1. clasificarea pid.pdf

8/19/2019 1. Clasificarea PID.pdf

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CLASIFICAREA PNEUMOPATIILOR

INTERSTITIALE DIFUZE

Ionela Belaconi

Institutul de Pneumoftiziologie “Marius Nasta”,Bucuresti

Sibiu, 2014


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Scopuri

• Ce sunt pneumopatiile interstitiale difuze

• Care sunt cauzele PID

• Familiarizarea cu noua clasificare a PID

• Boli rare si noi entitati aparute

• Boli care nu se incadreaza in tipare


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Ce se intampla in plaman ?

• Acumulare de tesut

inflamator, cicatriceal sau colagen

• Afectarea schimbului gazos

• In timp -> distrugerea structurii

plamanului

•

Leziuni in “fagure de miere”

www.lungsandyou.com/what-is-IPF


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Plaman cu PID

Plaman cu FPI Plaman cu azbestozaPlaman normal


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Pneumopatiile interstitiale difuze

• Afectare pulmonara difuza interstitiala

• Histopatologic:

– Predomina inflamatie & fibroza

• Etiologie cunoscuta vs necunoscuta

– Predomina reactie granulomatoasa

• Etiologie cunoscuta vs necunoscuta

• Entitati diferite care manifesta similaritati

– Clinice, radiologice, functionale, patologice


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Departajare

Diagnostic diferential si clasificare:

- cauze cunoscute

- cauze necunoscute (idiopatice)

Istoricul este imperativ:

- expunere de mediu/ocupationala

- medicamente

- radiatii

Comorbiditati: BTC


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Pneumopatiile interstitiale difuze

PID de cauze

cunoscute: asoc.

BTC, mediu ,

medicamente

Pneumopatii

interstitiale

idiopatice

PID

granulomatoase:

sarcoidoza

Alte forme PID:

LAM, HX, pn.

eozinofilica

FPI Altele

Pneumopatie interst.

descuamativa


acuta


nespecifica

Bronsiolita

respiratorie - BPI

Pneumopatie

criptog. in organizare


limfocitara

ATS/ERS International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Resp Crit Care Med,

Vol 165, pp 277-304, 2002


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• PII – cuprinde 7 entitati clinico-patogenice

suficient de diferite

• Boli rare, putini medici au experienta

suficienta

• Frecventa: FPI, NSIP, COP, AIP, RB-ILD, DIP, LIP

•

NSIP –

arie de incertitudine• HRCT


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Fibrozele pulmonare idiopatice (FPI)

• Leziuni ale celulelor alveolare epiteliale;apoptoza

• Transformare epitelial -> mezenchimal

• Celulele mezenchimale (miofibroblaste,fibroblaste, fibrocite)

• Predispozitie genetica:

– Proteinele surfactantului – Telomere si telomeraza

– Polimorfism MUC5B


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Clasificarea ATS/ERS 2002 a Pneumopatiilor

Interstitiale Idiopatice (PII)

Tip Histologic Diagnostic clinic

UIP (Pneumopatie interstitiala uzuala) Fibroza pulmonara idiopatica

NSIP (Pneumopatie inters. ne-specifica) NSIP (Pneumopatie inters. ne-specifica)

Pneumopatie in organizare COP (Pneumopatie criptogenica in

organizare)

DAD (Leziuni alveolare difuze) AIP (Pneumopatie interstitiala acuta)

Bronsiolita respiratorie RB-ILD (Bronsiolita respiratorie – boala

pulmonara interstitiala)

DIP (Pneumopatie interstitiala

descuamativa)

DIP (Pneumopatie interstitiala

descuamativa)

LIP (Pneumopatie interstitiala limfocitara) LIP (Pneumopatie interstitiala limfocitara)

Pneumopatii interstitiale neclasificabile (?)

Travis WD, King TE, Bateman ED, et al. ATS/ERS international multidisciplinary consensus classification of idiopathic interstitial

pneumonias. General principles and recommendations. Am J Respir Crit Care Med 2002; 165: 277 –304


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Noutati in aceasta decada

• Acute Exacerbations of Idiopathic Pulmonary FibrosisHarold R. Collard, Bethany B. Moore, Kevin R. Flaherty, Kevin K. Brown, Robert J. Kaner, Talmadge E. King, Jr., Joseph A. Lasky, James E. Loyd,Imre Noth, Mitchell A. Olman, Ganesh Raghu, Jesse Roman, Jay H. Ryu, David A. Zisman, Gary W. Hunninghake, Thomas V. Colby, Jim J. Egan,David M. Hansell, Takeshi Johkoh, Naftali Kaminski, Dong Soon Kim, Yasuhiro Kondoh, David A. Lynch, Joachim Mu¨ller-Quernheim, Jeffrey L.Myers, Andrew G. Nicholson, Moise´s Selman, Galen B. Toews, Athol U. Wells, and Fernando J. Martinez, with the Idiopathic PulmonaryFibrosis Clinical Research Network Investigators

