features and management of reshective lung diseases Flashcards
what is the physiological definition of restrictive lung disease
FVC <80% of normal
what could be causing restriction
lungs pleura nerve or muscle bone other
what are examples of interstitial lung diseases
idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis
what are pleural causes of resteictive disease
pleural effusions
pneumothorax
pleural thickening
what are skeletal causes
kyphoscoliosis
ankylosing spondylitis
rib fractures
thoracoplasty
what are muscle causes
amyotrophic lateral sclerosis
what are sub duaphragmatic causes
obesity
pregnancy
where is the interstitium of the lung
between the epithelium of the alveolus and the endothelium of the capillary
what are interstitial lung diseases
> 200 diseases causing thickening of ghe interstitium and can result in pulmonary fibrosis
what is sarcoidosis
multisystem granulomatoud disease of unlnown cause histological hallmark - non caseating granuloma
who is sarcoidosis more common in
adults <40
F>M
how do u investigate sarcoidosis
history ans exam
CXR
what do patients w sarcoidosis commonly present
bilateral hilar lymphadenopathy
how do tou investigate gland enlargement
pulmonary fucntion tests bloods urinalysis ECH TB skin test eye exam
what is a further assessment in the airways
bronchoscopy including transbronchial biopsies and endobronchial ultrasound
what arw surfival biopsies
mediastinoscopy
video assisted thoracoscopic lung biopsy (VATS)
treatments
erythema nodosum/arthralgia - NSAIDS
skin lesions/ anteriour uveitis/cough - tooical steroids
carduac, nerot etc - systemic steroids
what is the tyoucal presentation of idiopathic pulmonary fibrosis
chronic breathlessness and cough
60-70 yrs
failed Rc for LVF or infection
clubbed and crackles
what are options of ILDS
mediam survival 3 yrs
rever to ILD clinucal
OAF (oral antifibrotic) - pirfenidone, nintedanib, palliative care
surgical optjon - transplant
