PNEUMOPATII INTERSTIIALE NEGRANULOMATOASE

PNEUMOPATI INTERSTIIALE
DE CAUZA CUNOSCUT DE CAUZA NECUNOSCUT

FORME GRANULOM

Pneumoniile

de hipersensibilit Berilioza

Sarcoidoza
Histiocitoza

X Vasculite granulomatoase Granulomatoza bronhocentric Granulomat limfomatoid

FORME NE GRANULOM.

.Asbestoza Medicamente Iradierea Fum,gaze

Fibroza Afect

pulmonar idiopatic
idiopatic interstitial

Pneumonia

pulm in bolile de es conj Proteinoza alveolar Amiloidoza Sindr hemoragipare pulmon

PNEUMONIA IDIOPATIC INTERSTIIAL

Grup de boli difuze parenchimatoase De cauza necunoscut Fibroz Inflamatie Distrugerea arhitecturii pulmonare

PNEUMONIA IDIOPATIC INTERSTITIAL

Dispnee i tuse/scd cap de efortprogresive Fara cauz- obstr/alte fibroze Radiografie cu infiltrate/desen reticulat CT Gazometrie modific fumat

PNEUMONIA IDIOPATIC INTERSTIIAL

tipuri de pneumonie interst idiopatic 1 2 3 4 FIBROZA PULMONAR IDIOPATIC-tipul clasic PNEUMONIA INTERSTIIAL NESPECIFIC PNEUMONIA CRIPTOGENIC-BOOP PNEUMONIA DESCUAMATIV-broniolita asoc bolii interst pulmon

PNEUMONIA INTERSTIIAL ACUT-sindr Hamman-Rich

PNEUMONIA LIMFOID

PNEUMONIA IDIOPATIC INTERSTITIAL

Aduli,B,F Factori de risc -fumatul -infectii virale -imunodepresia Ex clinic/anamnez -normal -dispnee progresiv la efort/ -dispnee sever relativ brusc instal -tuse seac -raluri-crepitaii bazale -febr -hipocratism -semne ale insuficienei resp pna la detres resp -semne ale cordului pulmonar

PNEUMONIA IDIOPATIC INTERSTITIAL

Investigaii Radiografia pulmonara/CT cu rez inalt -opaciti bazale bilaterale -desen reticulat -broniectazii de traciune -asp de fagure -asp de geam mat -asp de consolidare pneumonic -opaciti bilaterale migrat

FIBROZA PULMONAR IDIOPATIC

PN INTERST NESP

PN CRIPT-BOOP

BOOP

BOOP

Consolidri cronice

PNEUMONIA INTERSTIIAL ACUT -sindr Hamman-Rich

FIBROZ PULMONAR IDIOPATIC

Geam mat+fibroz+br-ectazii

Geam mat+fibroza/ingroarea septala

PNEUMONIA IDIOPATIC INTERSTITIAL

Spirometria Gazele sangv DLCO Investigaii biochimice Biopsia transbronic/transtoracic

PNEUMONIA IDIOPATIC INTERSTITIAL

Anatomopatologie -inflamaie min/moderat -depozite de colagen -ngroarea pereilor alveolari -focare de proliferare fibroblastic -es de granulaie n alveole i bronhiole -macrofage intraalveolare -descuamarea es epit bronic de ctre mcf -fibroz masiv,acut,mb hialine -infiltrat limfocitar interst

PNEUMONIA IDIOPATIC INTERSTITIAL

Evoluie,prognostic -rasp diferit la tratam n fc de asp anatomopat - de la detres resp la evol lent spre CP si IR Tratament -Prednison 0,5-1mg/kc +Ciclofosfamid 2mg/kc / +Azatioprin 2mg/kc -tratamentul/ profilaxia infeciilor -transplant pulmonar unic n studiu -pirfenidone-inhib sinteza de colagen -Ifn -1b

AFECTAREA PULMONARA IN BOLILE DE COLAGEN

LUPUS ERITEMATOS SISTEMIC ARTRITA REUMATOID SCLEROZA SISTEMIC POLIMIOZITA/DERMATOMIOZIT

LUPUS ERITEMATOS SISTEMIC

Afectiune sistemica autoimuna care afecteaz F tinere cu evoluie progresiv Pleurit-revrsat pleural Pneumonite lupice BOOP Alveolit hemoragic Afectarea mm resp Afectarea cilor resp sup Hipertensiune pulmonar Boal trombembolic Pneumonii bacteriene

LUPUS

Modificarile fct pulmon sunt frecv in lupus-80% Chiar si in absenta simpt/rx Scd DLCO Scd volumelor pulmonare Modific gazelor sangv-hipoxemie Insoesc afectarea renal

LUPUS
Revarsat lich -cel mai frecv -75%,B,mic,unilateral Afectarea interstitial -fibroz/inflamatie/infiltrate + Ac antiADNdc Pneumonita -debut brusc febra+tuse+IR -infiltrat mononuclear+fibroz+alveolit+vasculit sindr plmnului mic in lupus -Volum pulm scazut -Diafragme ascens -Atelect bazale -Afect muscular/dfg HTP+CP

SINDR PLMNULUI MIC IN LUPUS

ARTRITA REUMATOID

Afectare pleural Pneumonita interstitial Noduli pulmonari Afectarea cilor resp Boala vasc pulmonar Afectarea pulmonar det de mdc

ARTRITA REUMATOID
Afectarea pleural -cea mai frecv -mai ales la B -insoit de noduli reumatoizi -toracenteza Pneumonita interstitial -tuse+dispnee+crepitatii+DLCO+hipoxie+FR Mdc-metotrexat/sruri de aur/infliximab-tbc Noduli pulmonari reumatoizi -frecv,B -asoc cu exacerb articular -necroz fibrinoid+celule epit gigante in palisad -se pot rupe in pleur -sindr Caplan-noduli reumatoizi+artrite simetrice la p cu pneumoconioz Caile aeriene -afect cilor resp mici/BOOP/Broniolit obliterativ

PROTEINOZA ALVEOLAR

PROTEINOZA ALVEOLAR

Rar Depunerea unui material eo in alveole i ci aeriene Defect genetic/ctigat al catabolismului surfactantului-mcf rspunz/ac anti surfactant Tuse+expect+dispnee progresiv Rx-infiltrate nodulare perihilare asemnt cu EPA -asp miliar -multinodular -geam mat Bronhoscopie-lich lptos+corpi eo cu gran paspozitiv Complic-infeciile Tratam-lavaj+de susinere+corticoterap

AFECTAREA PULMONAR DETERMINAT DE MEDICAMENTE

Tuse-IEC Bronhospasm-BETABLOC Dureri toracice-METOTREXAT BOOP-SARURI DE AUR,PENICILAMIN Pneumonii interstitiale-AMIODARONA, NITROFURANTOIN,CHINIOTERAPIA ex BLEOMICINA Hemoragii alveolare-PENICILAMINA, ANTICOAGULAREA Granuloame-FLUOXETIN,TALC HTP-AMFETAMINE,FENFLURAMINA,COCAIN

SARCOIDOZA

HISTIOCITOZA X