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8/20/2019 Pulmo Rad Conference Final
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Manilyn B. Lejos
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To present a case of an uncommondisease Idiopathic Pulmonary Fibrosis
To discuss about the incidence,
pathothysiology diagnostics andmanagement of the disease.
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Patient G.S. 65 y/o malearried
!atholic"i#es at $o#eleta, !a#ite
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COUGH
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%etired Policeman &tra'c enforcer($ot )no*n hypertensi#e
$ot )no*n +$ot )no*n to ha#e ronchial -sthmaccasional smo)er
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Pre#ious &0120(Pulmonary Functiontest &PFT( done
FEV1: 2.36
FVC: 2.77FEV1/FVC: 85!o"#al
September 2, 0123
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!4% 0120 !4% 0120
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C$%:&an'a"y 6(
2)1*
C$%: +','s- 12(
2)1*
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!4% an 2, 0125 !4% arch 2, 0125
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2)1
5 • &rd !onsult( %e#ie* of history done.
7 Itchy throat
7 cough */ hoarseness
7 dyspneaPresent eds
2.-nti8)och &on going 9% 0 *)s( : $o -%+s0.-nsimar
.ontelu)ast
3.!oale#a
5.mepra;ole
6.Seretide 05/051 0 pu
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2)15 • &rd !onsult( %e#ie* of history done.
$oted *ith !T scan chest -l#eointerstitial>densities,
$on8speci?c. Interstitialpneumonia.
P@ 10 sat 15A 9% B5> *t 263 lbs !rac)les Scattered $o !"-+
Initial -ssessment Idiopathic Pulmonary Fibrosis: gradual progression
Plan Secure !T scan Plates for re#ie*.
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PFT 0120 PFT 0123
• 2ECHO:!oncentric "C9 @F 55A +ilated %-. P- pressure 1 $ormal
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!4% 0120 !4% 0120
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!4% an 6, 0123 !4% -ugust 20,0123
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!4% an 2, 0125 !4% arch 2, 0125
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!T scan chest
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!T scan chest
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2)15
• &rd !onsult( For pen "ung iopsy 8 done
• -ssesment Idiopathic Pulmonary Fibrosis&biopsy pro#en(
• Plan To include in !linical Trial &@sbriet(perferidone 06= mg &2 capsule( P TI+.
• +iscontinue T meds and Seretide
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en D FemaleaEority of P &7( history of Smo)ingUnno0n : if the incidence and
pre#alence of IPF are inuenced bygeographic, ethnic, cultural or racialfactors.
American Journal of Respiratory and critical care medicine Vol 183 2010. Evidencebased medicine for treatment and management of !".
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IPF is fatal lung disease characteri;ed by 0o"senin, ysnea "o,"essie loss o4 l'n, 4'n-ion.
HH decline in forced #ital capacity &FC!( isconsistent *ith disease progression and ispredicti#e of reduced sur#i#al time.
#e$ England Journal %edicine. %ay 2&' 201(. E)cacy and safety #intedanib in !"
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elie#ed to arise from an aberrant "oli4e"a-ion o4 "o's -iss'e -iss'e "e#oelin,
ano"#al 4'n-ion an si,nalin, o4 aleola"ei-elial ells an in-e"s-i-ial "olas-s.
The acti#ation of cell8signaling path*ays through tyrosine
)inases such as #ascular endothelial gro*th factor &C@GF(,?broblast gro*th factor &FGF(, and platelet8deri#ed gro*thfactor &P+GF( has been implicated in the pathogenesis ofthe disease.
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Ci,a"e--e s#oin, : strong association &01pac) years smo)ers(
Eni"on#en-al e9os'"es & metal dus),
*ood dus), farming, raising birds, hairdressing, stone cutting, li#estoc), animasdus)(
Mi"oial a,en-s : @C and 9epatitis ! GE% : assoc *ith icroaspirations
Gene-i 5A only &familial(
American Journal of Respiratory and critical care medicine Vol 183 2010. Evidence
based medicine for treatment and management of !".
