cursrespiratorcolagenoze
TRANSCRIPT
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