Immunomodulation in antiphospholipid-antibody-associated endocarditis: a case report and review of the literature

BACKGROUND: Non-infectious endocarditis is a rare complication in patients with systemic lupus erythematosus or antiphospholipid syndrome (APS). The mitral valve is mainly affected, usually showing vegetations on the ventricular and atrial side of the valve. CASE SUMMARY: A 27-year-old female patient with a known APS was referred to our hospital with night sweats, weight loss, reduction in performance, and dizziness. A floating structure associated to the mitral valve was identified in a transoesophageal echocardiogram with typical changes, in accordance with a non-infectious endocarditis (Libman–Sacks). Only a trace of mitral regurgitation was present and a mass on the posterior mitral valve leaflet. Laboratory findings showed antibody and inflammatory marker measurements either negative or within normal range. The patient received therapeutic oral anticoagulation using a vitamin K antagonist and a combined immunosuppression consisting of hydroxychloroquine and prednisolone. The symptoms of the patient resolved within 3 months after starting the initial treatment. The follow-up echocardiogram showed an almost normal mitral valve function with only a slight regional thickening of the posterior mitral leaflet and no stenosis. Following a 7-year period of observation being on a medical regimen of hydroxychloroquine and a vitamin K antagonist, no evidence of clinical and/or echocardiographic recurrence was detected. DISCUSSION: This case report represents a successful medical management of non-infectious endocarditis using immunosuppressive and anticoagulation therapies without significant residual lesions. Although optimal management of non-bacterial endocarditis remains in the area of uncertainty, this combination therapy deems promising.