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Jneplained chroniceertional dyspnea
!ough bibasalinspiratory crac)les
?nger clubbing
Fi,'"e: ;y#-o#s inia-ie o4
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E9l'sion o4 o-e" no0n a'ses o4
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9%!T &7( "e-i'la" oai-ies -ssoc. *ith -"a-ion
"onie-asis
Honeyo#in, critical forma)ing a de?nite diagnosis
clustered cystic airspacessubpleural K *ell8de?ned
*alls G"o'n ,lass oai-ies less
etensi#e than the reticulation. +istribution asal an
e"ie"al K a-y.
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?+ an B@ Ui#a,es so0 asal"eo#inan-( e"ie"al
"eo#inan- "e-i'la"ano"#ali-y 0i- #'l-ile
(C and D) UIP pattern, with less
severe honeycombing: axial and
coronal CT images show basal
predominant, peripheral
predominant reticlar abnormality
with sbpleral honeycombing(arrows)!
?E an F @ =ossile U=a--e"n: a9ial an o"onali#a,es so0 e"ie"al"eo#inan-( asal"eo#inan- "e-i'la"
ano"#ali-y 0i- a#oe"a-e a#o'n- o4
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ronchoal#eolar la#age -" L Transbronchial "ung iopsyL Serologic TestingL
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Fi,'"e 2. ;'",ial l'n, iosy sei#ens e#ons-"a-in, U
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@mphysema a( Pulmonary emphysema,
accentuated in the upper partof the lung
b( Predominantly basal andsubpleural ?brosis *ithlargely reticular and lesspronounced ground8glass
components.
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ronchiectasis
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ronchiolitis ronchiolocentric
interstitial ground8glassopaci?cations,
accentuated in the upperpart of the lung.
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6 inute8 Mal) Testing Shorter *al) distance and delayed heart8ratereco#ery increased ris) of subseNuent
mortality
9istopathology -n increased number of ?broblast foci increased ris) of mortality
Pulmonary9ypertension
&7( Pulmonary hypertension &de?ned as amean pulmonary -rtery pressure of . 05 mm 9gat rest( increased ris) of mortality for patients
*ith IPF. &7( ean P-P of 2= mm 9g bestdiscriminator of mortality
@mphysema $T !"@-% if IPF *ith coeisting emphysemarepresents a distinct prognosis
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E%VE!>
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6. !+C ?#ono-e"ay@ aEority should not be t but may be reasonablechoice in minority &*ea) recommendation, lo*Nuality e#idence(.HH 9igh #alue of cost of t *ith lo* Nuality data
7.
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1).=i"4e"ione?y"ione o#o'n
0i- leio-"oi(an-iina##a-o"y(an-i"o-i( anan-io9ian- "oe"-ies(0i- an-a,onis# o4 >GF1 ee-s@
-P-$ &2( Pilot study sho*ed bene?t butincomplete due to premature interruption intrial. &0( 0nd study Pirferidone #s placebo 8 7decline in C! 50 *) treatment.
JS- &2( et the primary endpoint of changein A FC! fa#or pirferidone. &0( ther study, didnot meet the endpoint
11.!in-eani ?inii-o" #'l-ile
-y"osie inase@
reduced the decline in FC!, *hich isconsistent *ith a slo*ing of disease progression>
nintedanib *as fre8Nuently associated *ithdiarrhea, *hich led to discontinuation of thestudy medica8tion in less than 5A of patients.
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%EM+%;
ygen Strong recommendation by physilogicrationale *ith hypoemia OOA $o data on long8term use.
"ung transplant Strong recommendationB year sur#i#arl rate post transplant $o clear data : re timing or singel/dual lobe
echanical Centilation B6A mortality : on C &unless reasonable(. $on8in#asi#e positi#e pressure #entilation use
Pulmonary%ehabilitation
Impro#es Mal)8distance K uality of life aEority should recei#e rehab but may not bereasonable in a minority. Jnclear "ong term bene?t
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