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

Case Report

A 22-year-old Saudi Arabian male patient, not known to take medication or have any chronic medical diseases, was admitted to our hospital. For 12 days, he exhibited acute pleuritic chest pain, exertional dyspnea, and hemoptysis associated with subjective fever and sweating. No lower limb pain or swelling was observed. The patient had a history of recurrent painful oral ulcers (one to four small ulcers) over 3–5 days for 4 months, which spontaneously disappeared. The patient also exhibited frequent recurrent painful genital ulcers, mainly on the scrotum. Although the patient was not on a specific diet, he experienced significant weight loss of nearly 8 kg (13% of total body weight) over 6 months. No significant family history of rheumatological or autoimmune diseases, or similar conditions was noted. He was single, lived on a farm, and frequently ingested raw milk.

During examination, the patient was fully conscious and was neither disoriented nor in distress. The patient's blood pressure was 126/70 mmHg, with heart rate of 110 beats per minute, respiratory rate of 20 per minute, oxygen saturation of 95% on ambient air, temperature of 37.4 °C, and body mass index of 19 kg/m2. The patient's mouth exhibited three small, rounded tender ulcers on the oral mucosa and at the base of the tongue. The genitals exhibited one minor tender nonpurulent ulcer on the scrotum.

The patient's laboratory data were as follows: white blood count of 13.7 × 109/L (neutrophils 68%, lymphocytes 25%), hemoglobin level of 11.8 mg/dL, platelet count of 337 × 109/L, coagulation profile normal (INR 1.1), D-dimer of 0.04 mg/L, erythrocyte sedimentation rate (ESR) of 78 mm/hour, and C-reactive protein (CRP) of 10.9 positive. Kidney function tests, liver function tests, and electrolytes were within the normal range. Screening tests for antinuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA), anti-cardiolipin, hepatitis B virus (HBV), hepatitis C virus (HCV), Brucella stiter and culture, and human immunodeficiency virus (HIV) were negative. The tuberculin skin test was 8 mm. Screens using three samples of sputum for acid-fast bacilli, Tuberculosis (TB) polymerase chain reaction (PCR), and GeneXpert were negative. Electrocardiograph (ECG) detected sinus tachycardia. Chest X-ray and transthoracic echocardiography (TTE) results were unremarkable. Fundus examination found bilateral vitreous degeneration and acute posterior uveitis. Abdominal ultrasound with Doppler showed the homogeneous echo pattern of a normal liver with no dilatation of the intrahepatic biliary radicles. The patient exhibited a normal portal vein (PV) and common bile duct (CBD) diameter. The spleen showed a bulky enlarged shape with span of around 13 cm and a homogeneous echo pattern. There was normal patency and color of Doppler signal flow of the portal, superior mesenteric, and splenic veins, with no evidence of thrombosis. Vasculitis was detected via fundus fluorescein angiography (FFA). CT angiography showed filing defects on the right interlobar and lower lobar segmental arteries. Lateral crescent filling defects were seen, denoting partial thrombosis. A right apical segment solitary nodule with size of 2.7 mm was also detected. Pulmonary artery aneurysms were ruled out (Figure 1).

Figure 1.

Spiral chest CT scan angiography

Pulmonary perfusion scan (V/Q scan) showed multiple pulmonary thrombi in the right lung middle lobe, upper lobe anterior segment, and lower anterior basal lobe, while laterobasal and posterobasal segments exhibited perfusion defects (Figure 2).

Figure 2.

Axial and reconstructed views of SPECT-CT slices show multiple right lung middle lobe (medial and lateral segmental), upper lobe anterior segment, lower lobe anterobasal, laterobasal, and posterobasal segment perfusion defects (arrow heads)

After ruling out common infectious diseases including tuberculosis and brucellosis, the patient was diagnosed with BS complicated by PAT based on clinical and radiological findings. Spiral CT ruled out pulmonary artery aneurysm, which is as a common vascular complication. The patient received pulse intravenous steroid therapy with methylprednisolone (1 g for 3 days) and a subcutaneous dose of enoxaparin (1 mg/kg twice per day). The patient's clinical condition started to improve. The patient was discharged on prednisolone (tapering dose of 50 mg/day), colchicine (0.5 mg two times per day), adalimumab SubQ (40 mg every other week), and apixaban (5 mg twice per day). The patient was also to be reevaluated in rheumatology, pulmonology, and ophthalmology clinics on a monthly basis. The patient was in complete remission at 9-month follow-up. ESR and CRP were repeated after 3 months and showed normal ranges. Spiral CT was performed 9 months later. There was no evidence of aneurysms, intracardiac thrombus, or new pulmonary thrombi. Patient was screened for thrombophilia including anti-thrombin III, protein C, protein S, lupus anticoagulant, and anti-cardiolipin antibody-Immunoglobulin-G (Ig-G), and all were within normal. No complications were observed during follow-up.