Background:Behçet’s disease (BD) or “Silk Road” disease is a rare multisystemic inflammatory disease of unknown etiology.Vascular involvement manifested as thrombosis, arterial aneurysm, and occlusion can carry a high mortality risk. BD can be a diagnostic conundrum with its broad array of clinical presentations.Objectives:Identifying vasculo-Behçet’s disease and its management.Methods:A 25-year-old man born in Malaysia and known for cirrhosis due to idiopathic Budd Chiari syndrome presented to the emergency room with a transient ischemic attack. An inferior vena cava (IVC) occlusive thrombus and a patent foramen ovale (PFO) were discovered. Thrombolysis, angioplasty, PFO closure, and a transjugular intrahepatic portosystemic shunt (TIPS) procedure were performed. The following year, the patient experienced numerous IVC and TIPS-associated thromboses as well as a right atrial thrombus attached to his PFO closure device, all of which were refractory to anticoagulation. A few months later, the patient suffered from an acute right anterior cerebral artery stroke, with no etiology uncovered at the time. It was later determined that the patient had experienced years of recurrent oral and genital aphthae, thereby prompting a strong clinical suspicion of BD. Six months later, after only one appointment at the rheumatology clinic during which he was prescribed colchicine, the patient presented to the hospital with hemoptysis. A computed tomography (CT) pulmonary angiogram revealed a right lower lobar pulmonary arterial aneurysm with a peripheral thrombus, a right bronchial artery dilatation, and pulmonary emboli. The patient declined anticoagulation and was sent home. Two months later, he returned to the hospital, this time with hematemesis. A repeat CT pulmonary angiogram was performed and showed an increasing pulmonary emboli burden and an enlarging aneurysm. A thrombophilia workup was negative.Results:A diagnosis of BD with pulmonary aneurysms was made and treatment was initiated with methylprednisolone pulses and monthly intravenous cyclophosphamide as recommended by the European League Against Rheumatism. A month later, there was radiological evidence of significant improvement in the burden of pulmonary emboli, an interval decrease in the aneurysm’s diameter, and resolution of the right atrial thrombus.Conclusion:BD with vascular involvement or vasculo-Behçet’s disease can affect small, medium, and large vessels of both the venous and arterial vasculatures and is thought to originate from vessel wall inflammation.Thrombi in vasculo-Behçet’s disease are typically quite adherent to the vessel walls and tend not to embolize. In this case, pulmonary arterial thrombosis burden was significantly decreased after immunosuppression alone, favoring a diagnosis of in situ thrombosis rather then thromboembolism. Moreover, pulmonary artery aneurysm, Budd-Chiari syndrome, and vena cava thrombosis, which are quite uncommon and carry the highest mortality risk in vasculo-Behçet’s, were all present in this case. Early recognition can be life-saving as immunosuppression is the first-line therapy rather than anticoagulation, which carries a significant risk of pulmonary hemorrhage in the presence of a pulmonary artery aneurysm.References:[1]Seyahi, E., Behcet’s disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol, 2016. 30(2): p. 279-295.[2]Hamuryudan, V., et al., Pulmonary artery aneurysms in Behcet syndrome. Am J Med, 2004. 117(11): p. 867-70.[3]Kobayashi, M., et al., Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease. Histopathology, 2000. 36(4): p. 362-71.[4]Seyahi, E. and S. Yurdakul, Behcet’s Syndrome and Thrombosis. Mediterr J Hematol Infect Dis, 2011. 3(1): p. e2011026.[5]Hatemi, G., et al., 2018 update of the EULAR recommendations for the management of Behcet’s syndrome. Ann Rheum Dis, 2018. 77(6): p. 808-818Disclosure of Interests:None declared
Pathophysiology of the Behçet's Disease