Restrictive lung disease Clinical features & Management Flashcards
what is the physiologial definition of restriction
Forced vital capacity less than 80%
clinically how could you think about causes of restrictive lung disease
lungs (inc.disease of) pluera nerve or muscle bone other
what are 3 common interstitial lung diseases
idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis
what pleural abnormalities can lead to restrictive lung disease
pleural effusions
pneumothorax
plueral thickening
what skeletal abnormalities can cause restrictive lung disease
kyphoscoliosis
Ankylosing spondylitis
thoracoplasty
rib fractures
what muscular abnormalities can cause restrictive lung disease
amyotrophic lateral sclerosis
what are sub-diaphragmatic changes that can cause restrictive lung disease
obesity
pregnancy
what is the interstitium
the space between the alveoli and the capillaries in the lung
In general how can you define ILDs
200 diseases causing thickening of the interstitium and can result in pulmonary fibrosis
what is sarcoidosis
Multisystem granulomatous disease of unknown cause
typically who gets sarcoidosis
adults below 40
more women than men
occurs worldwide
how would you begin to investigate sarcoidosis
history and exam CXR pulmonary function tests bloods eye exam urinalysis ECG
what are further assessments you could do to a patient with suspected restrictive lung disease
Bronchoscopy
trnsbronchial biopsies
endobronchial ultrasound
what is the prognosis in the late stages of restrictive lung disease
remission rate 0-20%
in late stage of the disease what can restrictive lung disease progress to
honeycomb lung
how should sarcoidosis be treated if it is mild and normal lung function with no vital organ involvement
no treatment
how should sarcoidosis be treated if there is arthralgia and erythema nodosum present
NSAIDs
how should sarcoidosis be treated if Skin lesions / anterior uveitis / cough are present
topical steroids
how should sarcoidosis be treated if Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia are present
systemic steroids
what is the outlook for sarcoidosis
generally ok less than 1% caucasians die
what is idiopathic pulmonary fibrosis (aka UIP)
chronic disease that results in increased scarring of the lungs due to repeated injury to alveoli
how will a patient with IPF usually present
typically 60-70 with dry cough breathlessness and possible clubbing may be crackles commoner in men
what is the survival rate for patients with IPF
medial survival rate is 3 years
what are medical treatment options for IPF
OAF -Pirfenidone, Nintedanib
palliative care
transplant
