ENFERMEDAD PULMONAR INTERSTICIAL Dra. Vernica Llamoca Palominovkllamoca@o!mail.comMEDICINA II" Ne#molo$%a& 'e A$o(!o 'el )*+,-n'ice.+/ In!ro'#ccin.)/ Pa!o$enia.0/ Cla(i1icacin.&/ Mani1e(!acione( cl%nica(.,/ An!ece'en!e(.2/ E34mene( a#3iliare(.5/ Ne#mo6a!%a(.7/ Tra!amien!o.8/ F#en!e( 'e in1ormacin*&"*7")*+, Dra. Vernica Llamoca Palomino+/ In!ro'#ccin.La En1erme'a' P#lmonar in!er(!icial 9ILD:; (e re1iere a #n conn(ea(ociancon#naam6liaal!eracinanivel alveolar B 'e la ar=#i!ec!#ra 'e la( v%a( re(6ira!oria(.*&"*7")*+, Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+, Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca PalominoPa!o$eniaCla(i1icacin.Se 'ivi'en enC ca#(a( conoci'a( e i'io64!ica(. .El!ra!amien!oBel6ron(!icovar%an'e6en'ien'o'ela ca#(a;6orlo=#e'e!erminarel'ia$n(!icocorrec!oe( im6or!an!e..In1eccione(comone#mon%a(6oron$o( 9cocci'ioi'omico(i(;cri6!ococo(i(;Pne#mocB(!i( l#la('eLan$eran(; ne#moni!i(in!er(!icial'e(cama!iva;en1erme'a' 6#lmonarin!er(!icial"@ron=#ioli!i(re(6ira!oriaBla1i@ro(i( 6#lmonar i'io64!ica;.E3 1#ma'ore( 9la (arcoi'o(i( B la ne#moni!i( 6or i6er(en(i@ili'a':..Se cree =#e 1#mar 'aAa la 6are' alveolar; acien'o =#e la mem@rana @a(al alveolar e(!e m4( acce(i@le a lo( an!ic#er6o( circ#lan!e(.*&"*7")*+,Dra. Vernica Llamoca PalominoAn!ece'en!e( 1amiliare( .Se6#e'en6ro'#cir'i1eren!e(!i6o('eILD91i@ro(i( 6#lmonar i'io64!ica B ne#moni!i( in!er(!icial no e(6ec%1ica: 'en!ro 'e #na (ola 1amilia. .Sarcoi'o(i( oc#rre en #>(6e'e( $en>!icamen!e (#(ce6!i@le(. .Pa!rn a#!o(mico 'ominan!eC 1i@ro(i( 6#lmonar i'io64!ica 9FPI:; e(clero(i( !#@ero(a B la ne#ro1i@roma!o(i(..Pa!rn a#!o(mico rece(ivoC en1erme'a' 'e Niemann"Pick; en1erme'a' 'e Ga#cer; B el (%n'rome 'e Ferman(kB"P#'lak.*&"*7")*+,Dra. Vernica Llamoca PalominoAn!ece'en!e( 'e me'icamen!o(.Le(in 6#lmonar in'#ci'a 6or ra'iacin e(!4 'irec!amen!e relaciona'a con el vol#men 'e 6#lmn irra'ia'o B la 'o(i( ac#m#la!iva 'e la irra'iacin. .Lo((%n!oma((e'e(arrollanalre'e'or'e&"+)(emana( 'e(6#>('elae36o(icin;mien!ra(=#elo((%n!oma( !ar'%o( 'e 1i@ro(i( (e 'e(arrollan 'e(6#>( 'e 2"+) me(e(.