cursrespiratorcolagenoze

Determinari pulmonare in colagenoze si vasculite

Conf. Dr. Adriana Iliesiu

Colagenoze

• Grup de afectiuni cu anumite trasaturi comune:

– Cauza necunoscuta

– Afecteaza tesutul conjunctiv din organism

( polimorfism al manifestarilor clinice)

– Degenerescenta fibrinoida – leziunea principala

Pulmonar - afecteaza mezenchimul, 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 gastrointestinal-

90% scleroza esofagiana

• Cord –(miocard, pericard)

• Rinichi – HTA (crize hipertensive) si proteinurie

Sclerodermie – manifestari pulmonare

• Fibroza interstitiala

• HTA pulmonara

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 30-50%

• Pneumonita lupica 10%

(dg. dif. cu infectia pulmonara)

• Fibroza interstitiala 5%

• HTA pulmonara <5 %

Poliartrita reumatoida

• Articulare – sinovita articulatii periferice, simetrica

• Extra-articulare– Noduli reumatoizi

– Vasculita reumatoida (cutanat, neurologic)

– Pleuro-pulmonare

– Neurologice, oculare

– Osteoporoza

Poliartrita reumatoida

Pleurita – lichidului pleural (glucoza, complement)

Fibroza pulmonara

Noduli pulmonari (unici sau conglomerati)

- Caviteaza – pneumotorax sau fistula bronho-pulmonara

- sd. Caplan : + pneuconioza ( fibro-noduli difuzi)

HTA pulmonara

Dermatomiozita si polimiozita

! Manifestare paraneoplazica

• Afectare interstitiala

• HTA pulmonara

Determinari pulmonare in colagenoze

LES Sclerodermie Desmatomiozita Polimiozita

Poliartritareumatoida

Afectare Interstitiala (alveolita fibrozanta)

+ (30%)

+++ (75%)

++ ++(60%)

Bronsiolita obliteranta ± ± ++ ++Pneumopatii nodulare + (20%)Afectare pleurala +++ +++Hemoragie alveolara + ± ±Hipertensiune pumonara

++ +++ +

Afectarea pulmonara in vasculite

= inflamatia si lezarea vaselor cu 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)

LES, SS, PR, DM

Purpura Henoch-Schonlein

Arterita temporala + Limfocite

Arterita Takayasu T

Vasculite – manifestari clinice

Semne generale

febra, stare generala, artralgii, leziuni cutanate

Pulmonar

Dispneea, tusea, sinuzita, epistaxis, astm bronsic

Manifestari sistemice

Ulcere cutanate, eruptii purpurice, vezicule

Iridociclita, ulceratiile orale si genitale

Afectarea pulmonara in vasculite

VSH, Ig, C´seric, FR, ac. ANCA

Rx• Infiltrate difuze

• Revarsate pulmonare

• Noduli pulmonari

CT

Arteriografie

Gazometria sanguina

– hipoxemie cu alcaloza respiratorie

Diagnostic +

Biopsia cutanata

Biopsie

transbronsica

toracotomia exploratorie

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