Severe valvular regurgitation and antiphospholipid antibodies in systemic lupus erythematosus: A prospective, long-term, followup study

Abstract

Objective. To assess whether the presence of antiphospholipid antibodies is related to the incidence and progression of severe valvular dysfunction and the need for valve replacement in patients with systemic lupus erythematosus (SLE). Methods. In this prospective, long-term followup study, the initial echocardiographic findings in a cohort of 61 consecutive SLE patients were compared with those of 40 matched controls. All patients were serially evaluated for 14 ؎ 3 years and had a followup echocardiogram 8 ؎ 3 years after the initial evaluation. Serial determinations of anticardiolipin antibodies and lupus anticoagulant were performed in all cases. Results. The number of SLE patients with valvular abnormalities increased from 39% to 73% between the initial and the followup echocardiography, but only 7 patients (12%) developed severe valvular regurgitation. Severe valvular regurgitation was significantly associated with the presence of high levels of IgG anticardiolipin antibodies (P ‫؍‬ 0.001). The combined incidence of stroke, peripheral embolism, need for valve surgery, and death was 86% in patients with severe valvular regurgitation, compared with 25% in those without (P ‫؍‬ 0.003). Conclusion. In SLE patients, the presence of high levels of IgG anticardiolipin antibodies is associated with the development of severe valvular regurgitation and with a high incidence of thromboembolic events and the need for valvular surgery.

Establishing the Diagnosis of Libman–Sacks Endocarditis in Systemic Lupus Erythematosus

“…9 The associated link of antiphospholipids with lupus is wellknown 11,12 and helps provide an additional mechanism of injury to the cardiac valve as seen in Libman-Sacks endocarditis, possibly one of deposition of immunoglobulin and complement. 13 Studies of echocardiographic findings when compared with anticardiolipin and antiphospholipid antibody titers have shown significant correlation for more severe valve dysfunction with increasing titers. 8,11 [var vr_print_hyperlink]Our patient's white blood cell count was normal on admission and decreased during the course of her hospitalization.…”

“…A previous C3 level 1 year before admission was within the normal range at 113 mg/dL. A quantitative assay of rheumatoid factor was 10 (range [10][11][12][13][14]. The erythrocyte sedimentation rate (ESR) was 106 mm/h (range 0-20 mm/h) and C-reactive protein (CRP) was elevated at 11.4 mg/dL (range <0.9 mg/dL).…”

Autologous hematopoietic stem cell transplantation in systemic lupus erythematosus patients with cardiac dysfunction: feasibility and reversibility of ventricular and valvular dysfunction with transplant-induced remission

“…This is not a surprising finding since correlation between severity of lupus and presence of cardiac involvement has not been demonstrated in other studies. 13,15 The potential cardiac toxicity of both autologous and allogeneic HSCT in patients with hematological malignancies and cardiac co-morbidities has been reported for decades, 5,16,17 with poor pre-transplant LVEF being associated with a higher incidence of cardiac morbidity and mortality in some studies. 18 As a result, patients with hematological malignancies and coexistent significant cardiac dysfunction are precluded from transplant.…”

“…The association between APS and severity of valvular dysfunction has been reported. Perez-Villa et al 15 demonstrated that worsening valve function and eventual need for valve surgery were associated with higher anticardiolipin antibody titers, but not with activity of SLE. Since we had reported previously on induction of remission from APS among patients with SLE undergoing HSCT, 29 it is possible that this reduction in antiphospholipid antibodies may account for the improvement in valvular function observed.…”

“…On the other hand, the role of immunosuppression in Libman-Sacks endocarditis and lupus-related cardiac dysfunction is not clear. The APS and antiphospholipid antibodies have been reported to be correlated to valve dysfunction, with one prospective echocardiographic study 15 showing that patients with these antibodies developed valve lesions. Given that HSCT may induce remission from APS, 29 it may perhaps be that the patients' valvular function may improve due to this.…”