Ghembaza A, Boussouar S, Saadoun D
Sorbonne Université, Pitié-Salpêtrière University Hospital, Paris, France; Department of Internal Medicine and Clinical Immunology, Paris, France; Centre de référence des maladies auto-immunes systémiques rares, centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire, AP-HP, 75013 Paris, France; Institut national de la santé et de la recherche médicale, Paris, France; Inserm, UMR_S 959, 75013, Paris, France; RHU IMAP, CNRS, FRE3632, 75005 Paris, France.
Cardiovascular and Thoracic Imaging Unit, hôpital Pitié Salpêtrière, AP-HP, Paris, France; Inserm, laboratoire d'imagerie biomédicale, CNRS, Institute of Cardiometabolism and Nutrition, Sorbonne Université, Paris, France.
Rev Mal Respir. 2022 Jun;39(6):523-533. doi: 10.1016/j.rmr.2022.04.010. Epub 2022 May 31.
Behcet's disease (BD) is a multisystemic vasculitis involving arteries and veins of all sizes. While joint and dermatological manifestations are the most common features of BD and are associated with a good prognosis; vascular involvement, remains the principal cause of death. Arterial manifestations occur in 5-10% of cases and manifest as occlusion/thrombosis or aneurysms. Arterial aneurysms are likely multiple and the most common sites are pulmonary arteries, aorta and arteries of lower limbs. Parenchymal involvement is less frequent and may manifest as consolidation or nodules, which may evolve to excavation. Aneurysms may occur at the sites of arterial puncture; then, non-traumatic techniques are favored. Patients with arterial manifestations may present with fever and increased inflammatory markers. Artery damage is rare, serious, and may result in massive hemoptysis. The prognosis of pulmonary artery aneurysms is severe (mortality estimated up to 26%) but has been improved by earlier diagnosis and the introduction of immunosuppressants. Treatment of severe arterial manifestations is based on high-dose corticosteroids along with cyclophosphamide or anti-TNF antagonists. Anticoagulation could be added to immunosuppressants in case of venous thrombosis if a coexisting pulmonary aneurysm is ruled out. Endovascular treatment should be performed in case of severe symptomatic pulmonary aneurysms, along with an adequate medical management. Long-term maintenance therapy of these severe forms is of paramount importance because of relapse risk (40% at five years).
白塞病（BD）是一种累及各种大小动静脉的多系统血管炎。虽然关节和皮肤表现是BD最常见的特征，且预后良好；但血管受累仍是主要死因。动脉表现见于5% - 10%的病例，表现为闭塞/血栓形成或动脉瘤。动脉动脉瘤可能为多发，最常见的部位是肺动脉、主动脉和下肢动脉。实质受累较少见，可表现为实变或结节，可发展为空洞。动脉瘤可发生在动脉穿刺部位；因此，倾向于采用非创伤性技术。有动脉表现的患者可能出现发热和炎症指标升高。动脉损伤罕见但严重，可能导致大量咯血。肺动脉瘤的预后严重（估计死亡率高达26%），但早期诊断和引入免疫抑制剂已改善了预后。严重动脉表现的治疗基于高剂量糖皮质激素联合环磷酰胺或抗TNF拮抗剂。如果排除并存的肺动脉瘤，在静脉血栓形成时可在免疫抑制剂基础上加用抗凝治疗。对于严重有症状的肺动脉瘤，应进行血管内治疗并辅以适当的药物治疗。由于复发风险（五年时为40%），这些严重类型的长期维持治疗至关重要。
