CONGRESS ABSTRACT

A 76-year-old man comes to our attention transported by 118 for an episode of a fall to the ground with loss of consciousness, undergoing cardiac massage and defibrillation.  The ECG shows extensive signs of necrosis in the anterior region. The echocardiogram shows a left ventricle with a large apical aneurysm, a severely depressed global systolic function (EF 25%). The coronary angiography study is then performed, which highlights the chronically occluded anterior descending artery in the middle section and tributary of aneurysmal territory. The remote pathological anamnesis includes Chron's disease, a previous anterior uveitis of the left eye with loss of vision, recurrent phlebitis in the lower limbs, a previous right femoro-popliteal bypass, a previous anterior STEMI treated with primary PTCA and implantation of medicated stents on the anterior descending branch. The patient reported also recurrent episodes of lesions of the buccal mucosa since childhood and recurrent episodes of facial Herpes in the frontal area; therefore, in light of the overall symptomatic picture, after a collegial evaluation with internist colleagues, a diagnosis of Behçet's Syndrome was made. Also known as "Silk Road Disease" due to its higher prevalence in the Middle East and Central Asia, it is a recurrent, multi-symptomatic, chronic systemic vasculitis with multi-organ involvement. Its etiology remains unknown but it is believed to be an autoimmune disease. Behçet's Syndrome cannot be diagnosed either by pathognomonic symptoms or by laboratory tests. In order to formulate the diagnosis, based on the international classification criteria, defined starting from the clinical picture, the presence of recurrent oral aphthae is mandatory, at least 3 times in the span of 12 months, in combination with two of the following signs: recurrent genital ulceration, ocular lesions, skin lesions or pathergy. Other disease characteristics that may be important for the diagnosis of individual cases are: superficial thrombophlebitis, deep vein thrombosis, arterial occlusions and/or aneurysms, involvement of the central nervous system, arthritis, involvement of the gastrointestinal tract, family history. In light of the recent diagnosis, cortisone therapy was started and our patient, after undergoing ICD implantation for secondary prevention, was discharged with a referral for specialist rheumatology evaluation for therapeutic optimization and subsequent follow-up.