features and management of reshective lung diseases Flashcards

1
Q

what is the physiological definition of restrictive lung disease

A

FVC <80% of normal

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2
Q

what could be causing restriction

A
lungs
pleura 
nerve or muscle
bone
other
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3
Q

what are examples of interstitial lung diseases

A

idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

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4
Q

what are pleural causes of resteictive disease

A

pleural effusions
pneumothorax
pleural thickening

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5
Q

what are skeletal causes

A

kyphoscoliosis
ankylosing spondylitis
rib fractures
thoracoplasty

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6
Q

what are muscle causes

A

amyotrophic lateral sclerosis

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7
Q

what are sub duaphragmatic causes

A

obesity

pregnancy

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8
Q

where is the interstitium of the lung

A

between the epithelium of the alveolus and the endothelium of the capillary

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9
Q

what are interstitial lung diseases

A

> 200 diseases causing thickening of ghe interstitium and can result in pulmonary fibrosis

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10
Q

what is sarcoidosis

A

multisystem granulomatoud disease of unlnown cause histological hallmark - non caseating granuloma

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11
Q

who is sarcoidosis more common in

A

adults <40

F>M

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12
Q

how do u investigate sarcoidosis

A

history ans exam

CXR

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13
Q

what do patients w sarcoidosis commonly present

A

bilateral hilar lymphadenopathy

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14
Q

how do tou investigate gland enlargement

A
pulmonary fucntion tests
bloods
urinalysis
ECH
TB skin test
eye exam
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15
Q

what is a further assessment in the airways

A

bronchoscopy including transbronchial biopsies and endobronchial ultrasound

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16
Q

what arw surfival biopsies

A

mediastinoscopy

video assisted thoracoscopic lung biopsy (VATS)

17
Q

treatments

A

erythema nodosum/arthralgia - NSAIDS
skin lesions/ anteriour uveitis/cough - tooical steroids
carduac, nerot etc - systemic steroids

18
Q

what is the tyoucal presentation of idiopathic pulmonary fibrosis

A

chronic breathlessness and cough
60-70 yrs
failed Rc for LVF or infection
clubbed and crackles

19
Q

what are options of ILDS

A

mediam survival 3 yrs
rever to ILD clinucal
OAF (oral antifibrotic) - pirfenidone, nintedanib, palliative care
surgical optjon - transplant