*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca PalominoE36o(icione( am@ien!ale( B oc#6acionale(.Enlane#moni!i(6ori6er(en(i@ili'a'9alveoli!i(al>r$ica e3!r%n(eca:;la6re(encia'e1ie@re;e(calo1r%o(B#na ra'io$ra1%a'e!ra3anormal(onrelaciona'a(conel l#$ar'e!ra@amico;en1erme'a'mi3!a'el!erico( (#$e(!ivo(C la 6ro!e%na (#r1ac!an!e A B J 9SP"A; SP"J:; lo( monoci!o( =#imioa!raBen!e 6ro!e%na"+ 9MCP"+:; B Ker@ von L#n$ren 9KL: "2; $lico6ro!e%na 'e al!o 6e(o molec#lar e36re(a'a 6or ne#moci!o( !i6o II..KL"2 (e a(oci con la maBor (en(i@ili'a'; e(6eci1ici'a' 6ara el 'ia$n(!ico 6ara la 6re(encia 'e ILD. El 6a6el cl%nico 'e e(!o( marca'ore( en (#ero en el 'ia$n(!ico 'e la ILD e( claro B e(!a( 6r#e@a( $eneralmen!e no e(!4n 'i(6oni@le( comercialmen!e.*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca PalominoRa'io$ra1%a 'e !ra3 *&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca Palomino*&"*7")*+,Dra. Vernica Llamoca PalominoTomo$ra1%a com6#!ariEa'a 'e al!a re(ol#cin.9TCAR:(erecomien'aen'ecH@i!o(#6inoB6rono6ara evi!ar la a!elec!a(ia 'e6en'ien!e(..Pro6orciona #na maBor 6reci(in 'ia$n(!ica. .Pa!rone( 'e(cri!o(CD A'eno6a!%a iliar @ila!eral B (im>!rica (#6erior; o6aci'a'e( re!ic#lare( (#$ieren (arcoi'o(i( # o!ra en1erme'a' $ran#loma!o(a.D La( 6laca( 6le#rale( con calci1icacin lineal (#$ieren a(@e(!o(i(.D N'#lo( cen!rolo@#lillare( 'e la re$in (#@6le#ralC ne#moni!i( 6or i6er(en(i@ili'a'; (arcoi'o(i(; i(!ioci!o(i( 'e c>l#la( 'e Lan$eran(.*&"*7")*+,Dra. Vernica Llamoca PalominoD L#i(!e( irre$#lare( a(ocia'o( con n'#lo( en la( Eona( 6#lmonare((#6eriore(Bme'ia((#$iereni(!ioci!o(i( 6#lmonar'ec>l#la('eLan$eran(DO6aci'a'e(re!ic#lare((#@6le#rale(B@i@a(ale( a(ocia'o(conlo(cam@io(en6anal'ea@eolo(. *&"*7")*+,Dra. Vernica Llamoca PalominoCa6aci'a' 'e 'i1#(inC mon3i'o car@ono.La6re(encia'e'e(a!#racinar!erial;laim6o(i@ili'a' 6arare'#cirele(6aciom#er!oconele( 'el !ra!amien!o. .El'ia$n(!ico'i1erencial6rinci6al(ee(!a@leceen!rela (arcoi'o(i(B la ne#moni!i( 6or i6er(en(i@ili'a'*&"*7")*+,Dra. Vernica Llamoca PalominoNe#mo6a!%a $ran#loma!o(a.El!ra!amien!onoinvier!ela1i@ro(i(;(#(o@3i!ovaria@lela ciclo1o(1ami'a B la aEa!io6rina 9+ a ) m$ 'iario(Qk$: con o (in $l#cocor!icoi'e(. Re(6#e(!a o@reo;con'en(acione(envi'rioe(merila'oo no'#lare(6e=#eAa(Ben$ro(amien!oB'ila!acin'ela 6are' @ron=#ial; en la 6eri1eria B la Eona @a(al. *&"*7")*+,Dra. Vernica Llamoca PalominoNEUMON-A ORGANIMATIVA CRIPTUGENA.La@io6(iaC!e( 'e in!err#m6ir el !a@a=#i(mo.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL POR TAJALUISMO.Histiocitosis pulmonar de clulas de Langerhans.E( #na ne#mo6a!