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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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    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

    SarcoidozaSa

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