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 (BS) is a chronic systemic form of relapsing remitting vasculitis. Vasculitis involves multiple organs, including the skin, joints, eye, mucosa, veins, arteries, nervous and gastrointestinal systems, and others. BS is usually diagnosed between the third and fourth decade of life, being slightly more common in males compared with females, and presents with more serious features in young adult males.[1,2] BS is more frequent in the Middle East, the Mediterranean, and the Far East regions. Prevalence of BS varies by geographical area; Turkey has had the highest number of reported cases at 420 cases per 100,000 persons, followed by northern China, Iran, and Korea. In contrast, the prevalence in Saudi Arabia is around 20 cases per 100,000 people.[3] The classic clinical presentation of BS is painful oral aphthous ulcers, genital ulcers, and ocular involvement, symptoms that together are called the triple-symptom complex. However, other symptoms may be initially involved as well. After ruling out other differential diagnoses, diagnosis of BS is based on diagnostic clinical criteria as determined by the International Study Group (ISG).[1,4] BS patients need physicians from different subspecialties to care for their acute and chronic health issues.

Vascular involvement in BS patients has major impacts on their quality of life. Vascular Behçet's syndrome (VBS) is unique in that it affects both the venous and arterial systems. The frequency of vascular complications in Behçet's syndrome is around 12–15%,[5,6] being more common in young males. Most VBS patients (~ 75%) experience their first vascular event within 5 years of disease onset, 10.8% of whom present a vascular event before fulfilling the ISG criteria. In a previous study, most patients (87%) who presented initial vascular involvement exhibited deep venous thrombosis (DVT), while only 1 out of 882 patients had pulmonary artery involvement.[5] Venous thrombosis in BS can severely impact patient health, increasing the risk of developing Budd–Chiari syndrome and resulting in a 10% mortality rate in BS patients. Arterial involvement leads to an even higher risk (three times) of mortality in BS patients by increasing the risk of developing severe clinical issues such as arterial aneurysms.[2]

There are several forms of venous thrombosis in BS, including pulmonary artery thrombosis.[5] DVT is the most common venous thrombosis in BS patients. Although incidence of DVT in Behçet's syndrome is high, pulmonary artery thrombosis (PAT) is considered a rare complication. There have been a couple of reported cases of patients with Behçet's syndrome who exhibited pulmonary thrombosis during BS pathogenesis (Table 1). The most common presenting symptom of PAT is hemoptysis.[6] Conventional chest radiography is typically used to initially assess pulmonary symptoms and signs of BS. Spiral computed tomography (CT) is considered an excellent next step as it is a noninvasive procedure.[5] Pulmonary artery thrombosis may accompany aneurysms, which may lead to infarction, atelectasis, and hemorrhage.

The reported frequency of gastrointestinal involvement ranges from 1% to 30%. Symptoms include nausea, abdominal pain, and diarrhea, with complications such as fistulas and bowel perforation.[7] Splenomegaly is not a typical characteristic of BS; only a few case reports have found splenomegaly in BS patients.[8]

We report here in the case of a young male not known to have any chronic diseases who presented with recurrent aphthous ulcers, chest pain, hemoptysis, and splenomegaly. We discuss the challenges of diagnosis and treatment of PAT in Behçet's syndrome. Written consent was secured, and ethical approval by the Institutional Review Board (IRB) at Taif Health Affairs and the Medical Research Unit at King Abdul-Aziz Special Hospital was obtained.