DISCUSSION
The cardiovascular system can be affected during systemic inflammatory and rheumatologic disorders.2, 6 Valvular disease, conduction disturbances, myocardial and pericardial involvement, and also vasculitis can be detected. Similarly, intracardiac masses associated with a systemic inflammatory disease such as Wegener’s granulomatosis, sarcoidosis, rheumatoid arthritis, and Behçet’s disease are reported.2, 6 ICT may develop in the presence of a known systemic disorder or might be the isolated and first clinical presentation.2 APS syndrome or Hughes’ syndrome is a rare prothrombotic autoimmune disease characterized by recurrent vascular thrombosis events (arterial, venous, or even microvascular circulation), fetal loss, and persistent (at least 12 weeks apart) elevated levels of antiphospholipid antibodies, including the lupus anticoagulant (LA), and /or moderate-to-high levels of the isotypes of immunoglobulin M (IgM) or immunoglobulin G (IgG) anticardiolipin (aCL) antibodies or the isotypes of IgG or IgM anti-β2GPI, leading to a hypercoagulable state and related symptoms.(1-4) The diagnosis is confirmed in the presence of at least 1 clinical and 1 laboratory criteria. The annual incidence of APS was estimated at around 5 new cases per 100 000 people.9 This syndrome may be developed as a primary condition or a secondary disorder related to an underlying disease, most commonly systemic lupus erythematosus. Mavrogeni et al5 described cardiac involvement as a common complication found in 30% of patients following APS. Also, this syndrome, itself, was considered a risk factor for cardiovascular disease.10 Cardiac complications are categorized into 2 main subgroups of valvular heart disease (more common)5, 11 and non-valvular involvement (less frequent), including ICT, myocardial disease, LV and RV dysfunction, and pulmonary hypertension.5 Cardiac thrombosis is an uncommon complication(4-7) and is responsible for 1.9% of all cases of ICT.15 Albeit ICT is a life-threatening complication, appropriate treatment may decrease its morbidity and mortality rates. Ye Z-X et al16described a 22-year-old woman with an RA thrombus, deep vein thrombosis/pulmonary thromboembolism, and positive aCL antibodies. Voigtlaender et al17 described a 67-year-old woman with an RA thrombus, thrombocytopenia, and positive LA antibodies. Zahid et al18 reported a case of an RA thrombus and pulmonary thromboembolism, as the first presentations of APS, in a 26-year-old man confirmed by positive LA/aCL antibodies.
In 2011, Permanyer et al19 described a 43-year-old woman with a cardiac mass at the injunction of RA to IVC (dilated IVC), which was finally was diagnosed as APS syndrome due to strongly positive LA/aCL antibodies. In a literature review conducted by Waisayarat et al9 on 9 patients with ICT and APS, most of the patients were women aged between 30 and 40 years old. The heart chamber with the most involvement was RA, and LA and LV were less commonly involved. LA antibodies, followed by aCL antibodies, were positive in most cases, and anti-β2GPI was positive in only 2 cases.
In another literature review, Dhibar et al20 showed that fever of unknown origin was a rare presentation in primary APS. Their analysis on 28 cases presenting with ICT showed a relatively female predominance with more common right heart involvement, not least RA, and to a lesser extent, LA or LV involvement. Only 2 cases had a simultaneous involvement of LV/LA and right heart. Primary APS was more prevalent, and in most cases LA/aCL antibodies were positive. Positive anti-β2GPI was reported in only 3 patients, all of whom had an RA mass and positive LA and/or aCL antibodies. In contrast to these case reports, our patient, who was a man presenting with extensive ICT and simultaneous RA/RV/LV/and IVC masses, had anti-β2GPI as the only positive antibody in the laboratory assessment. Such discrepancies with prior cases prompted us to report this case as a relatively strange example. It is probable that in the follow-up of our patient, other groups of positive antibodies may be detected.