Am J Respir Crit Care Med Vol 176. pp 636 –643, 2007

• Idiopathic Nonspecific Interstitial Pneumonia - Report of an American Thoracic SocietyProject

William D. Travis, Gary Hunninghake, Talmadge E. King, Jr., David A. Lynch, Thomas V. Colby, Jeffrey R. Galvin, Kevin K. Brown, Man PyoChung, Jean-Francxois Cordier, Roland M. du Bois, Kevin R. Flaherty, Teri J. Franks, David M. Hansell, Thomas E. Hartman, Ella A. Kazerooni,Dong Soon Kim, Masanori Kitaichi, Takashi Koyama, Fernando J. Martinez, Sonoko Nagai, David E. Midthun, Nestor L. Mu¨ller, Andrew G.Nicholson, Ganesh Raghu, Moise´s Selman, and Athol Wells

• An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-basedGuidelines for Diagnosis and Management

Ganesh Raghu, Harold R. Collard, Jim J. Egan, Fernando J. Martinez, Juergen Behr, Kevin K. Brown, Thomas V. Colby, Jean-Francxois

Cordier, Kevin R. Flaherty, Joseph A. Lasky, David A. Lynch, Jay H. Ryu, Jeffrey J. Swigris, Athol U. Wells, Julio Ancochea,Demosthenes Bouros, Carlos Carvalho, Ulrich Costabel, Masahito Ebina, David M. Hansell, Takeshi Johkoh, Dong Soon Kim,Talmadge E. King, Jr., Yasuhiro Kondoh, Jeffrey Myers, Nestor L. Mu¨ller, Andrew G. Nicholson, Luca Richeldi, Moise´s Selman,Rosalind F. Dudden, Barbara S. Griss, Shandra L. Protzko, and Holger J. Schu¨nemann, on behalf of the ATS/ERS/JRS/ALATCommittee on Idiopathic Pulmonary FibrosisAm J Respir Crit Care Med Vol 183. pp 788 –824, 2011


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Revizuirea clasificarii PII

• NSIP acceptata si definita

• PID asociate fumatului – mai bine intelese sidefinite

• Exacerbarile acute – mai bine definite• PII – tipuri mixte

• Entitati rare, tipuri histopatologice

•Clasificare clinica mai pragmatica

• Genetica si mostenirea pneumopatiilorinterstitiale


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Clasificarea revizuita PII ATS/ERS 2012 -

diagnostic multidisciplinar

MAJORE

Fibroza pulmonara idiopatica (FPI)

Pneumopatie Interstitiala Nespecifica Idiopatica (NSIP)

Bronsiolita respiratorie – Boala Pulmonara Interstitiala (RB-ILD)

Pneumopatie interstitiala descuamativa (DIP)

Pneumonie criptogenica in organizare (COP)

Pneumopatie interstitiala acuta (AIP)

RARE

Pneumopatie interstitiala limfocitara idiopaticaFibroelastoza pleuro-pulmonara idiopatica *

NECLASIFICABILE (10-25%)

* Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes

Taryn L. Reddy, Masaki Tominaga, David M. Hansell, Jan von der Thusen, Doris Rassl, Helen Parfrey, Suzy Guy, Orion Twentymane,Alexandra Rice, Toby M. Maher, Elisabetta A. Renzoni, AtholU.Wells and AndrewG.Nicholson

Eur Respir J 2012; 40: 377 –385


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Pneumopatii Interstitiale Difuze

PID de cz cunoscuta

e.g. medicam. sau b.

vasc. de colagen

Pneumopatii interstitiale

idiopatice

(PII)

PID

granulomatoase

e.g. sarcoidoza

Alte forme de

PID e.g. LAM,

HisX, etc

Non-Familiale Familiale

(>80%) (2-20%)

Cronice FibrozanteAcute/Subacute

FibrozanteAsoc. cu fumatul

- Fibroza pulmonara

idiopatica- Pneumopatie interstitiala

nespecifica idiopatica

- Pneumopatie

criptogenica inorganizare

- Pneumopatie

interstitiala acuta

- Bronsiolita respiratorie –

b. pulm. interstitiala- Pneumopatie interst.

descuamativa

PII rare:

- Pneumopatie interstitiala limfocitara (LIP)

- Fibroelastoza idiopatica pleuroparenchimatoasa (PPFE)Modified from American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. (2013). Am Respir

–


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Clasificarea revizuita PII ATS/ERS 2012

• Ca si grup PII pot fi diferentiate de alte forme

de PID prin metode clinice si paraclinice (ce

includ anamneza, ex. fizic, imagistica, analize

de laborator si histopatologia)


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Idiopathic Interstitial Pneumonia What Is the Effect of a Multidisciplinary Approach to

Diagnosis?

