8 pneumopatii interstitiale dfuze
TRANSCRIPT
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Pneumopatii interstitiale 2
Dragos BUMBACEA
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2
db 2006
Clasificare
Pneumonite de hipersensibilitate (PH) Manifestari pulmonare in colagenoze:
Fibroza interstitiala difuza Hipertensiunea pulmonara Pleurezie Alte
Alte boli: Limfangioleiomiomatoza (LAM) Histiocitoza pulmonara cu celule Langerhans (HPCL) Bronsiolita obliteranta cu pneumonie in organizare (BOOP)
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db 2006
PH
Reactie imunologica la un agent inhalat, in
special un antigen organic, care se
produce la nivelul parenhimului pulmonar
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4
db 2006
Etiologie (> 300 cauze)
Fermieri (cultivatori plante, lucratori in
laptarii)
Contaminarea sistemelor de ventilatie silegata de apa (dusuri, cazi baie, sauna,
piscina, etc.)
Legata de pasari (crescatori, prelucrareapasarilor)
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Clasificare
PH acuta expunere masiva acuta
PH subacuta expunere persistenta la un nivel ridicat
PH cronica expunere cronica, la nivel mic
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PH acuta
debut brusc (4-6 ore) cu febra, frisoane,
greata, tuse, constrictie toracica, dispnee
fara wheezing
examen fizic: raluri crepitante difuze,
polipnee
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PH acuta CT
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PH subacuta
debut progresiv cu tuse productiva, dispnee,
astenie fizica, anorexie, scadere ponderala
uneori episoade acute rare cu tuse si alterarea
starii generale
examen fizic: polipnee, raluri crepitante difuze
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PH subacuta CT
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PH cronica
de obicei absenta episoadelor acute
debut insidios cu tuse, dispnee, astenie fizica,scadere ponderala
in final tablou de fibroza pulmonara difuza
examen fizic: hipocratism digital (formeavansate),
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PH cronica - CT
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Diagnostic
expunere cunoscuta la un agent incriminat expunere investigatia mediului precipitine
Tablou clinic, radiologic si functional compatibil Limfocitoza in LBA cu CD4/CD8 scazut Test de provocare inhalatorie (mediu sau
artificial) Tablou histopatologic: granuloame non-
cazeoase sau infiltrat mononuclear
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db 2006
Diagnostic diferential
Alte boli determinate de expunere la antigene
organice: febra de inhalatie, sindromul de praf
organic toxic, bronsita cronica, astm Alte boli cu tablou clinic, Rx si functional
similare: sarcoidoza
fibroza pulmonara idiopatica fibroza pulmonara din colagenoze
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Tratament si prognostic
PH acuta: disparitia simptomelor (12 ore -
zile) si a manifestarilor Rx si functionale
(saptamani) PH subacuta: rezolutie completa in
saptamani-luni, uneori necesita CS
PH cronica: rezolutie partiala, deseorinecesita CS
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Limfangioleiomiomatoza (LAM)
Rara, femei de varsta fertila
Proliferarea interstitiala atipica a muschiului
neted + formare de chiste Patogenie necunoscuta:
Pierderea functiilor de supresie tumorala a unor
enzime
Anomalii ale proteinelor implicate in sinteza
catecolaminelor
Estrogeni
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LAM -Hp
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LAM - diagnostic
Dispnee; wheezing, tuse, hemoptizie, sputa
Complicatii:
Pneumotorax spontan: frecvent, recurent, posibilbilateral
Chilotorax, rar chiloperitoneu, f. rar chilopericard
Limfangioleiomioame (edeme, dureri abdominale)
Angiolipoame renale Hemoptizii (chiar masive)
Meningioame
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LAM
RgP
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LAM
CT
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LAM functia pulmonara
Sindrom obstructiv sau mixt
Hiperinflatie ( CPT), cu air-trapping (VR si a VR/CPT)
Scaderea reculului elastic
Scaderea capacitatii de difuziune (DLco)
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LAM - diagnostic
Femeie tanara cu emfizem
pneumotorax recurent chilotorax
Confirmare HRCT
Rareori biopsie pulmonara necesara
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LAM diagnostic diferential
Emfizem (deficit alfa-1 antitripsina)
Astm
Histiocitoza pulmonara cu celule
Langerhans
Sarcoidoza chistica stadiul IV
Scleroza tuberoasa
Pneumonita de hipersensibilitate cronica
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LAM prognostic si tratament
Supravietuire 8-10 ani (mai putin in
sarcina sau administrare de estrogeni)
Tratament: Ovarectomie bilaterala
Progestative
Evitarea sarcinii? Transplant pulmonar
