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 Poliartrita
Polimiozita reumatoida
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 Vasculite leucocitoclastice

(fenomen imun mediat celular (complexe imune)
Ac ANCA)
LES, SS, PR, DM
Purpura Henoch-Schonlein
Granulomatoza Wegener

Arterita temporala + Limfocite

Sd. Churg Strauss
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 Diagnostic +
• Infiltrate difuze
• Revarsate pulmonare
• Noduli pulmonari
Biopsia cutanata
CT
Arteriografie Biopsie
transbronsica
Gazometria sanguina toracotomia exploratorie
– 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