Superior Vena Cava Syndrome secondary to Migratory Thrombophlebitis: A Rare Complication of Behçet’s Disease

Behçet’s disease (BD) is a chronic, systemic vasculitis characterized by a triad of recurrent aphthous ulcers (mouth sores), genital ulcers, and ocular symptoms [1]. While these are the cornerstone manifestations, BD can affect various organ systems. A rare but potentially life-threatening complication of BD is superior vena cava (SVC) syndrome [2]. We present a case report of a young patient with established BD who developed migratory thrombophlebitis, involving multiple veins, leading to SVC syndrome as a rare but serious complication. We discuss the clinical picture, diagnostic workup, management strategies employed, and the importance of considering SVC syndrome in the differential diagnosis of patients with BD, particularly those presenting with atypical features.

An 18-year-old school boy with no significant medical history presented to the clinic with a 1-week history of fever, and bilateral facial and neck swelling. He was a lifetime non-smoker and a nonalcoholic. He had no personal or family history of coagulopathies or cancer. There was no history of surgical or medical interventions to his neck or limbs. On physical examination, his vitals were stable. He had diffused facial and bilateral neck swelling, and several distended veins in the neck and upper chest region. Considering the results of the coagulation studies and the age of the patient, a case of SVC due to a hypercoagulable state was suspected. Anticoagulation therapy was started at that time, which led to just temporary relief, before a relapse. Two years later, he had recurrence of the same symptoms, but this time associated with recurrent genital ulcers, arthralgias, bilateral lower extremity weakness, areflexia, anesthesia, and urinary incontinence (Figure 1). On diving deep, it was disclosed that the patient has had recurrent ulcers for the past four years and were passed on for benign aphthous ulcers. They were only highlighted when the patient had already developed nervous system involvement. A possible diagnosis of SVC syndrome secondary to BD was considered.

Investigations

Laboratory data revealed normal hemoglobin of 14.91 g/ dL, leukocytosis at 19,860/mm3 predominantly neutrophils (83%) with an absolute neutrophil count of 16.48 x 109. He had a prolonged Prothrombin time (PT) of 23 sec with an International normalized ratio (INR) of 1.8. He also had low Protein S at 68% and weakly positive Lupus anticoagulant antibodies with an LA1/LA2 ratio of 1.4. Antinuclear antibody, immunofluorescent antibody, and anti-dsDNA were negative. The patient had a high C reactive protein (CRP), a high sensitivity of 164.24 mg/dL, and an elevated erythrocyte sedimentation rate (ESR) of 105 mm/h. Based on these findings, possible thrombosis due to a hypercoagulable state was presumed. Further investigations including Doppler ultrasonography revealed thrombosis of the right internal jugular vein (Figure 2). Computed Tomography Angiography (CTA) of the chest and neck showed right internal jugular vein thrombosis (Figure 3), extending down to involve the brachiocephalic vein and SVC (Figures 4&5). Regression of thrombus on CTA in association with vasculitis established the diagnosis of migratory thrombophlebitis.

Differential Diagnosis

Clinical presentation of SVC obstruction along with the abnormal coagulation studies led to the initial assumption of SVC syndrome due to a hypercoagulable state. However, based on the clinical picture and laboratory values, SVC syndrome from migratory thrombophlebitis due to BD was made.

Treatment

The choice of treatment was based on immunosuppression therapy to treat BD. Treatment included high-dose corticosteroids, including a 5-day pulse of methylprednisolone (1000 mg/day), and continuous prednisone 60 mg/day along with azathioprine (1000 mg/month). For SVC, anticoagulation therapy was sustained for 6 months.

Outcome and Follow-Up

The patient had an uneventful recovery after the initiation of therapy; the edema of the neck and face resolved, and the labs gradually trended down. Imaging showed a clearing of veins. The patient made a smooth transition to regular life.

Behcet’s disease (BD) is a multisystem systemic inflammatory condition having a relapsing and remitting course. It mainly presents with mucocutaneous, ocular, and GI symptoms. However, the involvement of skin, urogenital, neurological, and vascular systems can also occur [3]. Vascular involvement in BD is rare. A respective study including 5970 patients revealed vascular involvement in 14.7% of cases [4]. Venous involvement is way more common than the arterial system, with literature showing so in up to 10-25% of cases [5]. Deep vein thrombosis (DVT) is the most common vascular presentation in patients with BD, present in 75% of the cases. It most commonly affects the femoral and the popliteal veins [6]. However, our patient hadn’t had DVTs. Vena cava thrombosis is the second most common venous malformation in patients with BD. Around 15% of the patients with BD develop vena cava thrombosis, leading to SVC syndrome [6]. The SVC is a major vein responsible for draining blood from the head, neck, upper extremities, and chest into the heart. Obstruction of the SVC presents with a constellation of symptoms due to impaired venous drainage. These symptoms can include facial and neck swelling, headache, dizziness, upper body plethora (redness), and dyspnea (difficulty breathing) [7].

The most common causes of SVC syndrome include malignancies (lymphoma, lung cancers) [8], and venous catheterization [9]. However, our patient had no history of cancer or venous catheterization. Treatment of vascular thrombosis in patients with BD is not well understood. Treatment is targeted to improve the condition of the patient and prevent relapses. Immunomodulation, along with oral glucocorticoids are the firstline agents [10]. In many cases, anticoagulation therapy is also considered. Using anticoagulation therapy to resolve the clot in patients with vascular BD is a debatable subject among physicians. Several studies state that using Heparin or Warfarin can cause serious bleeding and immense inflammation in some of these patients [11,12]. In the modern era, there is increasing evidence for the role of biologics in the treatment of BD. Drugs like canakinumab, tocilizumab, gevokizumab, and anakinra are used not only to cure the neurological manifestations of BD but have also shown benefits in vascular lesions as well [13].

Superior vena cava thrombosis may be seen because of thrombophlebitis during Behcet’s disease. Although Behcet’s disease is rarely considered during the investigation of SVC syndrome, it should be regarded during the search for the etiology of venous thrombosis in young and middle-aged patients without a history of cancer or coagulopathy.