Bronchiectasis, IPF, sarcoidosis Flashcards

1
Q

Pathophysiology of bronchiectasis

A
  • The irreversible airway dilation seen in bronchiectasis occurs as a result of chronic inflammation caused by immune response to infection.
  • The dilation creates a vicious cycle in which the airway is more susceptible to repeat colonisation, causing recurrent episodes.
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2
Q

Main organisms associated with bronchiectasis

A
  • Haemophilus influenzae
  • Streptococcus pneumoniae
  • Staphylococcus aureus
  • Pseudomonas aeurginosa
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3
Q

Symptoms and signs of bronchiectasis

A
  • Symptoms
    • Persistent cough
    • Copious purulent sputum
    • Intermittent haemoptysis
    • Dyspnoea
  • Signs
    • Finger clubbing
    • Coarse inspiratory crepitations
    • Wheeze
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4
Q

Complications of bronchiectasis

A
  • Pneumonia
  • Pleural effusion
  • Pneumothorax
  • Haemoptysis
  • Cerebral abscess
  • Amyloidosis
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5
Q

Investigations for bronchiectasis

A
  1. CXR - may show tram tracks/fluid levels
  2. High resolution CT scan - gold standard, helps diagnose the condition and assess severity
  3. Spirometry
  4. Obtain a sputum sample for culture and microscopy
  5. Other investigations:
    • Bloods: FBC, U&Es, serum immunoglobulins, Aspergillus antigen test
    • Assessment of genetic disease if indicated
    • Bronchoscopy may be indicated
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6
Q

Management of bronchiectasis

A
  1. Airway clearance - taught by chest physiotherapist
  2. Assess response to beta-2 agonists and anticholinergic bronchodilator therapy
  3. Start empirical antibiotic therapy (which specifically includes inhaled antibiotics) while awaiting sputum microbiology - amoxicillin or clarithromycin
  4. If poor response consider lung surgery - lung resection surgery or lung transplant surgery
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7
Q

Treatment of ABPA

A
  • Oral corticosteroids (prednisolone) and itraconazole
  • Chest physiotherapy
  • Bronchodilators
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8
Q

Interstitial lung disease aetiology

A
  • The most common presentation is idiopathic pulmonary fibrosis
  • ILD can also occur as a result of medication use, autoimmune disease such as sarcoidosis or lupus, or occupational exposure to inhaled substances
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9
Q

Clinical features of idiopathic pulmonary fibrosis

A
  • Progressive dyspnoea that is worse on exertion
  • Dry cough
  • Finger clubbing
  • Bilateral inspiratory crackles on auscultation
  • PFTs demonstrate a restrictive type pattern in IPF
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10
Q

Complications of idiopathic pulmonary fibrosis

A
  • Respiratory failure
  • Increased risk of lung cancer
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11
Q

Investigations for idiopathic pulmonary fibrosis

A
  1. Conduct an adequate history and physical examination
  2. Perform blood tests - ABG, FBC
  3. PFTs and gas trasfer (DLCO reduced in IPF)
    • spirometry shows restrictive pattern (FEV1 normal/decreased, with FEV1/FVC increased)
  4. CXR
    • will show bilateral lower zone reticulonodular shadows
  5. Perform high resolution CT of the thorax
    • may demonstrate ground glass appearance
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12
Q

Management of idiopathic pulmonary fibrosis

A

Supportive care: oxygen, pulmonary rehabilitation, opiates, palliative care input.

All patients should be considered for current clinical trials or lung transplantation. It is strongly recommended that high-dose steroids are not used except where the diagnosis of IPF is in doubt.

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13
Q

Idiopathic pulmonary fibrosis prognosis

A

Average life expectancy ranging from 3 to 5 years from diagnosis

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14
Q

Define sarcoidosis

A

Sarcoidosis refers to a chronic granulomatous disorder of unknown cause affecting multiple organ systems and is characterised histologically by non-caseating granulomas.

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15
Q

Epidemiology of sarcoidosis

A
  • Worldwide prevalence of 6 per 100,000
  • Higher incidence of Afro-Caribbean and Scandinavian populations
  • Traditionally affects people in their second to fourth decade
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16
Q

Clinical features of sarcoidosis

A
  • Affects multiple systems
  • Generally presents with a flu like illness, in which patients may be pyrexial
  • Pulmonary features are the most common symptoms
  • Patients may present with a dry cough and dyspnoea
  • Cutaneous changes such as maculopapular rash and erythema nodosum may be seen on the legs
  • Associated with Lyme disease
  • Dry eyes and anterior uveitis may also be present
  • Bell palsy, polyarthritis and hypercalcaemia/hypercalciuria
17
Q

Investigations for sarcoidosis

A
  1. Chest x-ray
  2. Bloods: calcium (sometimes elevated), ESR and serum ACE may be evaluated; LFTs may also be deranged
  3. Urine: calcium (often elevated)
  4. Spirometry
  5. Tuberculin skin test
    • will be negative in patients with sarcoidosis
18
Q

Pathophysiology of asbestos related lung disease

A
  • Broadly classified, asbestos exposure can lead to benign pleural plaques and interstitial lung disease (asbestosis)
  • It can predispose an individual to the development of lung cancer, particularly cancer of the pleura, known as mesothelioma
19
Q

Which individuals would be considered high risk due to asbestos exposure?

A

Those who have worked in shipyards, train lines and in the plumbing industy.

20
Q

What should be discussed with patients who develop asbestos related lung disease?

A

They may be eligable for financial compensation

21
Q

Describe benign pleural plaques.

How can the be visualised?

A

These plaques are not premalignant and are generally asymptomatic. This is the most common form of asbestos related lung disease.

They may be visualised on CXR but CT scans are a lot more precise.

22
Q

What is asbestosis?

Treatment of asbestosis

A

This refers to interstitial lung disease caused by asbestos exposure; it is directly proportional to the amount and duration of the exposure.

There is no specific therapy available. Treatment should focus on minimising further exposure, smoking cessation and managing any other comorbid lung disease.

23
Q

Mesothelioma symptoms

A
  • Chest pain
  • Shortness of breath
  • Weight loss
24
Q

Mesothelioma investigations and treatment

A
  • Investigations include a CXR, CT scan and pleural biopsy
  • Treatment is supportive, with pleural surgery being indicated for palliative symptomatic relief