Pulmonary artery thrombosis as the first presentation of Behçet’s syndrome: a case report and review of the literature

Abstract

Background Behçet’s syndrome is a type of systemic chronic vasculitis of unknown etiology, frequently characterized by recurrent oral and genital ulcers and uveitis. It is less commonly characterized by arthritis and skin, vascular, and gastrointestinal involvements. Behçet’s syndrome affects various sizes of vessels by perivascular infiltration and vasculitis. Unlike other classic types of vasculitis, Behçet’s syndrome patients can present with both arterial and venous involvement. Although vascular Behçet’s syndrome is found in only around 15% of Behçet’s syndrome patients, it is the major cause of morbidity and mortality among them. Furthermore, although deep venous thrombosis has high incidence in Behçet’s syndrome patients, pulmonary artery thrombosis is an uncommon complication. Combining the findings of this and previous case reports of pulmonary artery thrombosis in Behçet’s syndrome patients, we sought to determine the best treatment options for pulmonary artery thrombosis in Behçet’s syndrome patients. Case presentation We present the case of a 22-year-old Arabian male who was admitted to an emergency department with acute chest pain, dyspnea, and hemoptysis for 2 weeks. He gave a long history of recurrent oral and genital ulcers for the last 4 months but without seeking medical advice. Spiral computed tomography showed arterial filling defects with a pulmonary nodule for which the presence of a pulmonary artery aneurysm ruled out. The lung perfusion scan showed multiple pulmonary perfusion defects. After excluding common infectious diseases such as tuberculosis and brucellosis, a diagnosis of Behçet’s syndrome with pulmonary artery thrombosis was made. Steroids with enoxaparin were initiated. The patient was discharged later on prednisolone (tapering dose) with adalimumab and apixaban. He was on regular follow-up for the next 9 months. Conclusions Vascular involvement in Behçet’s syndrome is a major contributor to morbidity and mortality of Behçet’s syndrome patients. Consequently, early detection of vascular involvement has a major impact on the prognosis of patients with Behçet’s syndrome.

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

“…These have similar clinical features to those with PAAs, although massive hemoptysis is observed less frequently in patients with isolated PAT [ 50 , 51 ]. In the case of isolated PAT, CXR could be normal; thus CT scanning is an optimal noninvasive imaging modality for initial and follow-up evaluations [ 35 , 52 , 53 ]. In situ thrombosis is caused by various thrombophilic conditions related to BD, with the first being factor V Leiden deficiency, which makes it difficult to choose anticoagulant therapy, as it may increase the risk of hemoptysis [ 54 ].…”

A Case Report of Behcet's Disease With Thromboses in the Superior Vena Cava and External Iliac Vein

“…Venous system abnormalities are significantly more common than those of the arterial system. Behcet's disease is known to involve the major vessels and cause DVT (deep venous thrombosis) in many patients [10].…”

Case Report and Literature Review: Behçet’s Disease With a Novel TFPI Gene Mutation

“…The mechanism underlying vascular involvement in BD is not clear. Despite the fact that vascular syndrome is manifest in only approximately 15% patients, it contributes greatly to the morbidity and mortality of BD ( 16 ). Most BD patients with vascular involvement experience the first vascular event within 5 years since onset of the disease, which is earlier than fulfilling the diagnostic criteria in 10.8% of these patients ( 8 ).…”