%a 'i1#(a B 6oco 1rec#en!e =#e (e enc#en!ra li$a'a al !a@aco B =#e a1ec!a 6rinci6almen!e a lo( varone( en!re lo( )* B &* aAo(. .Cl%nicaC e(!a'o a(in!om4!ico a(!a #na en1erme'a' 'e avance r46i'o. To(; 'i(nea; 'olor !or4cico; 6>r'i'a 'e 6e(o B 1ie@re. En cerca 'e ),S 'e lo( 6acien!e( a6arece ne#mo!ra3. La emo6!i(i( B la 'ia@e!e( in(%6i'a (on mani1e(!acione( 6oco 1rec#en!e(.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL POR TAJALUISMO.Histiocitosis pulmonar de clulas de Langerhans.TCARCcom@inacin'en'#lo(B=#i(!e('e6are' 'el$a'a.Laanomal%am4(1rec#en!e'ela1#ncin 6#lmonar e( #na re'#ccin marca'a 'e la DLCO..Fi(!olo$%aCi(!ioci!o(i('ec>l#la('eLan$eran( 6#lmonare(e(la6re(encia'ele(ione(e(clero(an!e( no'#lare(=#econ!ienenc>l#la('eLan$eran(B(e acom6aAan 'e in1il!ra'o( cel#lare( mi3!o(. .El !ra!amien!o clave e( in!err#m6ir el !a@a=#i(mo.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL POR TAJALUISMO.La( CTD a men#'o (on 'i1%cile( 'e e3cl#ir 6#e(!o =#e la( mani1e(!acione(6#lmonare(enoca(ione(6rece'en vario( me(e( o aAo( a la( mani1e(!acione( $eneraliEa'a( m4(!%6ica(.La1ormam4(comHne(lane#mon%a in!er(!icial con 6a#!a i(!o6a!ol$ica ine(6ec%1ica..Esclerosis generalizada progresiva.La(6r#e@a('ela1#ncin6#lmonarCre(!ric!ivacon al!eracin 'e la ca6aci'a' 'i1#(ora; 1rec#en!emen!e an!e( 'e=#ea6areEcan'a!o(cl%nico(ora'io$r41ico('e ne#mo6a!%a. *&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL ACOMPAVADA DE TRASTORNOS DEL TEWIDO CONWUNTIVO.Artritis reumatoide.M4(comHnenvarone(.Irri!acin6le#ralcono(in 'errame;ILDa(!aen)*S'elo(ca(o(;n'#lo( necro@i!ico(9n'#lo(re#ma!oi'e(in!ra6#lmonare(no ne#moconi!ico(:cono(incavi!acin;(%n'rome'e Ca6lan9ne#moconio(i(re#ma!oi'e:;i6er!en(in 6#lmonar(ec#n'ariaava(c#li!i(6#lmonarre#ma!oi'e; ne#mon%aor$aniEa!ivaBo@(!r#ccin'ela(v%a( re(6ira!oria( (#6eriore( 'e@i'a a la ar!ri!i( ari!enoi'e.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL ACOMPAVADA DE TRASTORNOS DEL TEWIDO CONWUNTIVO.Lupus eritematoso diseminado.La6le#ri!i(cono(in'erramee(lamani1e(!acinm4( 1rec#en!e.O!ra(Ca!elec!a(ia(;'i(1#ncin'ia1ra$m4!ica con6>r'i'a'evolHmene(6#lmonare(;en1erme'a' va(c#lar6#lmonar;emorra$ia6#lmonar;e'ema 6#lmonar#r>mico;ne#mon%ain1eccio(aBne#mon%a or$aniEa!iva.Lane#moni!i(lH6icaa$#'ae(raraB(e carac!eriEa6orca6ilari!i(6#lmonar=#e6ro'#ce emorra$ia alveolar. *&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL