Restrictive lung disease Clinical features & Management

Question 1

what is the physiologial definition of restriction

Answer 1

Forced vital capacity less than 80%

Question 2

clinically how could you think about causes of restrictive lung disease

Answer 2

lungs (inc.disease of)
pluera 
nerve or muscle
bone 
other

Question 3

what are 3 common interstitial lung diseases

Answer 3

idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

Question 4

what pleural abnormalities can lead to restrictive lung disease

Answer 4

pleural effusions
pneumothorax
plueral thickening

Question 5

what skeletal abnormalities can cause restrictive lung disease

Answer 5

kyphoscoliosis
Ankylosing spondylitis
thoracoplasty
rib fractures

Question 6

what muscular abnormalities can cause restrictive lung disease

Answer 6

amyotrophic lateral sclerosis

Question 7

what are sub-diaphragmatic changes that can cause restrictive lung disease

Answer 7

obesity

pregnancy

Question 8

what is the interstitium

Answer 8

the space between the alveoli and the capillaries in the lung

Question 9

In general how can you define ILDs

Answer 9

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

Question 10

what is sarcoidosis

Answer 10

Multisystem granulomatous disease of unknown cause

Question 11

typically who gets sarcoidosis

Answer 11

adults below 40
more women than men
occurs worldwide

Question 12

how would you begin to investigate sarcoidosis

Answer 12

history and exam
CXR
pulmonary function tests
bloods 
eye exam
urinalysis
ECG

Question 13

what are further assessments you could do to a patient with suspected restrictive lung disease

Answer 13

Bronchoscopy
trnsbronchial biopsies
endobronchial ultrasound

Question 14

what is the prognosis in the late stages of restrictive lung disease

Answer 14

remission rate 0-20%

Question 15

in late stage of the disease what can restrictive lung disease progress to

Answer 15

honeycomb lung

Question 16

how should sarcoidosis be treated if it is mild and normal lung function with no vital organ involvement

Answer 16

no treatment

Question 17

how should sarcoidosis be treated if there is arthralgia and erythema nodosum present

Answer 17

NSAIDs

Question 18

how should sarcoidosis be treated if Skin lesions / anterior uveitis / cough are present

Answer 18

topical steroids

Question 19

how should sarcoidosis be treated if Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia are present

Answer 19

systemic steroids

Question 20

what is the outlook for sarcoidosis

Answer 20

generally ok less than 1% caucasians die

Question 21

what is idiopathic pulmonary fibrosis (aka UIP)

Answer 21

chronic disease that results in increased scarring of the lungs due to repeated injury to alveoli

Question 22

how will a patient with IPF usually present

Answer 22

typically 60-70
with dry cough
breathlessness
and possible clubbing
may be crackles 
commoner in men

Question 23

what is the survival rate for patients with IPF

Answer 23

medial survival rate is 3 years

Question 24

what are medical treatment options for IPF

Answer 24

OAF -Pirfenidone, Nintedanib

palliative care
transplant

Question 25

what is Oral anti-fibrotic - OAF and what does it do

Answer 25

it slows down the deterioration in vital capacity however it wont actually make the patient feel better

Question 26

what is Hypersensitivity pneumonitis

Answer 26

inflammation of the alveoli within the lung caused by hypersensitivity to inhaled organic dusts.