Determinari pulmonare in boli ale tesutului conjunctiv si

Determinari pulmonare in boli ale tesutului conjunctiv si vasculite Conf. 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 (dg. dif. cu infectia pulmonara) 10% • 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 bronhopulmonara - sd. Caplan : + pneuconioza ( fibro-noduli difuzi) HTA pulmonara

Dermatomiozita si polimiozita ! Manifestare paraneoplazica • Afectare interstitiala pulmonara • HTA pulmonara

Determinari pulmonare in colagenoze LES Afectare Interstitiala (alveolita fibrozanta) Bronsiolita obliteranta + (30%) ± Sclerodermie Desmatomiozita Poliartrita reumatoida +++ (75%) ± ++ ++ (60%) ++ ++ Pneumopatii nodulare Afectare pleurala Hemoragie alveolara Hipertensiune pumonara +++ + (20%) +++ ± +

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 (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 Diagnostic + Rx • Infiltrate difuze • Revarsate pleurale • Noduli pulmonari Biopsia cutanata Biopsie in afectare pulmonara transbronsica toracotomia exploratorie CT Arteriografie 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 • p. ANCA • Biopsie – granulom necrozant

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