A strange cause of coronary artery aneurism: Behcet disease

Clinical History

24-year-old male patient complaining of costal pain, haemoptoic sputum and fever. Laboratory data included microcytic anaemia and APR elevation. A CT showed an endocavitary mass affecting right ventricle and pulmonary trunk, a thrombus confirmed by surgery. 3 years later he complained of oral and genital aphthas, shortness of breath and thoracic oppression.

Imaging Findings

Given the history of thrombus, a CT was performed in the emergency department to rule out a pulmonary embolism.
The contrast enhanced MDCT pulmonary angiography demonstrated a filling defect in the right cardiac cavities suggesting a thrombus adjacent to the AV valve.

An MRI was performed confirming the previous findings.

The patient persisted with thoracic oppression, therefore a conventional coronary angiography was performed showing an important chronic coronary disease affecting right coronary and the anterior descending artery and suggesting a right coronary aneurysm.
He underwent a coronary CT angiography demonstrating a right coronary artery aneurysm located on the right AV sulcus, 60x55x45 mm long, with mural thrombus and a permeable lumen of 22 mm. Distal to the aneurysm, the RCA was occluded with distal revascularisation via left phrenic artery.

Discussion

Behcet disease is a rare multisystemic chronic inflammatory disease of unknown cause. The clinical triad consists of recurrent oral and genital ulcers and ocular involvement. Other manifestations are skin lesions, vasculitis, CNS and lung affection [1].

Vascular complications develop in about 20-40 % of patients with Behcet [2]. The underlying pathologic process is vasculitis and perivascular inflammatory infiltrates affecting vessels of various sizes. The major cause of mortality in these patients is the vascular involvement, the venous system being more affected than the arteries (85% vs 15%). Nonetheless the arterial involvement confers a worse prognosis and a higher mortality. The arterial affection includes aneurysms and occlusion, mostly involving the abdominal aorta and its branches, but also the thoracic aorta, in fact, Behcet is the most common cause of pulmonary artery aneurysm. These aneurysms may accompany thrombosis of the pulmonary arteries and occlusions.

The initial assessment of thoracic involvement is a chest radiography, which can also be used for follow up and therapeutic response.
MDCT is a non invasive examination, which allows the assessment of thoracic manifestations, with a high spatial and temporal resolution. It can be performed as an alternative to venography and angiography, examinations that should be avoided because of their increased risk for aneurysm formation and venous thrombosis, aside from that, completely thrombosed aneurysms may not be detected at angiography [2].

Systemic corticosteroids are required for disease exacerbation, and sometimes adding some other form of immunosuppressive treatment is necessary when serious end organ involvement occurs. Steroid therapy may sometimes be effective for treating unruptured aneurysms, as these may show some regression, however, aneurysms often respond poorly to medical treatment [3]. Surgery is the first choice treatment for ruptured aneurysm.

It is very important to diagnose the vascular involvement because of its relevance for the patient’s prognosis and its high mortality. The rupture of an aneurysm in these patients is a cause of sudden death. In these patients prognosis depends on vascular involvement, arterial affection being more serious but less common than venous affection.

Differential Diagnosis List

Final Diagnosis

Coronary artery aneurysm in patient with Behcet Disease

URL:	https://www.eurorad.org/case/10676
DOI:	10.1594/EURORAD/CASE.10676
ISSN:	1563-4086