Determinari pulmonare in boli ale

tesutului conjunctiv si vasculite

Prof. Dr. Adriana Iliesiu

 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 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 – determinari
pulmonare

• Fibroza pulmonara
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 – lichidul pleural
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 pulmonara

• 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
Definitie: 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

(complexe imune)
(fenomen imun mediat celular
Ac ANCA)
Sd. Goodpasture

Granulomatoza Wegener
LES, SS, PR, DM
Purpura Henoch-Schonlein
Sd. Churg Strauss

Arterita temporala
Arterita Takayasu
 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

Diagnostic +
Rx
• Infiltrate difuze
• Revarsate pleurale Biopsia cutanata
• Noduli pulmonari
Biopsie in afectare pulmonara
CT transbronsica
Arteriografie toracotomia exploratorie
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