Pulmonary Artery Thrombosis as the First Presentation of Behçet's Syndrome

A Case Report and Review of the Literature

Ziyad Alakkas; Waad Kazi; Mohamed Mattar; Eman Abdul Wahhab Salem; Naglaa Fawzy Seleem


J Med Case Reports. 2021;15(322) 

In This Article


Behçet's syndrome is a type of systemic vasculitis that affects vessels of various sizes and multiple organ types. Vascular complications have a major impact on the morbidity and mortality of BS patients. The prevalence of vascular involvement in BS varies by country, where the highest rates have been reported in Morocco (62.3%)[9] but lower rates in Japan (7.4–8%).[10] Venous involvement is more common (nearly 30% of patients) than arterial involvement (3–5%).[1] Although isolated deep venous thrombosis is the most common form (67%) of major vascular disease in Behçet's syndrome,[5] PAT is considered a rare complication of Behçet's syndrome. However, PAT is one of the main causes of morbidity and mortality in BS patients.[11,12] Its etiology is mainly related to the disease behavior, including recurrent blood vessel inflammation that strongly adheres to vessels and makes embolization difficult, dissimilar to classic thrombi.[6] The thrombi are usually tightly adhered to the vessel walls and may be confused with embolisms, so it is important to initially image the vascular system with Doppler US and utilize advanced radiological methods such as CT venography (CTV), magnetic resonance angiography (MRA), and magnetic resonance venography (MRV) for appropriate diagnosis. CT angiography yields a lot of information regarding anatomy but has limitations, especially in the early stages of disease pathogenesis.[1]

In this case, a young adult Arabian male who previously had recurrent oral and genital ulcers and did not seek any medical advice was finally admitted to the ED after exhibiting a vascular complication of Behçet's syndrome. Since the patient had a history of fever, weight loss, and hemoptysis, tuberculosis was highly suspected, and the patient was admitted to an isolation room. However, tuberculosis was ruled out. He also had the classic BS manifestations of oral and genital ulcers plus ocular symptoms. Since the patient also presented with acute chest pain, dyspnea, and hemoptysis, vascular involvement of either pulmonary artery aneurysm or pulmonary artery thrombosis was suspected. Spiral CT showed pulmonary artery thrombosis with a pulmonary nodule and a pulmonary artery aneurysm ruled out. Later, V/Q scan confirmed presence of thrombosis. A diagnosis of pulmonary artery thrombosis was made before fulfilling the criteria of BS. Based on pathology behavior, autopsy results, and radiological findings of previous studies, pulmonary artery thrombosis has been a more consistent component of BS than pulmonary thromboembolism.[5] Pulmonary nodules are commonly associated with pulmonary artery involvement in BS patients.[13]

The primary treatment objectives for BS patients exhibiting vascular involvement are to suppress inflammation and prevent further complications as soon as possible. There are no standard guidelines for the management of venous thrombosis in Behçet's syndrome. High doses of steroids, azathioprine, and cyclophosphamide are the main immunosuppressants used to treat pulmonary thrombosis. Treating patients via pulse IV steroid therapy with methylprednisolone followed by oral prednisolone has been common practice. Administration of anti-tumor necrosis factor (TNF) monoclonal antibodies with anticoagulation could be considered for treating patients with refractory venous thrombosis who are at low risk of bleeding. However, pulmonary artery aneurysms should be ruled out before initiation of anticoagulation.[14] Anti-TNF could also be used instead of cyclophosphamide[1]

The cornerstone treatment for PAT in Behçet's syndrome is immunosuppression. However, using anticoagulants in pulmonary thrombosis is controversial, as it may worsen the symptoms,[11] but it is considered necessary in other situations.[15] A previous metaanalysis, which compared three groups utilizing different types of treatment regimens, determined that immunosuppression with anticoagulation in Behçet's syndrome had better outcomes in terms of recurrence of DVT compared with anticoagulation treatment alone. In the same study, there were no significant benefits to treating patients with immunosuppression and anticoagulation as compared with immunosuppression treatment alone.[16,17] Based on two studies, bleeding complications have been reported in 2.4–4.5% of anticoagulated patients.[17] To date, no comprehensive studies have been done to determine the appropriate treatment method for BS patients exhibiting PAT or venous thrombosis, so more studies are needed to cover this information gap.

Splenomegaly is an atypical manifestation of BS;[7,8] the possible causes of splenomegaly, such as infectious agents (e.g., brucellosis or TB) or thrombosis-like Budd–Chiari syndrome, were ruled out in our case.