Kevin R. Flaherty, Talmadge E. King, Jr., Ganesh Raghu, Joseph P. Lynch III, Thomas V. Colby,

William D. Travis, Barry H. Gross, Ella A. Kazerooni, Galen B. Toews, Qi Long, Susan Murray,

Vibha N. Lama, Steven E. Gay, and Fernando J. MartinezAm J Respir Crit Care Med Vol 170. pp 904 –910, 2004

• Un diagnostic multidisciplinar, realizat dupa o

reevaluare atenta a informatiilor clinice,radiologice si histopatologice de obicei difera dediagnosticul dat individual de clinician, imagistsau anatomopatolog lucrand separat

• Informatia histopatologica are cel mai mareimpact asupra dg final, mai ales daca dg initialclinic si imagistic nu e de IPF


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“Obtinerea unui diagnostic corect este

un proces dinamic” Clinic

- Anamneza

- Ex. fizic

- Laborator

- PFC

Radiologic

- Rx. torace

- CT cu cupe fine

Anatomopatologic

- Biopsie pulmonara

Abordare multidimensionala si

multidisciplinara

ChirurgPneumolog Radiolog Anatomopatolog


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• Pacientii la care s-a ridicat suspiciunea de de

PID trebuie trimisi in centre specializate

pentru a incerca un diagnostic de certitudine

si pentru sugestii in vederea tratamentului



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• HRCT este o componenta esentiala a dg PII

• Rolul LBA se crede ca este in declin

•

Biopsiile transbronsice nu sunt utile indiagnosticul PII (utile in sarcoidoza sau pt

excluderea unor infectii)

• Biopsiile pulmonare sunt sigure


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• Odata ce anatomopatologul recunoaste un

anumit tip histopatologic…..clinicianul tb sa

reconsidere diagnosticul


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• O mai buna intelegere a suprapunerilor intre

tipurile histologice si HRCT

– Mai multe tipuri pot fi gasite la acelasi pacient

(mai ales la fumatori)

– Cand apare asa ceva abordarea multidisciplinara

poate determina semnificatia clinica a fiecarui tip


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Tipuri ce pot coexista

HRCT sau Biopsie

• UIP si NSIP (UIP discordant)

• Fumator cu UIP sau NSIP - complica

interpretarile

• Exacerbari acute (DAD) peste UIP sau NSIP

• Fibroza si emfizem


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Idiopathic Nonspecific Interstitial Pneumonia - Report of an American

Thoracic Society Project

William D. Travis1, Gary Hunninghake, Talmadge E. King, Jr., David A. Lynch,

Thomas V. Colby, Jeffrey R. Galvin, Kevin K. Brown, Man Pyo Chung, Jean-

Francxois Cordier, Roland M. du Bois, Kevin R. Flaherty,Teri J. Franks, David M. Hansell, Thomas E. Hartman, Ella A. Kazerooni, Dong

Soon Kim, Masanori Kitaichi, Takashi Koyama, Fernando J. Martinez, Sonoko

Nagai, David E. Midthun, Nestor L. Mu¨ller, Andrew G. Nicholson, Ganesh

Raghu, Moise´s Selman, and Athol Wells

Am J Respir Crit Care Med Vol 177. pp 1338 –1347, 2008

- NSIP idiopatic reprezinta o entitate clinica

distincta, ce apare de obicei la femei de varsta

mijlocie si nefumatoare, cu un bun prognosticpe termen lung


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Sumarul modificarilor majore

• Entitatile principale raman nemodificate – NSIP idiopatic este acceptat ca entitate clinica distincta

• PII majore sunt grupate in cronice fibrozante, legate de

fumat si acute/subacute

• PII majore vs rare si cazuri neclasificabile

•Este propusa o clasificare pe baza comportamentuluiclinic

• Sunt revizuite tipurile moleculare si genetice intalnite


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Va multumesc

Pentru atentie si interes pentru domeniul

bolilor pulmonare interstitiale!

1. clasificarea pid.pdf

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