pulmo rad conference final

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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• &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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pulmo rad conference final

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