Pneumopatii interstitiale difuze

Boli difuze parenchimatoase

pulmonare

Dr. Irina Strambu

Sef lucrari
• 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
• 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 bronhiolo-
alveolar, 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
• 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

Expunere la
factori externi
Investigatii Anamneza
Semne extrarespiratorii

Teste biochimice

Radiografie standard Explorari functionale

HRCT LBA

Prelevari histologice
Anamneza

Sex feminin LAM ?

> 50 ani Fibroza idiopatica

Varsta

Tanar Sarcoidoza

Histiocitoza X

Colagenoze

Fumator Histiocitoza X, DIP, RB-ILD

Infectat HIV 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