposure

c) Chest pain, SOB

d)
- Plumbers/ electricians/ maintenance
- Demolition
- Construction
- Plumbing and pipe lagging
- Braking lining manufacture/mechanics
- Railway engineering
- Asbestos mining

95
Q

If occupational asthma is diagnoses, who should it be reported by and to who?

A

If diagnosed it should be reported by the employer to HSE under RIDDOR (Reporting of injuries, Diseases and Dangerous Occurrences Regulations)

96
Q

Presentation of occupational asthma

A

Initially symptoms of wheee, dyspnoea, and chest tightest at work or after work, improving over week

Overtime this pattern can be lost

97
Q

List 5 occupations and their causes that may lead to occupation asthma

A

Paint spraying - Isocyanates

Cleaners - cleaning agents

Bakers - flour

Laboratory workers - animals

Electronics - rosin flux fume

Healthcare - latex

Carpentry - wood dust

Detergent manufacture - enzymes

Hairdressers - persulfate salts

98
Q

What serial PEFR is diagnostic of occupational asthma?

A

Serial peak flow of > 20% variation across shift and lower/more variable peak flow on workdays

99
Q

Describe the management of occupational asthma

A
  • Management is that of the BTS guidelines for asthma
  • Elimination of exposure (symptoms usually resolve after this)
100
Q

What is pneumoconiosis

A

A group of diseases caused by inhalation of mineral dust

101
Q

Give 5 pneumoconiosis and the mineral dust that causes it

A

Coal workers pneumoconiosis - coal dust

Asbestosis - asbestose

Silicosis - silica

Slate works pneumoconiosis - slate

Kaolin - china clay

Stannosis - tine ore

Siderosis - iron oxide

102
Q

Simple coal worker pneumoconiosis

a) symptoms

b) Spirometry changes

c) CXR changes

A

a) Often symptomatic

b) Can show obstructive (emphysema) or restrictive (fibrosis) pattern

c) Nodular opacities

103
Q

Progressive massive fibrosis

a) What is it?

b) Symptoms

A

a) Fibrotic masses, in upper or middle zones, 3-10cm diameter and can cavitate

b) Dyspnoea and productive cough (black sputum)

104
Q

Acute silicosis (silicoproteinosis)

a) Symptoms

b) CXR changes

c) Treatment

A

a) Progressive dyspnoea and cough

b) Pulmonary oedema

c) No specific treatment

105
Q

Name 3 causes of occupational COPD

A

Mineral dusts e.g., coal, silica, cement

Organic dusts e.g., wood, flour, cotton (Byssinosis)

Chemical e.g., isocyanates, cadmium, welding fumes

106
Q

Name 3 occupational respiratory infections

A

Viral respiratory tract infections

Tuberculosis

Legionnaires disease

Psittacosis (Chlamydia psticcaci)

107
Q

Name 3 occupational causes of lung cancer

A
  • Passive smoking
  • Mineral dusts
  • Asbestos
  • Arsenic
  • Nickel
108
Q

Name the 4 common (histological) subtypes of rheumatoid arthritis - ILD

A
  • Idiopathic/usual interstitial pneumonia (UIP)
  • Nonspecific interstitial pneumonia (NSIP)
  • Lymphocytic intersiital pneumonia (LIP)
  • Organising pneumonia (COP)
109
Q

Name the 4 common (histological) subtypes of RA - ILD

A
  • Idiopathic/usual interstitial pneumonia (UIP)
  • Nonspecific interstitial pneumonia (NSIP)
  • Lymphocytic intersiital pneumonia (LIP)
  • Organising pneumonia (COP)
110
Q

Which lobe is affected in RA-ILD?

A

Lower lobe

111
Q

What is there treatment for RA-ILD

A
  • Methotrexate
  • Anti-TNF alpha
  • Corticosteroids (best for with with organising pneumonia subtype)
112
Q

Which type of systemic sclerosis is ILD most commonly associated with?

A

Diffuse cutaneous SSc

113
Q

Which antibody is strongly associated with ILD in systemic sclerosis

A

Scl-70

114
Q

ILD in systemic sclerosis

a) Symptoms

b) Management

A

a) Breathlessness and cough

b)
- Cyclophosmade (most effective)

Various immunnossuppressive agents used: Steroids
- Colchicin
- D-penicillamine
Chloambucil

115
Q

ILD in systemic sclerosis

a) Symptoms

b) Management

A

a) Breathlessness and cough

b)
- Cyclophosmade (most effective)

Various immunosuppressive agents used: Steroids, colchicine, D-penicillamine, Chlorambucil

116
Q

Lupus pneumonitis (ILD - SLE)

a) Signs on histology

b) What must you exclude in the ddx and why?

c) Treatment

A

a) Feature of interstitial pneumonitis

b) Important to exclude infections as they might present in similar fashion as acute lupus pneumonitis

c) Heavy immunosuppression and plasmapheresis (Corticosteroids + Azathioprine or cyclophosphamide)

117
Q

Name 3 ways that ILD in dermatomyositis may present

A
  1. Rapidly progressive Acute interstitial pneumonia/Hamman-Rich syndrome
  2. Slowly progressing symptoms of usual interstitial pneumonia ) or cryptogenic organising pneumonia
  3. Abnormal CXR/HRCT/PFT but no respiratory symptoms
118
Q

ILD - Sjrogen’s syndrome

a) Respiratory symptoms

b) Treatment

A

a) Cough and breathlessness

b)
Immunosuppressants: corticosteroids and azathioprine/cyclophosphamide

119
Q

ILD - mixed connective tissue disease

a) What is the ILD spectrum of this similar to?

b) In which patients is the degree of lung fibrosis more severe in?

c) Treatment

d) In what patients is treatment poor in?

A

a) ILD spectrum similar to that seen in SSc

b) The degree of lung fibrosis is severe in patients with SSc features

c) Corticosteroids and cyclophosphamide

d) Response not very good in patients with SSc like disease

120
Q

Methotrexate lung injury (methotrexate interstitial pneumonitis)

a) Symptoms

b) Pulmonary function test findings

c) CXR changes

d) HRCT changes

e) Treatment

A

a) cough, dyspnoea, fever

b) restrictive defect, low diffusion capacity

c) alveolar infiltrates, reticulonodular shadowing, predominantly diffuse or lower lobe involvement

d) Patchy ground glass shadowing

e) Discontinuation of therapy alone, corticosteroids

121
Q

Is there a correlation between ILD progression and control of connective tissue disease?

A

No

122
Q

Name the roles involved in the MDT care of ILD

A
  • ILD consultant
  • ILD CNS
  • Radiologist
  • Thoracics
  • Pathologists
  • Pharmacists
  • Dieticians
  • Physiotherapists
  • OTs
123
Q

Name the roles involved in the MDT care of ILD

A
  • ILD consultant
  • ILD CNS
  • Radiologist
  • Thoracics
  • Pathologists
  • Pharmacists
  • Dieticians
  • Physiotherapists
  • OTs
  • Pulmonary rehab

Medicine - Year 2 (27 decks)

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