Morbus Adamantiades-Behçet (MAB) is an inflammatory disease typically manifesting with oral and genital aphthosis, erythema nodosum, and vasculopathy, and in only around 2%, cardiac involvement. Its prevalence is usually higher along the historic Silk Road, but rarer in Scandinavia where 0.64-4.9 in 100,000 people are affected. We herein present two Swedish patients with cardiac manifestations of Morbus Adamantiades-Behçet. Along with the intracardial thrombi, which both patients presented with, one patient also had cerebrovascular insults leading to visual field deficits as well as involvement of peripheral nerves. Being of Scandinavian origin and showing uncommon symptoms as their initial manifestations of MAB, the 62- and 35-year-old patients presenting herein constitute rare cases.
Keywords: Morbus Adamantiades–Behçet; autoinflammation; intracardiac thrombus; rheumatology.