7 Pneumopatii Interstitiale Difuze

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

• PID = boala inflamatorie a parenchimului pulmonar (alveolita) • PID ≠ fibroza pulmonara – Doar unele dintre PID evolueaza catre fibroza ireversibila – Altele pot regresa / stationa

Etiologie •

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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

Diagnostic • “Sindromul” interstitial – – – –

Clinic Radiologic (+ CT) Functional Lavaj bronhoalveolar

• Diagnosticul etiologic – – – –

Anamneza Manifestari extrapulmonare Aspect CT si LBA sugestiv Biopsie pulmonara

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 …

Aspect radiologic • Radiografie pulmonara standard: sdr. Interstitial • Tomografie computerizata de inalta rezolutie (HRCT): informatii valoroase – Tip de leziuni – Localizare – Evolutivitate

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)

Tomografie computerizata • HRCT: detalii (sectiuni subtiri 1 mm, algoritm de amplificare) • Tipuri de leziuni: – – – –

Noduli Linii Chisturi Opacitati

• Localizarea leziunilor

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 bronhioloalveolar, pneumonia lipoidica, proteinoza

Tomografie computerizata • Localizare – Centolobulara: sarcoidoza, AAE, pneumoconioza – Septala: IVS, carcinomatoza, sarcoidoza – Subpleurala: FPI – Panlobulara: AAE, medicamente, FPI descuamativa – Hipertransparenta panlobulara: TEP, emfizem panlobular – Mozaic

Fibroza pulmonara idiopatica: aspect reticular bazal bilateral

Sdr. hemoragic alveolar: aspect infiltrativ “vatos” bilateral

“Sticla mata”

Sarcoidoza

Sarcoidoza

Sarcoidoza

Acelasi caz dupa 4 ani

PR tratata cu saruri de aur

Poliartrita Reumatoida cu afectare pulmonara

PR

PR

FPI

FPI

FPI

FPI (“fagure de miere”)

“Fagure de Miere”

FPI, pneumotorax drenat

FPI (acelasi caz)

FPI (acelasi caz): “fagure de miere”

FPI (acelasi caz)

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)

Bronhoscopie • Aspecte endoscopice normale • Utilitate: – Lavaj bronhiolo-alveolar – Biopsie pulmonara transbronsica

Lavaj bronhiolo-alveolar • Normal: – – – – – –

Macrofage alveolare 80-90% Limfocite 5-15% PMN 1-3% Eozinofile < 1% Mastocite <1% Fara celule epiteliale, f. rare celule bronsice, fara hematii

Lavaj bronhiolo-alveolar

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

Diagnostic diferential • Tuberculoza pulmonara • Limfangita carcinomatoasa • Infectii – Pneumocistoza – Pneumonii virale – Bronhopneumonie

• ARDS

Prelevare fragment bioptic pulmonar • • • •

Bronhoscopie: biopsie transbronsica Biopsie cu ac fin sub CT (leziuni solide) Toracoscopie Biopsie chirurgicala deschisa (standard de aur)

Etiologie •

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• • • • • • •

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

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

“Sindrom interstitial” clinic

Investigatii

Expunere la factori externi

Anamneza

Semne extrarespiratorii Teste biochimice Radiografie standard

HRCT

Explorari functionale

LBA Prelevari histologice

Anamneza Sex

Varsta

feminin

LAM ?

> 50 ani

Fibroza idiopatica

Tanar

Sarcoidoza Histiocitoza X Colagenoze

Fumator Infectat HIV

Histiocitoza X, DIP, RB-ILD Pneumonia lipoidica (LIP) Pneumocistoza

Fibroza pulmonara idiopatica (UIP) • Clinic: 45-64 ani – Dispnee progresiva de efort – Crepitante uscate bazale • Rx: reticulo-nodular, subpleural (sticla mata-precoce, fagure de miere-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 …

Tratament • “Oala” mare a bolilor tratabile cu cortizon • Initiere: 1 mg/kgc/zi prednison, 2 luni, scazut progresiv pana la 1 an • Asociere: imunosupresive – Permit scaderea dozei de cortizon – Ciclofosfamida, metotrexat, azatioprina • Puls-terapie sau continuu • Control hematologic periodic • Oxigenoterapie de lunga durata, tratamentul complicatiilor

Tratamente noi • Interferon – gamma 1b • N-acetilcisteina • Bosentan • Transplant pulmonar

LAM dupa transplant unipulmonar

Monitorizare • De obicei la 3 luni • Se urmaresc: – Clinic – Functional (TLCO, test de efort) – Radiologic

• Evolutie: variabila de la caz la caz • Prognostic variabil

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)

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