7 Pneumopatii interstitiale difuze

8/14/2019 7 Pneumopatii interstitiale difuze

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Pneumopatii interstitiale difuze

Boli difuze parenchimatoase

pulmonare

Dr. Irina Strambu

Sef lucrari


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Grup mare de afectiuni de cauze diverse

Afectare difuza a parenchimului pulmonar

Manifestari clinice similare

Sindrom functional similar

Aspecte imagistice caracteristice

Aspecte histopatologice variabile


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PID = boala inflamatorie a parenchimuluipulmonar (alveolita)

PID fibroza pulmonara Doar unele dintre PID evolueaza catre fibroza

ireversibila

Altele pot regresa / stationa


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Etiologie

Factori externi

Medicamente (amiodarona, nitrofurantoin, aur, metotrexat,ciclofosfamida, bleomicina) Infectii (imunocompromisi: Pn. Carinii, legionella, aspergillus, virusuri) Alergeni (alveolita alergica extrinseca) Pulberi

Boli sistemice Colagenoze (LES, PR, Scleroza sistemica, sdr. Sjogren, miopatii) Vasculite (Wegener, Churg-Strauss, poliangeita microscopica) autoimune

Sarcoidoza Histiocitoza X Limfangioleiomiomatoza Plamanul eozinofil Sdr. de hemoragie alveolara Fibroza pulmonara idiopatica (criptogenetica) etc


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Diagnostic

Sindromul interstitial Clinic

Radiologic (+ CT)

Functional

Lavaj bronhoalveolar

Diagnosticul etiologic Anamneza

Manifestari extrapulmonare

Aspect CT si LBA sugestiv

Biopsie pulmonara


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Clinic

Dispnee progresiva de efort Tuse seaca

Inconstant

Febra

Degete hipocratice

Cianoza

Semnele IVD

Obiectiv Crepitante difuz bilateral (velcro)

Alte semne ale bolii de baza Eritem nodos, modificari articulare, eritem in fluture, modificari

cutanate, adenopatii


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Aspect radiologic

Radiografie pulmonara standard: sdr. Interstitial

Tomografie computerizata de inalta rezolutie (HRCT):informatii valoroase

Tip de leziuni

Localizare

Evolutivitate


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Radiografia standard

Sindrom interstitial Modificari difuze bilaterale

Noduli / micronoduli

Opacitati reticulare fine

Opacitati reticulo-nodulare Opacitati infiltrative vatoase bilaterale

Alte modificari sugestive Pleurezie (LES, PR)

Pneumotorax (linfangio-leiomiomatoza) Adenopatii hilare bilaterale (sarcoidoza)


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Tomografie computerizata

HRCT: detalii (sectiuni subtiri 1 mm, algoritm deamplificare)

Tipuri de leziuni: Noduli

Linii

Chisturi

Opacitati

Localizarea leziunilor


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Noduli densi: sarcoidoza, carcinomatoza, bronsiolita, pneumoconioza moi: AAE, bronsiolita

Linii Septale: insuf ventriculara stg, limfangita carcinomatoasa Reticulare: FPI, colagenoze, azbestoza, proteinoza Benzi parenchimatoase: cicatrici postpleurezie / pneumonie,

azbestoza

Chisturi Izolate: histiocitoza, LAM fagure de miere: azbestoza, FPI, colagenoza, sarcoidoza

Opacitati parenchimatoase Sticla mata: AAE, FPI descuamativa, toxicitate medicamentoasa,

proteinoza Condensare: pneumonia eozinofilica, carcinom bronhiolo-

alveolar, pneumonia lipoidica, proteinoza


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Localizare

Centolobulara: sarcoidoza, AAE, pneumoconioza

Septala: IVS, carcinomatoza, sarcoidoza

Subpleurala: FPI

Panlobulara: AAE, medicamente, FPI descuamativa Hipertransparenta panlobulara: TEP, emfizem

panlobular

Mozaic


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Fibroza pulmonara idiopatica: aspect reticular bazal bilateral


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Sdr. hemoragic alveolar: aspect infiltrativ vatos bilateral


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Sticla mata


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Sarcoidoza


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Sarcoidoza


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Sarcoidoza


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PR tratata cu saruri de aur


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PoliartritaReumatoidacu afectare pulmonara


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PR


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FPI


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FPI


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FPI


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FPI (fagure de miere)


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Fagure deMiere


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FPI, pneumotoraxdrenat


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FPI (acelasi caz)


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FPI (acelasi caz): fagure de miere


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FPI (acelasi caz)


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Functional

Sdr. restrictiv adevarat

Scadere CV, VR, CPT

Fara obstructie bronsica

Scadere (precoce) a TLCO

Constanta de transfer: scazuta Complianta pulmonara scazuta

Hipoxemie: initial la efort, apoi si in repaus

Test de efort (precoce)


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Bronhoscopie

Aspecte endoscopice normale

Utilitate:

Lavaj bronhiolo-alveolar Biopsie pulmonara transbronsica


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Lavaj bronhiolo-alveolar

Normal: Macrofage alveolare 80-90%

Limfocite 5-15%

PMN 1-3% Eozinofile < 1%

Mastocite


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Lavaj bronhiolo-alveolar


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LBA alte informatii

Carcinomatoza: celule tumorale

Infectii

bK

Pneumocystis carinii

Aspergillus Macrofage incarcate cu fier

Vasculite

Hemosideroza pulmonara idiopatica

Aspect laptos: proteinoza alveolara

Corpi azbestozici - asbestoza


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Diagnostic diferential

Tuberculoza pulmonara

Limfangita carcinomatoasa

Infectii Pneumocistoza

Pneumonii virale

Bronhopneumonie

ARDS


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Prelevare fragment bioptic pulmonar

Bronhoscopie: biopsie transbronsica

Biopsie cu ac fin sub CT (leziuni solide)

Toracoscopie

Biopsie chirurgicala deschisa (standard de aur)


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Etiologie

Factori externi

Medicamente (amiodarona, nitrofurantoin, aur, metotrexat,ciclofosfamida, bleomicina) Infectii (imunocompromisi: Pn. Carinii, legionella, aspergillus, virusuri) Alergeni (alveolita alergica extrinseca) Pulberi

Boli sistemice Colagenoze (LES, PR, Scleroza sistemica, sdr. Sjogren, miopatii) Vasculite (Wegener, Churg-Strauss, poliangeita microscopica) autoimune

Sarcoidoza Histiocitoza X Limfangioleiomiomatoza Plamanul eozinofil

Sdr. de hemoragie alveolara Fibroza pulmonara idiopatica (criptogenetica) etc


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Aspecte histopatologice

Specifice

Colagenoze Vasculite

Sarcoidoza

LAM

FPI 6 tipuri histologice Pneumonia interstitiala obisnuita (UIP)

Pneumonia interstitiala descuamativa (DIP)

Pneumonia interstitiala nespecifica (NSIP)

Pneumonia interstitiala acuta (AIP) BOOP

RB-ILD


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Sindrom interstitial clinicSindrom interstitial clinic

InvestigatiiInvestigatii

Radiografie standardRadiografie standard Explorari functionaleExplorari functionale

HRCTHRCT LBALBA

Prelevari histologicePrelevari histologice

AnamnezaAnamneza

Expunere laExpunere lafactori externifactori externi

Semne extrarespiratoriiSemne extrarespiratorii

Teste biochimiceTeste biochimice


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Anamneza

SexSex femininfeminin LAM ?LAM ?

VarstaVarsta> 50 ani> 50 ani Fibroza idiopaticaFibroza idiopatica

TanarTanar

Histiocitoza XHistiocitoza X

SarcoidozaSarcoidoza

ColagenozeColagenoze

FumatorFumatorHistiocitoza X, DIP, RB-ILDHistiocitoza X, DIP, RB-ILD

Infectat HIVInfectat HIV Pneumonia lipoidicaPneumonia lipoidica

PneumocistozaPneumocistoza


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Fibroza pulmonara idiopatica (UIP)

Clinic: 45-64 ani

Dispnee progresiva de efort Crepitante uscate bazale

Rx: reticulo-nodular, subpleural (sticla mata-precoce, fagure demiere-tardiv)

LBA

Celularitate mixta Neutrofile 10-30%

Absenta altor elemente anamnestice si de lab

Evolutie

Lenta stabilizare

Rapida deces in 6 luni (sdr. Hamman Rich) Complicatii

Insuficienta respiratorie cronica hipoxemica, CPC

Pneumotorax, infectii


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Tratament

Oala mare a bolilor tratabile cu cortizon

Initiere: 1 mg/kgc/zi prednison, 2 luni, scazut progresivpana la 1 an

Asociere: imunosupresive

Permit scaderea dozei de cortizon

Ciclofosfamida, metotrexat, azatioprina

Puls-terapie sau continuu

Control hematologic periodic

Oxigenoterapie de lunga durata, tratamentulcomplicatiilor


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Tratamente noi

Interferon gamma 1b

N-acetilcisteina

Bosentan

Transplant pulmonar


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LAM dupa transplant unipulmonar


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Monitorizare

De obicei la 3 luni

Se urmaresc: Clinic

Functional (TLCO, test de efort)

Radiologic

Evolutie: variabila de la caz la caz

Prognostic variabil


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Evolutie clinica

Variabila in functie de etiologie Stabilizare

Evoltie in pusee

Degradare progresiva continua

Complicatii Insuficienta respiratorie cronica hipoxemica

HTP, cord pulmonar cronic

Infectii (imunosupresie cortizonica)


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Lista de abrevieri

PID = pneumopatii interstitiale difuze FPI = fibroza pulmonara idiopatica LAM = limfangioleiomiomatoza AAE = alveolita alergica extrinseca LES = lupus eritematos sistemic PR = poliartrita reumatoida HRCT = tomografie computerizata cu rezolutie inalta TEP = trombembolism pulmonar IVS = insuficienta ventilatorie stanga CPT + capacitate pulmonara totala CV = capacitate vitala VR = volum rezidual TLCO = factor de transfer gazos prin membrana alveolo-capilara ARDS = sindrom de detresa respiratorie acuta LBA = lavaj bronhiolo-alveolar BOOP = bronsiolita obliteranta cu pneumonita de organizare RB-ILD = pneumopatia interstitiala legata de bronsiolita

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7 Pneumopatii interstitiale difuze