ACOMPAVADA DE TRASTORNOS DEL TEWIDO CONWUNTIVO.Polimiositis y dermatomiositis .Alre'e'or 'e +*S 6a'ece 'e ILD. .RaBo(Co6aci'a'e('i1#(a(re!ic#lare(Bno'#lare(cono (incom6onen!ealveolarcon6re'ileccin6orlaEona @a(al.An!ic#er6o(an!i"Wo"+=#e(e'iri$encon!rala i(!i'ilo !RNA (in!a(a..P#e'ea@er'e@ili'a''elo(mH(c#lo(re(6ira!orio(;lo c#al con!ri@#Be a la ne#mon%a 6or a(6iracin. .Sndrome de S!gren.E(!aen1erme'a'(eacom6aAa'ene#moni!i(lin1oc%!ica in!er(!icial;lin1oma;(e#'olin1oma;@ron=#ioli!i(B @ron=#ioli!i( o@li!eran!e. *&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL ACOMPAVADA DE TRASTORNOS DEL TEWIDO CONWUNTIVO.Di(nea'ee(1#erEoB!o(no6ro'#c!iva.La6re(en!acin 'elaen1erme'a'6#e'e(er@r#(caB1#lminan!eo@ien $ra'#al a lo lar$o 'e varia( (emana( o me(e(. E( 6o(i@le a@ercon(#mi'oel14rmaco'#ran!evario(aAo(an!e( 'e=#ea6areEca#nareaccin9amio'arona:o@ienla ne#mo6a!%aa6arece(emana(oaAo('e(6#>('e in!err#m6irel14rmaco9carm#(!ina:.Lama$ni!#'Bla $rave'a''elaen1erme'a'(#elen(er'irec!amen!e 6ro6orcionale(ala'o(i(.El!ra!amien!ocon(i(!een in!err#m6ir el me'icamen!o B me'i'a( 'e (o6or!e.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMOPAT-A INTERSTICIAL FARMACOLUGICA.P"#$E%N#S%S AL&E#LA" P'L(#NA" .FaB #n 'e1ec!o 'e la 1#ncin macro14$ica; concre!amen!e lainca6aci'a''e6roce(arlo(!en(oac!ivo(.Se carac!eriEa6orlaac#m#lacin'ema!erial li6o6ro!ein4ceo amor1o B 6o(i!ivo 6ara 4ci'o 6erB'ico 'e Sci11 en lo( e(6acio( a>reo( 'i(!ale(.*&"*7")*+,Dra. Vernica Llamoca PalominoNEUMON-A EOSINUFILA.L%N)AN*%#LE%#(%#(A$#S%S P'L(#NA".Poco1rec#en!e=#ea1ec!aala(m#( 'e la oo1orec!om%a. *&"*7")*+,Dra. Vernica Llamoca PalominoNEUMON-A EOSINUFILA.El(%n'rome'eFerman(kB"P#'lake(#n!ra(!orno a#!o(micorece(ivo=#e(eacom6aAa'ecoli!i( $ran#loma!o(aBILD.Secarac!eriEa6oral@ini(mo oc#loc#!4neo;'i4!e(i(emorr4$ica(ec#n'ariaa 'i(1#ncin6la=#e!ariaBac#m#lacin'ecromol%6i'o; ma!erial li6o1#(c%nico =#e (e enc#en!ra en la( c>l#la( 'el (i(!ema re!ic#loen'o!elial. *&"*7")*+,Dra. Vernica Llamoca PalominoTRASTORNOS FEREDITARIOS 8/ F#en!e( 'e in1ormacin.Lon$oD;Fa#ciA;Ka(6erD;Fa#(erS;Wame(onL; Lo(calEoW.FARRISONXSPRINCIPLESOFINTERNAL MEDICINE.+7! E'icin. Mc GraY Fill Me'ical. )*+)..Farrera(P;RoEmanC.FARRERASQROMMAN MEDICINAINTERNA.Decimo(>6!imaE'icin.El(evier. )*+)..)*+, U6ToDa!e.*&"*7")*+,Dra. Vernica Llamoca Palomino++"*7")*+, Dra. Vernica Llamoca Palomino