restricitive interstital lung disease

Question 1

what does restrictive interstitall lung disease lead to

Answer 1

diffusion impairment

Question 2

what makes up the interstitial space

Answer 2

the connective tissue space around the airways and vessels

Question 3

what is sarcoidosis

Answer 3

a multisystem granulomatous disorder of unknown atepoigy

Question 4

who does saecoidosis effect - incidence

Answer 4

young adults

females more than males

4/100000

Question 5

what organs are effected in sarcodisis

Answer 5

lymph node - 100%
lung - 90%
spleen - 75%
live -70%

Question 6

what is the prensentation of sarcoidosis

Answer 6

often sumptomatic

bilateral hilar lympjadenopathy

SOB

cough

abnormal CXR

Question 7

what is the treatment of sacodisis

Answer 7

most resolve after 2 years

or if needed Corticosteriods

Question 8

what does interstitial lung disease do

Answer 8

widens the space between the basement membrane of the alveolar epithelia and the interstitial capillary

Question 9

what does restrictive interstitial lung disease do

Answer 9

reduce lung compliance and stiffens lungs

Question 10

what happens when the lungs become stiffened

Answer 10

Low FEV1 and low FVC but FEV1/FVC remains the same

reduce gas transfer

Question 11

what is the presentation of diffuse lung disease

Answer 11

SOB

respiratory failure - type 1

heart failure

Question 12

what does a chest x-ray of a patient with interstitial lung disease look like

Answer 12

the lungs are shortened in the chest

Question 13

what are the two types of diffuse interstitial lung injury

Answer 13

acute

chronic

Question 14

what can the acute interstitial lung injury lead to

Answer 14

chronic interstitial lung disease

diffuse alveolar damage
DAD

Question 15

what are the causes of DAD (diffuse alveolar damage)

Answer 15

major trauma

chemical/toxic inhalation

drugs

infection

idiopathic

Question 16

what is the pathology of DAD

Answer 16

exudate creates interstitial inflammation that leads to interstitial fibrosis

Question 17

what is the outcome of chronic response intertidal lung injury

Answer 17

fibrosis or end stage honeycomb lung

Question 18

what is sarcoidosis an example of

Answer 18

chronic interstitial lung injury

Question 19

what is the possible outcome of untreated, out of control sarcoidosis

Answer 19

fibrous or end-stage honeycombing

Question 20

what is hypersensitivity pneumonitis

Answer 20

hypersensitivity to antigens such as

fungi
chemicals
bird proteins - feathers

Question 21

what is the acute presentation of hypersensitivity pneumonitis

Answer 21

fever
dry cough
myalgia (muscle pain)

chills 4-9hrs after exposure

crackles,
tachypnoea,
wheeze

Question 22

what is the chronic presentation of hypersensitivity pneumonitis

Answer 22

myalgia - muscle pain

SOB

chough

Question 23

what can hypersensitivity pneumonitis cause

Answer 23

respiratory failure

low gas transfer

Question 24

what is the pathophysiology being hypersensitive pneumonitis

Answer 24

type 3, 4

causing granuloma leading to interstitial pneumonitis

its a upper zone disease

Question 25

what is normally the cause of usual interstitial pneumonitis(UIP)

Answer 25

it can be seen in connective tissue disease but can be idiopathic

Question 26

what is UIP (usual interstitial pneumonitis)

Answer 26

progressive scarring of both lungs. involving the interstitial space of the lung.

Question 27

where is UIP normally present in the lung - and with what

Answer 27

basal and posterior fibrosis with honeycombing

Question 28

what are the risk factors of UIP

Answer 28

old male but it is idiopathic

Question 29

what are the clinical sings of UIP

Answer 29

SOB cough basal crackles cyanosis clubbing

Question 30

what is the prognosis of UIP

Answer 30

progressive disease most dead within 5 years

Question 31

what is the treatment of UIP (usual interstitial pneumonitis)

Answer 31

some steroid response but not good

Question 32

what is the physiological definition of restrictive lung disease

Answer 32

a FVC of less than 80% of predicted normal

Question 33

what are the broad categories that can cause restrictive lung disease

Answer 33

lungs pleura nerve/muscle bone other

Question 34

what is UIP also known as (usual interstitial pneumonitis)

Answer 34

idiopathic pulmonary fibrosis

Question 35

what are the ''lung'' causes of restrictive lung disease

Answer 35

idiopathic pulmonary fibrosis sarcoidosis hypersensitivity pneumonitis

Question 36

what are the pleural causes of restrictive lung disease

Answer 36

pleural effusion pneumothorax pleural thickening

Question 37

what are the nerve/ muscle causes of restrictive lung disease

Answer 37

amyotrophic lateral sclerosis

Question 38

what are some bone causes of restrictive lung disease

Answer 38

rib fractures kyphoscoliosis

Question 39

what are some other cases of restrictive lung disease

Answer 39

sub diaphragmatic causes obesity pregnancy

Question 40

what investigations do you do for sarcoidosis - findings

Answer 40

chest x-ray --consolidation bronchoscopy - biopsies pulmonary function test bloods

Question 41

what is a VATS

Answer 41

video assisted thoracoscopic lung biopsy

Question 42

what is the treatment for sarcoidosis from least sever to most sever

Answer 42

no treatment NSAIDs topical steroids systemic steroids

Question 43

what are NSAIDs

Answer 43

non steroidal anti-inflammatory drugs

Question 44

what is the prognosis of sarcoidosis

Answer 44

probably ok 1% die 10-20% sustain permanent complications

Question 45

what is the presentation of idiopathic pulmonary fibrosis

Answer 45

chronic SOB chough clubbing crackles typically 60-70yr old - men

Question 46

what are the options for idiopathic pulmonary fibrosis

Answer 46

median survival - 3years take oral anti -fibrotic (nintedanib) palliative transplant??