Background Antiphospholipid syndrome (APS) is characterized by spontaneous and recurrent vascular thromboses, abortion and thrombocytopenia. Cardiac manifestations are rare, but may occur as cardiac masses, such as thrombus (Libman-Sacks endocarditis). On the other hand, it is known that the most common type of primary cardiac tumors, myxoma, can produce clinical pictures similar to APS and SLE, and that tumor exeresis resolves symptoms.
Methods We examined 2 patients diagnosed with SLE and APS that presented cardiac mass. They were assessed with careful history taking, physical examination, laboratory tests, echocardiography and histological examination.
Results Case 1. A 13-years-old girl, with history of autoimmune hemolytic anemia treated with corticosteroids, folic acid and splenectomy a year before, presented a right ischemic stroke with hemiparesis sequel. Laboratory tests revealed ANA, antiDNAds, aCL and lupus anticoagulant positive and echocardiography showed a cardiac mass. She was underwent surgery to exeresis the mass, which was compatible with myxoma. She was diagnosed with immune syndrome secondary to myxoma and did not take treatment. Seven months after the complete exeresis of the myxoma, she was admitted to our hospital because of two months fever, polyarthralgia, oral ulcers and malar rash. Echocardiography showed pericardial effusion and blood tests showed lymphopenia, anemia, ANA and antiDNA positive and elevation of acute phase reactants; urinalysis was normal. She was diagnosed with SLE flare and was successfully treated with corticoids and started hydroxychloroquine, azathioprine and acetylsalicylic acid.
The second case is a 48-years-old woman diagnosed with SLE and associated APS (oral ulcers, thrombosis, arthritis, malar rash, ANA, Coombs and aCL positive), with a history of bilateral iliac arterial ischemia caused by myxoma emboli (confimated by histological examination) and a cardiac mass on echocardiogram. Fourteen months after the vascular surgery, the patient still needs treatment with rituximab, azathioprine and corticoids to control SLE activity and is on anticoagulant treatment to prevent thrombotic episodes.
Conclusions In patients with myxoma and symptoms of APS and/or SLE, there is doubt whether these are secondary to myxoma or if these diseases coexist, so it is recommended to closely monitor clinical activity after exeresis of myxoma and consider not suspending immunosuppressants and/or anticoagulants or the progressive withdrawal of drugs according to symptoms, in order to avoid possible serious complications of autoimmune disease.
Funding Source(s): None
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