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 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
• Boala interstitiala pulmonara
• HTA pulmonara
• Pneumonie de aspiratie (reflux gastroesofagian)

Lupus eritematos sistemic (LES)

• Manifestari sistemice
• Musculoscheletale
• Cutanate
• Hematologice
• Neurologice
• Cardiopulmonare
• Renale
• Gastrointestinale
• Oculare

• Pleurezie + pericardita 30-50%

• Pneumonita lupica 10%
(dg. dif. cu infectia pulmonara)
• Fibroza interstitiala 5%
• HTA pulmonara <5 %

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Poliartrita reumatoida
• Articulare – sinovita articulatii periferice, simetrica
• Extra-articulare
– Noduli reumatoizi
– Vasculita reumatoida (cutanat, neurologic)
– Pleuro-pulmonare
– Neurologice, oculare
– Osteoporoza
• 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 + (30%) +++ ++ ++

(alveolita fibrozanta) (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

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

Vasculite leucocitoclastice (complexe imune)

Sd. Goodpasture
LES, SS, PR, DM
Purpura Henoch-Schonlein
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
Rx
• Infiltrate difuze
• Revarsate pleurale
• Noduli pulmonari
CT
Arteriografie
Gazometria sanguina – hipoxemie cu alcaloza respiratorie

Diagnostic +
Biopsia cutanata
Biopsie in afectare pulmonara
transbronsica
toracotomia exploratorie

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

• pANCA
• Biopsie – granulom necrozant

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