CLASIFICAREA PNEUMOPATIILOR

INTERSTITIALE DIFUZE
Ionela Belaconi
Institutul de Pneumoftiziologie Marius Nasta,
Bucuresti
Sibiu, 2014

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

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

Plaman cu PID

Plaman normal

Plaman cu FPI

Plaman cu azbestoza

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

Departajare
Diagnostic diferential si clasificare:
- cauze cunoscute
- cauze necunoscute (idiopatice)
Istoricul este imperativ:
- expunere de mediu/ocupationala
- medicamente
- radiatii

Comorbiditati: BTC

Pneumopatiile interstitiale difuze

PID de cauze
cunoscute: asoc.
BTC, mediu ,
medicamente

PID
granulomatoase:
sarcoidoza

Pneumopatii
interstitiale
idiopatice

FPI

Alte forme PID:

LAM, HX, pn.
eozinofilica

Altele

Pneumopatie interst.
descuamativa

Bronsiolita
respiratorie - BPI

Pneumopatie interst.
acuta

Pneumopatie
criptog. in organizare

Pneumopatie interst.
nespecifica

Pneumopatie interst.
limfocitara

ATS/ERS International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Resp Crit Care Med,
Vol 165, pp 277-304, 2002

 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

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

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: 277304

Noutati in aceasta decada

Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Harold 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 Muller-Quernheim, Jeffrey L.
Myers, Andrew G. Nicholson, Moises Selman, Galen B. Toews, Athol U. Wells, and Fernando J. Martinez, with the Idiopathic Pulmonary
Fibrosis Clinical Research Network Investigators
Am J Respir Crit Care Med Vol 176. pp 636643, 2007

Idiopathic Nonspecific Interstitial Pneumonia - Report of an American Thoracic Society

Project

William D. Travis, 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. Muller, Andrew G.
Nicholson, Ganesh Raghu, Moises Selman, and Athol Wells

An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based

Guidelines 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. Muller, Andrew G. Nicholson, Luca Richeldi, Moises Selman,
Rosalind F. Dudden, Barbara S. Griss, Shandra L. Protzko, and Holger J. Schunemann, on behalf of the ATS/ERS/JRS/ALAT
Committee on Idiopathic Pulmonary Fibrosis
Am J Respir Crit Care Med Vol 183. pp 788824, 2011

Revizuirea clasificarii PII

NSIP acceptata si definita
PID asociate fumatului mai bine intelese si
definite
Exacerbarile acute mai bine definite
PII tipuri mixte
Entitati rare, tipuri histopatologice
Clasificare clinica mai pragmatica
Genetica si mostenirea pneumopatiilor
interstitiale

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 idiopatica
Fibroelastoza 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: 377385

Pneumopatii Interstitiale Difuze

PID de cz cunoscuta
e.g. medicam. sau b.
vasc. de colagen

Pneumopatii interstitiale
idiopatice
(PII)

Non-Familiale
(>80%)
Cronice Fibrozante

- Fibroza pulmonara
idiopatica
- Pneumopatie interstitiala
nespecifica idiopatica

PID
granulomatoase
e.g. sarcoidoza

Alte forme de
PID e.g. LAM,
HisX, etc

Familiale
(2-20%)

Acute/Subacute
Fibrozante
- Pneumopatie
criptogenica in
organizare
- Pneumopatie
interstitiala acuta

Asoc. cu fumatul

- 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

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)

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. Martinez
Am J Respir Crit Care Med Vol 170. pp 904910, 2004

Un diagnostic multidisciplinar, realizat dupa o

reevaluare atenta a informatiilor clinice,
radiologice si histopatologice de obicei difera de
diagnosticul dat individual de clinician, imagist
sau anatomopatolog lucrand separat
Informatia histopatologica are cel mai mare
impact asupra dg final, mai ales daca dg initial
clinic si imagistic nu e de IPF

Obtinerea unui diagnostic corect este

un proces dinamic
Clinic

Radiologic

- Anamneza

- Rx. torace

- Ex. fizic

- CT cu cupe fine

Anatomopatologic
- Biopsie pulmonara

- Laborator
- PFC

Pneumolog

Chirurg

Radiolog

Anatomopatolog

Abordare multidimensionala si
multidisciplinara

Clasificarea revizuita PII ATS/ERS 2012

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

Clasificarea revizuita PII ATS/ERS 2012

HRCT este o componenta esentiala a dg PII
Rolul LBA se crede ca este in declin
Biopsiile transbronsice nu sunt utile in
diagnosticul PII (utile in sarcoidoza sau pt
excluderea unor infectii)
Biopsiile pulmonare sunt sigure

Clasificarea revizuita PII ATS/ERS 2012

Odata ce anatomopatologul recunoaste un
anumit tip histopatologic..clinicianul tb sa
reconsidere diagnosticul

Clasificarea revizuita PII ATS/ERS 2012

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

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

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, JeanFrancxois 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. Muller, Andrew G. Nicholson, Ganesh
Raghu, Moises Selman, and Athol Wells
Am J Respir Crit Care Med Vol 177. pp 13381347, 2008

- NSIP idiopatic reprezinta o entitate clinica

distincta, ce apare de obicei la femei de varsta
mijlocie si nefumatoare, cu un bun prognostic
pe termen lung

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 comportamentului
clinic
Sunt revizuite tipurile moleculare si genetice intalnite

Va multumesc

Pentru atentie si interes pentru domeniul

bolilor pulmonare interstitiale!