Hemoptysis and a cardiac murmur: is it primary or secondary antiphospholipid syndrome?

Endocarditis is most frequently infective in origin, and thus, when a patient presents with a clinical picture suggestive of endocarditis, an extensive work up aimed at finding the infectious agent is warranted. Among systemic lupus erythematosus (SLE) patients, cardiovascular disease is prevalent in more than 50% of patients including valvular disease and non-infective endocarditis, known as Libman–Sacks (LS) endocarditis. The prevalence of LS syndrome among SLE patients with secondary antiphospholipid syndrome (APS) is higher than in SLE without APS. Here, we present a case of a patient diagnosed with primary APS who presented with hemoptysis and a cardiac murmur. The diagnosis of SLE was established following the findings of non-infective verrucous vegetations together with diffuse alveolar hemorrhage (DAH). Treatment with high-dose corticosteroids and intravenous immunoglobulins yielded substantial resolution of the vegetations and regression of the DAH. Hence, aortic valve replacement was successfully performed as an elective procedure and without any postoperative complications. The patient is in remission after a 6-month follow-up. The clinical findings of DAH and double valve non-infectious endocarditis prompted the diagnosis of SLE with secondary APS.