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Claudia Toma
Universitatea de Medicina si Farmacie Carol Davila
Institutul de Pneumoftiziologie Marius Nasta
Bucuresti
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Boli pulmonare
parenchimatoase
difuze (DPLD)
De cauza cunosc.(medicam)/
Colagenoze (PR)
/LIP
Pneumopatiiinterst idiopatice
(PII)
Fibrozante
cronice
Fibroza pulm.Idiopatica -
FPI (UIP)
PII non-
specifica
(NSIP)
Boligranulomatoase
(sarcoidoza, PN HSb)
Alte forme(LAM, Histiocitoza
cu cel Langerhans),
Pneumonia eozinof.
Non-familiale Familiale
(>80%) (2-20%)
Fibrozante
acute/subacute
Legate de
fumat
Pneumonia
criptogenica
in organizare
(BOOP/COP)
Pneumonia
interstitiala
acuta (AIP)
Pneumonia
interstitiala asociata
bronsiolitei resp.
(RB-ILD)
Pneumonia
interstitiala
descuamativa (DIP) 2014
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Pneumopatie interstitiala
(imunocompetent)
Istoric, Ex. Clinic, Lab (uzuale), Rx (vechi/recente), PFR
(stabilirea cronicitatii/progresiei/stabilitatii)
Niciun alt demers
diagnostic
Recuperare clinica?
2014
Inlaturare/evitare a unei cauze potential identificabile
(ambientala/iatrogena)
Demers diagnostic
Serologie (colagenoze)
Biopsie (cutan, mm, sinus/sept nazal, ren.)
Boala sistemica specifica?
DA NU
DA NU
Evaluare +
Management
al bolii respective
HRCT
(analiza pl./ ple./mediast. pt
stabilire pattern PID +
alegere loc de LBA/biopsie)
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2014
Sugestiv de:
Sarcoidoza, Berilioza,
Pneumonita de HSb,Limfangita carcinom,
Histiocitoza, Pneumonie
eozinof.
Elemente clasice de UIP?DA
NU
Bronhoscopie cu LBA si
Biopsie transbronsica
HRCT
(analiza pl./ ple./mediast. pt
stabilire pattern PID +
alegere loc de LBA/biopsie)
Fibroza pulm. idiopatica
(nu necesita alta confirmare)
Non-diagnostice Diagnostice
Pattern incert / sugestiv
de NSIP, BOOP/COP
Biopsie pulm. chirugicala
(toracosopie/toracotomie)
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Sindrom interstitial
Opacitati micronodulare
Opacitati liniare/reticulare
“Geam t”
“Fagure de miere”
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Normala Opacitati
micronodul.
Opacitati
liniare/retic.
Condensari
alveolare
Infiltrate
fugace
Sarcoidoza Sarcoidoza Sarcoidoza Hemoragiaalveolara
BOOP/COP
PN HSb PN HSb PN HSb Pneumonia
eozinofilica
Sdr. Loeffler
Azbestoza Silicoza Pneumoconioze BOOP/COP ABPA
Bronsiolitaobliteranta
Bronsiolitaobliteranta
Histiocitoza Proteinoza HSb lamedicamente
Colagenoze Carcinomatoza Colagenoze
FPI (initial) FPI
LAM LAM
Pneumonitaradica
Medicamente
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Apicala Bazala Periferica
Boli granulomatoare- Sarcoidoza- Histiocitoza
- PN HSb
FPI BOOP/COP
Pneumoconioze- Silicoza- Berilioza
PR asociata cu UIP Pneumonia eozinofilica
Colagenoze- PR- Spondilita ankilop.
Azbestoza
Pneumonita radica
Medicamente
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Bronsiolita obliteranta
PN HSb
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Miliara TB
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Carcinomatoza pulmonara
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Pneumonie atipica
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Histiocitoza cu cel. Langerhans
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NSIP
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BOOP/COP
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Proteinoza alveolara
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LAM
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Microchiste
Histiocitoza cu cel. Langerhans
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FPI
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HRCT (rezolutie inalta, cupe fine)Nativ/ cu substanta de contrast
Indicata in urmatoarele situatii:
• Simptome persistente (dispnee/tuse)
+Rx cu sdr. interstitial /normala
• In absenta unei boli obstructive/insuficienteicardiace
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Opacitati
micronodul.
Opacitati
liniare/retic.
“
Geam
t”
Condensari
alveolare
Chiste
PN HSb PN HSb PN HSb Pneumoniaeozinofilica
Histiocitoza
RB-ILD Colagenoze RB-ILD COP LAM
Sarcoidoza Azbestoza DIP Proteinoza
Silicoza FPI Medicamente Limfom
Histiocitoza Hemoragie
alveolara
Hemoragie
alveolara
Carcinomatoza
Pneumonialipoida
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Barbat 62 ani
Dispnee
Artralgii
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Femeie 43 ani
Dispnee progresivade efort
Tuse seaca PFR – sdr. restrictiv
TLco
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Barbat 60 ani
Fumator 20PA
Tuse seaca
Dispnee PFR – sdr restrictiv
usor, TLco
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Barbat 62ani
Dispnee de efort
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Barbat, 31 ani
Nefumator
Dispnee progresiva de
efort PFR – sdr. restrictiv
sever; TLco
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Microlitiaza alveolara
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Barbat, 31 ani
Fumator – 20PA Dispnee progresiva de
efort de 1an
PFR – VR , TLco
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B, 20 ani
Fumator
Diabet insipid
Dispnee la eforturimici
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Histiocitoza cu cel. Langerhans
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Femeie, 42 ani
Dispnee progresiva de
efort PFR – sdr. restrictiv
– VR , TLco
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Barbat 29 ani
Dispnee recenta
Tuse seaca
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Bronsiolita obliteranta
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Barbat, 74 ani
Febra
Tuse seaca Dispnee
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Barbat 21 ani
Astm controlatterapeutic
Infiltrat in urma cu1 an LSD – trat. antiTB
Subfebra
Tuse
Eozinofilie sg.(1200/mmc)
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Iun 2014 Aug 2014
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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Femeie 70 ani
Tuse cu expectoratiemuco-purulenta de 2
sapt. Hemoptizii de 4 zile
Artralgii
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De cauza cunoscuta colagenoze, medicam.)
Boli granulomatoase sarcoidoza, PN HSb)
Boli metabolice proteinoza, amiloidoza,
microlitiaza)
Boli cu formare de chiste pulmonare
Histiocitoza, LAM)
Bronsiolite, BOOP/COP
Pneumonia eozinofilica
Boli vasculare
Pneumopatiile interstitiale difuze idiopatice
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B, 45 ani Fumator 25PA
Dispnee de 9 luni
Rx toracica - normala
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Barbat 65 ani
Fumator 40PA
Dispnee si tuse seaca
de 1 an Hipocratism digital
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Raghu G. - Am J Respir Crit Care Med Vol 183. pp 788–824, 2011
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PID– grup heterogen de boli grupate impreuna
datorita similitudinilor clinice si radiologice
Diagnosticul diferential – numar mare de boli
cu cauza cunoscuta/necunoscuta
HRCT are importanta majora in diagnostic
(decubit dorsal/ventral; inspir/expir;nativ/contrast)
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Dragos Bumbacea
Casandra Cilt
Ionela Belaconi
Stefan Dumitrache-Rujinski