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restricitive interstital lung disease Flashcards

1
Q

what does restrictive interstitall lung disease lead to

A

diffusion impairment

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2
Q

what makes up the interstitial space

A

the connective tissue space around the airways and vessels

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3
Q

what is sarcoidosis

A

a multisystem granulomatous disorder of unknown atepoigy

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4
Q

who does saecoidosis effect - incidence

A

young adults

females more than males

4/100000

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5
Q

what organs are effected in sarcodisis

A

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

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6
Q

what is the prensentation of sarcoidosis

A

often sumptomatic

bilateral hilar lympjadenopathy

SOB

cough

abnormal CXR

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7
Q

what is the treatment of sacodisis

A

most resolve after 2 years

or if needed Corticosteriods

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8
Q

what does interstitial lung disease do

A

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

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9
Q

what does restrictive interstitial lung disease do

A

reduce lung compliance and stiffens lungs

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10
Q

what happens when the lungs become stiffened

A

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

reduce gas transfer

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11
Q

what is the presentation of diffuse lung disease

A

SOB

respiratory failure - type 1

heart failure

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12
Q

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

A

the lungs are shortened in the chest

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13
Q

what are the two types of diffuse interstitial lung injury

A

acute

chronic

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14
Q

what can the acute interstitial lung injury lead to

A

chronic interstitial lung disease

diffuse alveolar damage
DAD

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15
Q

what are the causes of DAD (diffuse alveolar damage)

A

major trauma

chemical/toxic inhalation

drugs

infection

idiopathic

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16
Q

what is the pathology of DAD

A

exudate creates interstitial inflammation that leads to interstitial fibrosis

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17
Q

what is the outcome of chronic response intertidal lung injury

A

fibrosis or end stage honeycomb lung

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18
Q

what is sarcoidosis an example of

A

chronic interstitial lung injury

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19
Q

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

A

fibrous or end-stage honeycombing

20
Q

what is hypersensitivity pneumonitis

A

hypersensitivity to antigens such as

fungi
chemicals
bird proteins - feathers

21
Q

what is the acute presentation of hypersensitivity pneumonitis

A

fever
dry cough
myalgia (muscle pain)

chills 4-9hrs after exposure

crackles,
tachypnoea,
wheeze

22
Q

what is the chronic presentation of hypersensitivity pneumonitis

A

myalgia - muscle pain

SOB

chough

23
Q

what can hypersensitivity pneumonitis cause

A

respiratory failure

low gas transfer

24
Q

what is the pathophysiology being hypersensitive pneumonitis

A

type 3, 4

causing granuloma leading to interstitial pneumonitis

its a upper zone disease

25
Q

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

A

it can be seen in connective tissue disease

but can be idiopathic

26
Q

what is UIP (usual interstitial pneumonitis)

A

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

27
Q

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

A

basal and posterior fibrosis with honeycombing

28
Q

what are the risk factors of UIP

A

old
male

but it is idiopathic

29
Q

what are the clinical sings of UIP

A

SOB

cough

basal crackles

cyanosis

clubbing

30
Q

what is the prognosis of UIP

A

progressive disease

most dead within 5 years

31
Q

what is the treatment of UIP (usual interstitial pneumonitis)

A

some steroid response but not good

32
Q

what is the physiological definition of restrictive lung disease

A

a FVC of less than 80% of predicted normal

33
Q

what are the broad categories that can cause restrictive lung disease

A

lungs

pleura

nerve/muscle

bone

other

34
Q

what is UIP also known as (usual interstitial pneumonitis)

A

idiopathic pulmonary fibrosis

35
Q

what are the ‘‘lung’’ causes of restrictive lung disease

A

idiopathic pulmonary fibrosis

sarcoidosis

hypersensitivity pneumonitis

36
Q

what are the pleural causes of restrictive lung disease

A

pleural effusion

pneumothorax

pleural thickening

37
Q

what are the nerve/ muscle causes of restrictive lung disease

A

amyotrophic lateral sclerosis

38
Q

what are some bone causes of restrictive lung disease

A

rib fractures

kyphoscoliosis

39
Q

what are some other cases of restrictive lung disease

A

sub diaphragmatic causes

obesity

pregnancy

40
Q

what investigations do you do for sarcoidosis - findings

A

chest x-ray –consolidation

bronchoscopy - biopsies

pulmonary function test

bloods

41
Q

what is a VATS

A

video assisted thoracoscopic lung biopsy

42
Q

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

A

no treatment

NSAIDs

topical steroids

systemic steroids

43
Q

what are NSAIDs

A

non steroidal anti-inflammatory drugs

44
Q

what is the prognosis of sarcoidosis

A

probably ok

1% die

10-20% sustain permanent complications

45
Q

what is the presentation of idiopathic pulmonary fibrosis

A

chronic SOB
chough

clubbing

crackles

typically 60-70yr old - men

46
Q

what are the options for idiopathic pulmonary fibrosis

A

median survival - 3years

take oral anti -fibrotic (nintedanib)

palliative

transplant??

respiration (17 decks)

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