Determinari pulmonare in boli ale tesutului conjunctiv si vasculite

Boli ale tesutului conjunctiv

Grup de afectiuni cu anumite trasaturi comune: Cauza necunoscuta Afecteaza tesutul conjunctiv din organism ( polimorfism al manifestarilor clinice) Degenerescenta fibrinoida leziunea principala
Pulmonar - afecteaza interstitiul, vasele sau ambele LES, Sclerodermia, Poliartrita reumatoida, Dermato- si Polimiozita

Sclerodermie = scleroza sistemica

= boala cronica sistemica de cauza neprecizata
Piele - fibroza cutanata Fenomen Raynaud
Tract gastrointestinal90% scleroza esofagiana Cord (miocard, pericard) Rinichi HTA secundara (crize hipertensive) si proteinurie

Sclerodermie manifestari pulmonare

Fibroza pulmonara interstitiala

Afectarea vasculara

Sclerodermie manifestari pulmonare

Boala interstitiala pulmonara HTA pulmonara

Pneumonie de aspiratie (reflux gastroesofagian)

Lupus eritematos sistemic (LES)

Manifestari sistemice Musculoscheletale Cutanate Hematologice Neurologice Cardiopulmonare Renale Gastrointestinale Oculare

Lupus eritematos sistemic (LES)

Pleurezie + pericardita Pneumonita lupica (dg. dif. cu infectia pulmonara) Fibroza interstitiala HTA pulmonara 30-50% 10%

5% <5 %

Poliartrita reumatoida
Articulare sinovita articulatii periferice, simetrica

Extra-articulare
Noduli reumatoizi Vasculita reumatoida (cutanat, neurologic) Pleuro-pulmonare Neurologice, oculare Osteoporoza

Poliartrita reumatoida
Pleurita lichidul pleural Fibroza pulmonara Noduli pulmonari (unici sau conglomerati) - Caviteaza pneumotorax sau fistula bronhopulmonara - sd. Caplan : + pneuconioza ( fibro-noduli difuzi) HTA pulmonara

Dermatomiozita si polimiozita
! Manifestare paraneoplazica
Afectare interstitiala pulmonara HTA pulmonara

Determinari pulmonare in colagenoze

LES
Afectare Interstitiala (alveolita fibrozanta) Bronsiolita obliteranta Pneumopatii nodulare Afectare pleurala Hemoragie alveolara Hipertensiune pumonara

Sclerodermie

Desmatomiozita Polimiozita

Poliartrita reumatoida

+ (30%)

+++ (75%)

++

++ (60%)

+++

++

++ + (20%) +++

+ ++

+++

Afectarea pulmonara in vasculite

Definitie: inflamatia si lezarea vaselor determinand sindroame clinice heterogene

Vasculita

primara (singura manifestare a bolii) secundara unei alte boli

( infectie, neoplasm, boli reumatice)

Afecteaza - un singur organ (de ex. pielea) - mai multe organe / sisteme

Afectarea pulmonara in vasculite

Vasculite granulomatoase
(fenomen imun mediat celular Ac ANCA) Granulomatoza Wegener
Sd. Churg Strauss

Vasculite leucocitoclastice
(complexe imune) Sd. Goodpasture Boli ale tesutului conjunctiv (LES, SS, PR, DM)

Purpura Henoch-Schonlein
Arterita temporala Arterita Takayasu

Afectarea pulmonara in vasculite

Inflamatie si reactie imuna (VSH, Ig, Cseric, FR, Ac. ANCA)

Diagnostic +
Rx
Infiltrate difuze Revarsate pleurale Noduli pulmonari

Biopsia cutanata Biopsie in afectare pulmonara transbronsica toracotomia exploratorie

CT Arteriografie Gazometria sanguina hipoxemie cu alcaloza respiratorie

Granulomatoza Wegener
= vasculita granulomatoasa tract respirator inalt (sinusuri si nazo-faringe) (durere, puroi, ulceratii) - pulmonar - infiltrate nodulare ( tuse, dispnee, hemoptizie) + glomerulonefrita

Granulomatoza Wegener
pANCA Biopsie granulom necrozant

Sindromul Churg-Strauss
= vasculita alergica si granulomatoasa
Astma + Eozinofilie + Granuloame extravasculare + Vasculita