PB0759 - Pulmonary artery thrombosis in Behçet's syndrome : a case series

Monday, June 26, 2023

18:30 – 19:30 ET

Room: Exhibition

Co-Author(s)

SC

Sarra CHADLI

Dr.
Internal medicine, Ibn Sina University Hospital Center, Rabat, Morocco
rabat, Rabat-Sale-Zemmour-Zaer, Morocco

Co-Author(s)

HK

Hajar KHIBRI

Prof.Dr.
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
SF

Safaa FARI

Dr.
Internal medicine, Ibn Sina University Hospital Center, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
MB

Meriem BOURKIA

Prof.Dr.
Internal medicine, Mohammed VI University Hospital Center, Rabat, Morocco
Tangier, Tanger-Tetouan, Morocco
NM

Naima MOATASSIM

Dr.
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
WA

Wafaa Ammouri

Prof.Dr.
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
MM

Mouna MAAMAR

Prof.Dr.
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
HH

Hicham HARMOUCHE

Prof.Dr
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco
MA

Mohammed ADNAOUI

Prof.Dr.
Internal medicine, Ibn Sina University Hospital Center, University Mohammed V, Rabat, Morocco
Rabat, Rabat-Sale-Zemmour-Zaer, Morocco

Presenting Author(s)

ZOUBIDA TAZI MEZALEK, MD (she/her/hers)

Professeur
Internal medicine - Clinical Hematology. Faculty of medicine.University Mohamed V. Rabat.Morocco
RABAT, Rabat-Sale-Zemmour-Zaer, Morocco

Background: Behçet's syndrome (BS) is a complex multisystemic vasculitis that affects vessels of all sizes and kinds, with a tendency toward the venous system and thrombosis. Arterial disease is rare and manifests primarily in the form of pulmonary arteries aneurysms (PAA) and thrombosis (PAT).

Aims: Our work aims to depict the profile and outcomes of BS patients with PAT.

Methods: We retrospectively conducted a descriptive and monocentric study between 2000 and 2022, including 572 patients diagnosed with BS.

Results: 16 cases were enrolled (2.8%). The mean age was 34 ± 6 years (19-58), and the sex ratio (M/F) was 4.7. Cardiovascular risk factors included smoking (12%) and high blood pressure (6%). PAT occurred after a median of 4.5 years following BS onset. Patients were asymptomatic (69%), or presented with dyspnea (35%), hemoptysis (31%), and thoracic pain (25%). On chest CTA, PAT was bilateral (78%) and proximal (46%). Concomitant PAA was observed (25%), while peripheral aneurysms were not reported. Deep venous thrombosis was found in the lower extremities (19%), superior (27%), and inferior vena cava (6%). Cardiac involvement was simultaneously observed (40%), mostly right intracardiac thrombosis (37%). Noncardiovascular involvements were mucocutaneous (87%), ocular (26%), articular (25%), and digestive (6%). Inflammatory parameters were often raised (80%). Patients were treated with colchicine (97%), glucocorticoids (93%), cyclophosphamide (47%), azathioprine (47%), and a TNF-a-inhibitor (6%). Low molecular weight heparin followed by oral antivitamin K was received (60%). No oral direct anticoagulant was prescribed. No surgery or endovascular procedure was performed. Relapse and death were respectively recorded in 15% and 26% of the cases.

Conclusion(s): PAT is a particular feature of BS that is, unlike common thrombotic diseases, due to vasculitis rather than hypercoagulability. It should be quickly evoked in young men presenting with simultaneous mucocutaneous and thrombotic lesions, as prompt immunosuppressive therapy is crucial for patients' survival.