Clinical features and restrictive lung disease Flashcards

1
Q

what is the physiological definition of restriction?

A

FVC <80% of the predicted normal

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2
Q

what diseases affect the lungs, that are also ILDs? (diseases that cause restrictive disorders)

A
  • idiopathic pulmonary fibrosis
  • sarcoidosis
  • hypersensitivity pneumonitis
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3
Q

what diseases affect the pleura? (diseases that cause restrictive disorders)

A
  • pleural effusions
  • pneumothorax
  • pleural thickening
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4
Q

what diseases have skeletal causes? (diseases that cause restrictive disorders)

A
  • kyphoscoliosis
  • ankylosing spondylitis
  • thoracoplasty
  • RIB fractures
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5
Q

what diseases have muscles causes? (diseases that cause restrictive disorders)

A
  • amyotrophic lateral sclerosis
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6
Q

what sub-diaphragmatic have skeletal causes? (diseases that cause restrictive disorders)

A
  • obesity

- pregnancy

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7
Q

what are ILDs? (HINT: there’s a shit tonne of them)

A

> 200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis (disease linked to environment)

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8
Q

what is sarcoidosis?

A

multisystem granulomatous disease of unknown cause

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9
Q

who is most affected by ILDs? (not IPF)

A

adults <40, F>M, worldwide

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10
Q

how do you investigate ILDs?

A

history, exam, CXR + pulmonary function tests (bloods/ urinalysis, ECG, TB skin test, eye exam) + further assessments (bronchoscopy including transbronchial biopsies and endobronchial ultrasound

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11
Q

what do you need for a surgical biopsy? (to investigate ILD)

A
  • mediastincoscopy

- video assisted thoracoscopic lung biopsy (VATS)

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12
Q

how should ILDs be treated if mild disease, no vital organ involvement, normal lung function, few symptoms? (not IPF)

A

no treatment

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13
Q

how should ILDs be treated if erythema nodosum/ arthralgia? (not IPF)

A

NSAIDs

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14
Q

how should ILDs be treated if skin lesions/ anterior uveitis/ cough? (not IPF)

A

topical steroids

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15
Q

how should ILDs be treated if cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia? (not IPF)

A

systemic steroids

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16
Q

what is the prognosis with ILDs? (not IPF)

A
  • <1 caucasians die
  • 10-20% sustain permanent pulmonary or extra-pulmonary complications
  • progressive respiratory failure, bronchiectasis, aspergilloma, haemoptysis, pneumothorax
17
Q

what is the prognosis for IPF?

A

poor, median survival is 3 years

18
Q

how do you manage IPF?

A

refer to ILD clinic, medical options (OAF, pirfenidone, nintedanib), surgical option (transplant)

19
Q

what are the symptoms/ signs for IPF?

A

chronic breathlessness & cough, failed Rx for “LVF” or infection, clubbed & crackles

20
Q

who does IPF affect?

A

typically 60-70 years old, more common in men