P205 Pulmonary artery aneurysms in Behçet's syndrome: a case series of 51 patients
Authors: Chadli Sarra , Khibri Hajar , Firdaous Safaa Fari , Moatassim Naima , Ammouri Wafaa , Maamar Mouna , Harmouche Hicham , Adnaoui Mohammed , Mezalek Zoubida Tazi 
Published: April 2023

Background/Aims

Behçet’s syndrome (BS) is a multi-systemic vasculitis highly prevalent in a broad range stretching from the Mediterranean region to eastern Asia. The vascular involvement, present in up to 40% of the cases, is characterised by a high tendency towards the venous system and thrombosis. Although pulmonary artery aneurysms (PAA) are considered to be rare (less than 5% in cohorts), they are a quasi-pathognomonic sign of BS and play a major role in patient’s morbidity and long-term mortality. Our work aims to depict the clinical profile, imaging features, therapeutic management, and outcomes of BS patients presenting with PAA.

Methods

We conducted a retrospective, descriptive and monocentric study including 531 BS patients admitted to the internal medicine department between 2000 and 2021. All patients fulfilled the International Study Group Criteria for Behçet’s disease. We enrolled all cases with documented PAA on CT angiography of the chest (CTA chest).

Results

Among 531 BS patients, 51 had PAA (10%). The mean age was 36 ± 11 [20-69] and the sex ratio (M:F) was 4:1. Cardiovascular risk factors comprised chronic tobacco consumption (13.5%) and high blood pressure (9.6%). PAA occurred after a median of 5.5 years following the disease onset. Patients were either asymptomatic (78%) or presented with hemoptysis (6%), dry cough (6%), chest pain (4%), and dyspnea (2%). On CTA chest, PAA were commonly multiple (78%), proximal (46%), and bilateral (29%). Concomitant arterial manifestations were observed in 25% of the patients, consisting of peripheral arteries aneurysms (17%), pulmonary artery thrombosis (7.7%), cerebral artery thrombosis (4%), and aortic aneurysms (2%). Venous thrombosis was also simultaneously found in 21% of the cases, observed in the superior (9.6%) and inferior vena cava (9.6%), cerebral veins (7.7%), and lower extremity veins (6%). On echocardiography, right intracardiac thrombosis was visualised (17%). The other extravascular involvements were mainly mucocutaneous (73%), ocular (46%), articular (29%), and neurological (10%). Our patients were treated with colchicine (100%) and an initial pulse of methylprednisolone (15mg/kg/day for three days), followed by oral prednisolone (1 mg/kg/day), gradually tapered (94%). The immunosuppressive therapy administered consisted of cyclophosphamide (38.5%), azathioprine (31%), and infliximab, a TNF alpha inhibitor (2%). Curative anticoagulation was prescribed in 21% of the cases. Only one patient underwent surgery. Relapse and death were recorded in respectively 15% and 6% of the cases.

Conclusion

PAA are a very unique feature of BS, with specific characteristics related to their inflammatory pathogenesis. We highly recommend evoking the diagnosis of BD in presence of PAA, especially in young male patients presenting with other vascular lesions and intracardiac thrombosis. The prompt initiation of anti-inflammatory and immunosuppressive therapy is of paramount importance to reduce the risk of massive hemorrhage from PAA rupture.

Disclosure

S. Chadli: None. H. Khibri: None. S. Fari Firdaous: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.