Post on 30-May-2018
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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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Tomografie computerizata
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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Tomografie computerizata
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