What is restictive lung disease Flashcards
what is the interstium of hte lung
ct, space around the airways and vessels and the space between the basement membranes of the alveolar walls.
what are the cells responseibel for restictive lung dissdease
parenchymal
what type of change in cells is seen I interstial lung diseaews
alvelar wal thickeing by interstila infiltrat3e
what is the effect on the lungs of interstial lung disease
redued compliane, both fev1 and fvc reduced, normal fev1/fvc ratio, reduced gas transfer, ventilatio/ perfusion inbalance
what are the symptoms of intersital lugn disease
shorternes of breath on exertation becoming shortnes of breath at rest
what does intersial lung iaes look like on a chest xray
reduced no of anterior ribs visible
what condition is result of a chronic response to interstial lung disese
diffuse alveolar damage (dad)
what can cause diffuse alvelar damage
trauma, chemacl injhyry, circulary shock, drugs, infection including viruses e..g covid, audto immune disease, radiation
what develops as a result of dad
first edema, then hyaline membarem which is replaced by interstil inflamation and then intersial fibrodis
what does dads look like in histolgy
protien rich oedema, fibrin, hyaline membars, denuned basement membrane, epitheal proliferation, fibroblast proliferation, scarring,
what is sarcoidosis
a multisystems granulomatous distorder of unkow aetilgoy, as part of a chronic responce
what is the histopaty of sarcoidosis
epitheloid and gisant cell granulomas, necrosis , little lymphoid infiltrate, variable associated fibrosis
which groups is sarcodosis most comon in
afterica american in usa
which organs are most invoided in sarcoidses
lymp 100
lungs 90
spleen 75
liver - 70
skin 50
salaviary gland - up to 50
what are normal signs of sarcododes
rash around shins (nodosum) joint pain , bi lateral lympadopathy , fever
what cells are involed in sarcodosis
epitheloids, macrophages lymphoctes t cells
what are teh symptons of pulmoary infliatra of sarcodios
cough, exertioal brathlessness and vague chest discomfort
what si the inversigation of r sarcodoes
cxr, fbc for ameia, biopsy or ebus cytolgy, serum ca++, serum ace levels, lung fucntion test, cardicat test
what is ace
a biomarker for damge to epithelum cells an increase in this signifies damage
What is hpersentify pneumonits
an allergic reaction affecting the small airays and alveoil in response to an inhaled antigen or occasionally following ingestion of a cuastive drug
are cigarette smokers more or less likely to develop hypersensitivity pneumonitis
less likley, due to increased antibody reactio to the antigne.
what can cuase hypersentiify pneumois
fungi e.g spergillus sp
bird/ ainal protien form feases/ bloom
chemical
thermophilic actinomyctes - high temp aerobic bacteria
what microogans cause farmers lung
therophillic actinoctes e..g mircopolyspora faeni or funcgi e..g. apergilluis umbrosus
what are the sympones of acute hypersenitive pneumonitis
dysponea,k cough, chills initally after exposure, mlagica ,dry cough, fever, malise, chrakes, tachopea, wheeze
what are teh symptons of chroncic hp
fingerclubbing, malaise, sob, cough, malaise, weight loss, crakces and wheeze
what is the histology of hp
soft centainar epitheloid granulmata, intersil pneumotitis, fomary hiotcyt,s bronchites obliternas
what type of hypersentigy is hp
3 and 4
where does hp normally occur in the lungs
upper zone
what is idiopathic fibrodise or unusal interial pneyonits
progessige and often fatla disea of undkown cuases, wher fibrois and honey combing occur in some area
what caaseses uip
ct dieae, durg greaction, ;post infeciton, asbestods
what are teh sympotsn of uip
cough, sputum, dysponea, bi lateral crackes , hyperteins, pnumoathx, pulmonary embolis, intercurrent infrciton,k
what is the differitial digaes ofr uip
another interstl lung diease
what test are used for uip
cxr, increased showdiog, hrct 0 increaed abnomarlies at bases, broinciea, honey cominig, bronchal lavage, hislogic, respfucntio test
how long do peoplelive with uip
2- 5 years
what is the treatet for uit
pirfenidone and nintedanib
what is most likely to have end stage fibories
uip