restricitive interstital lung disease Flashcards
what does restrictive interstitall lung disease lead to
diffusion impairment
what makes up the interstitial space
the connective tissue space around the airways and vessels
what is sarcoidosis
a multisystem granulomatous disorder of unknown atepoigy
who does saecoidosis effect - incidence
young adults
females more than males
4/100000
what organs are effected in sarcodisis
lymph node - 100%
lung - 90%
spleen - 75%
live -70%
what is the prensentation of sarcoidosis
often sumptomatic
bilateral hilar lympjadenopathy
SOB
cough
abnormal CXR
what is the treatment of sacodisis
most resolve after 2 years
or if needed Corticosteriods
what does interstitial lung disease do
widens the space between the basement membrane of the alveolar epithelia and the interstitial capillary
what does restrictive interstitial lung disease do
reduce lung compliance and stiffens lungs
what happens when the lungs become stiffened
Low FEV1 and low FVC but FEV1/FVC remains the same
reduce gas transfer
what is the presentation of diffuse lung disease
SOB
respiratory failure - type 1
heart failure
what does a chest x-ray of a patient with interstitial lung disease look like
the lungs are shortened in the chest
what are the two types of diffuse interstitial lung injury
acute
chronic
what can the acute interstitial lung injury lead to
chronic interstitial lung disease
diffuse alveolar damage
DAD
what are the causes of DAD (diffuse alveolar damage)
major trauma
chemical/toxic inhalation
drugs
infection
idiopathic
what is the pathology of DAD
exudate creates interstitial inflammation that leads to interstitial fibrosis
what is the outcome of chronic response intertidal lung injury
fibrosis or end stage honeycomb lung
what is sarcoidosis an example of
chronic interstitial lung injury
what is the possible outcome of untreated, out of control sarcoidosis
fibrous or end-stage honeycombing
what is hypersensitivity pneumonitis
hypersensitivity to antigens such as
fungi
chemicals
bird proteins - feathers
what is the acute presentation of hypersensitivity pneumonitis
fever
dry cough
myalgia (muscle pain)
chills 4-9hrs after exposure
crackles,
tachypnoea,
wheeze
what is the chronic presentation of hypersensitivity pneumonitis
myalgia - muscle pain
SOB
chough
what can hypersensitivity pneumonitis cause
respiratory failure
low gas transfer
what is the pathophysiology being hypersensitive pneumonitis
type 3, 4
causing granuloma leading to interstitial pneumonitis
its a upper zone disease
what is normally the cause of usual interstitial pneumonitis(UIP)
it can be seen in connective tissue disease
but can be idiopathic
what is UIP (usual interstitial pneumonitis)
progressive scarring of both lungs. involving the interstitial space of the lung.
where is UIP normally present in the lung - and with what
basal and posterior fibrosis with honeycombing
what are the risk factors of UIP
old
male
but it is idiopathic
what are the clinical sings of UIP
SOB
cough
basal crackles
cyanosis
clubbing
what is the prognosis of UIP
progressive disease
most dead within 5 years
what is the treatment of UIP (usual interstitial pneumonitis)
some steroid response but not good
what is the physiological definition of restrictive lung disease
a FVC of less than 80% of predicted normal
what are the broad categories that can cause restrictive lung disease
lungs
pleura
nerve/muscle
bone
other
what is UIP also known as (usual interstitial pneumonitis)
idiopathic pulmonary fibrosis
what are the ‘‘lung’’ causes of restrictive lung disease
idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis
what are the pleural causes of restrictive lung disease
pleural effusion
pneumothorax
pleural thickening
what are the nerve/ muscle causes of restrictive lung disease
amyotrophic lateral sclerosis
what are some bone causes of restrictive lung disease
rib fractures
kyphoscoliosis
what are some other cases of restrictive lung disease
sub diaphragmatic causes
obesity
pregnancy
what investigations do you do for sarcoidosis - findings
chest x-ray –consolidation
bronchoscopy - biopsies
pulmonary function test
bloods
what is a VATS
video assisted thoracoscopic lung biopsy
what is the treatment for sarcoidosis from least sever to most sever
no treatment
NSAIDs
topical steroids
systemic steroids
what are NSAIDs
non steroidal anti-inflammatory drugs
what is the prognosis of sarcoidosis
probably ok
1% die
10-20% sustain permanent complications
what is the presentation of idiopathic pulmonary fibrosis
chronic SOB
chough
clubbing
crackles
typically 60-70yr old - men
what are the options for idiopathic pulmonary fibrosis
median survival - 3years
take oral anti -fibrotic (nintedanib)
palliative
transplant??