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Histiocitoza Pulmonara cu Celule
Langerhans (HPCL)
Cauza necunoscuta Fumatul este factor predispozant / cauzal
Celula Langerhans = celula diferentiata dinlinia monocito-macrofagica Incluziuni citoplasmatice pentalaminare (corpi X)
Prezinta S100 si CD1 Aglomerate in grupuri
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HPCL
ME
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HPCL
MO
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HPCL
MO
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HPCL manifestari
Simptome: tuse neproductiva, dispnee, dureretoracica de tip pleuritic, astenie, scadereponderala, febra
Complicatii: Pneumotorax spontan recurent Hipertensiune pulmonara Hemoptizie
Tumori maligne sau benigne Chiste osoase Diabet insipid (leziune hipotalamica)
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HPCL RgP + CT
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HPCL functia pulmonara
Volume si debite normale
DLco in mod disproportionat
Rar sindrom restrictiv
Foarte rar sindrom obstructiv
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Diagnostic
Lavaj bronhoalveolar: > 5% celuleLangerhans (CD1+)
Biopsie pulmonara S-100 CD1a Rar microscopie electronica
Forme avansate (fibrozante) reducereanumarului de celule Langerhans
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Tratament
Oprirea fumatului ??? corticosteroizi + citotoxice ???
Tratamentul complicatiilor (pleurodeza) Transplant pulmonar
Prognostic Evolutie accelerata la fumatori Posibil remisiune la oprirea fumatului Supravietuirea mediana: 12,4 ani
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Bronsiolita obliteranta cu pneumonie in
organizare (BOOP) idiopatica (COP)
Proliferare excesiva a tesutului de
granulatie in caile aeriene mici (bronsiolita
proliferativa) si ducturile alveolare cuinflamatia cronica a alveolelor din jur
In absenta altor cauze (idiopatica)
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COP Hp
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COP
RgP
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COP
RgP
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COP CT
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COP - LBA
Tablou mixt: cresterea limfocitelor,
neutrofilelor, eozinofilelor si mastocitelor
Raport CD8/CD4 crescut
Relativ specific
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Diagnostic
Context clinico-radiologic si LBA sugestiv
In absenta: Pneumonie SDRA Pneumonita de hipersensibilitate Pneumonie eozinofilica cronica Pneumopatie medicamentoasa Determinare pulmonara in colagenoze
Se recomanda biopsie pulmonara toracoscopica
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Tratament
Corticoterapie prednison 1-1,5 mg/kgc in dozaunica matinala timp de 4-8 saptamani, apoiscadere progresiva, si oprit in 3-6 luni (in functie
de evolutie) +/- citotoxic ciclofosfamida
Prognostic bun Uneori recidiva dupa oprirea CS Rareori fibroza difuza cu insuficienta respiratorie
db 2006
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Granulomatoza Wegener (GW)
Afectare pulmonara
Afectare a cailor aeriene superioare
Afectare renala
Alte
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GW - manifestari
rinoree persistenta, purulenta sau
hemoragica, ulcere nazale sau orale,
durere sinusala tuse, hemoptizie, dispnee, durere toracica
uneori semne de condensare
febra,anorexie, scadere ponderala, staregenerala alterata
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GW - CT
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GW laborator
sindrom inflamator
retentie azotata
proteinurie, hematurie, leucociturie, cilindri
eritrocitari si leucocitari
ANCA (antineutrophil cytoplasmic antibodies)
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GW tratament si prognostic
Ciclofosfamida + corticosteroid Azatioprina pentru continuare
Metotrexat
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Afectarea pulmonara in PR
Pneumopatie interstitiala difuza BOOP
Noduli reumatoizi Sindrom Caplan Pleurezie reumatoida Infectii pulmonare (pneumonie, tuberculoza)
Pneumopatie medicamentoasa (metotrexat,saruri de aur)
Bronsiectazii
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Noduli reumatoizi RgP + CT
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Afectarea pulmonara in LES
Pleurezie lupica
Pneumonita acuta lupica
Pneumonita cronica lupica
Hipertensiune arteriala pulmonara
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Afectarea pulmonara din sclerodermie,
sindromul Sjogren
Fibroza pulmonara difuza
Hipertensiune arteriala pulmonara
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Sclerodermie fibroza pulmonara
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Sclerodermie fibroza pulmonara
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Sclerodermie HTAP