Dataset Information

Immunosuppressive therapy to reduce mitral regurgitation in Libman-Sacks endocarditis: a case report.

ABSTRACT:

Background

Libman-Sacks endocarditis is a cardiac manifestation of systemic lupus erythematosus (SLE) and is characterized by non-bacterial verrucous vegetations, causing valvular stenosis and/or regurgitation. The effectiveness of immunosuppressive therapy for valve dysfunction due to Libman-Sacks endocarditis has not been reported.

Case summary

A 67-year-old woman with a history of chronic atrial fibrillation was emergently admitted with acute decompensated heart failure. Transoesophageal echocardiogram revealed severe mitral regurgitation (MR) due to oedematous thickening and poor coaptation of the medial edge of A2/P2 segments and the lateral edge of A3/P3 segments. Serial blood culture results were negative, suggesting bacterial infective endocarditis to be a less likely cause of valvular damage. Because the patient developed photosensitivity, livedo reticularis, and pancytopenia, Libman-Sacks endocarditis with rapidly progressive SLE was diagnosed on the basis of positive test results of anti-double-stranded DNA-IgG and its complement titer. Two months after, immunosuppressive therapy including corticosteroids, a transoesophageal echocardiogram revealed thinning of the degenerative mitral valve leaflets and a reduction of MR from severe to mild.

Discussion

Corticosteroid therapy for Libman-Sacks endocarditis reportedly increases the extent of fibrosis and scarring of the valve leaflets, resulting in worse valve function. In our patient, MR decreased from severe to mild after corticosteroid therapy. Because low-echoic thickening of the mitral valve leaflets suggested acute oedematous changes without scarring and fibrosis and other clinical symptoms of SLE rapidly progressed, early initiation of immunosuppressive therapy for Libman-Sacks endocarditis lead to a benign clinical course in our patient.

SUBMITTER: Ishizu K

PROVIDER: S-EPMC6764580 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Publications

Immunosuppressive therapy to reduce mitral regurgitation in Libman-Sacks endocarditis: a case report.

Ishizu Kenichi K Isotani Akihiro A Yamaji Kyohei K Ando Kenji K

European heart journal. Case reports 20190901 3

<h4>Background</h4>Libman-Sacks endocarditis is a cardiac manifestation of systemic lupus erythematosus (SLE) and is characterized by non-bacterial verrucous vegetations, causing valvular stenosis and/or regurgitation. The effectiveness of immunosuppressive therapy for valve dysfunction due to Libman-Sacks endocarditis has not been reported.<h4>Case summary</h4>A 67-year-old woman with a history of chronic atrial fibrillation was emergently admitted with acute decompensated heart failure. Transo ...[more]

PMID: 31377769

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Project description:ObjectivesThe aim of this study was to determine whether Libman-Sacks endocarditis is a pathogenic factor for cerebrovascular disease (CVD) in systemic lupus erythematosus (SLE).BackgroundA cardioembolic pathogenesis of SLE CVD manifested as: 1) neuropsychiatric systemic lupus erythematosus (NPSLE), including stroke and transient ischemic attacks (TIA); 2) neurocognitive dysfunction; and 3) magnetic resonance imaging of focal brain lesions has not been established.MethodsA 6-year study of 30 patients with acute NPSLE (27 women, 38 ± 12 years of age), 46 age- and sex-matched SLE controls without NPSLE (42 women, 36 ± 12 years of age), and 26 age- and sex-matched healthy controls (22 women, 34 ± 11 years of age) who underwent clinical and laboratory evaluations, transesophageal echocardiography, carotid duplex ultrasound, transcranial Doppler ultrasound, neurocognitive testing, and brain magnetic resonance imaging/magnetic resonance angiography. Patients with NPSLE were re-evaluated after 4.5 months of therapy. All patients were followed clinically for a median of 52 months.ResultsLibman-Sacks vegetations (87%), cerebromicroembolism (27% with 2.5 times more events per hour), neurocognitive dysfunction (60%), and cerebral infarcts (47%) were more common in NPSLE than in SLE (28%, 20%, 33%, and 0%) and healthy controls (8%, 0%, 4%, and 0%, respectively) (all p ≤ 0.009). Patients with vegetations had 3 times more cerebromicroemboli per hour, lower cerebral blood flow, more strokes/TIA and overall NPSLE events, neurocognitive dysfunction, cerebral infarcts, and brain lesion load than those without (all p ≤ 0.01). Libman-Sacks vegetations were independent risk factors of NPSLE (odds ratio [OR]: 13.4; p < 0.001), neurocognitive dysfunction (OR: 8.0; p = 0.01), brain lesions (OR: 5.6; p = 0.004), and all 3 outcomes combined (OR: 7.5; p < 0.001). Follow-up re-evaluations in 18 of 23 (78%) surviving patients with NPSLE demonstrated improvement of vegetations, microembolism, brain perfusion, neurocognitive dysfunction, and lesion load (all p ≤ 0.04). Finally, patients with vegetations had reduced event-free survival time to stroke/TIA, cognitive disability, or death (p = 0.007).ConclusionsThe presence of Libman-Sacks endocarditis in patients with SLE was associated with a higher risk for embolic CVD. This suggests that Libman-Sacks endocarditis may be a source of cerebral emboli.

Project description:BackgroundLibman-Sacks endocarditis in patients with systemic lupus erythematosus (SLE) is commonly complicated with embolic cerebrovascular disease (CVD) or valve dysfunction for which high-risk valve surgery is frequently performed. However, the role of medical therapy alone for Libman-Sacks endocarditis and associated acute CVD remains undefined.ObjectiveTo determine in this cross-sectional and longitudinal study if conventional anti-inflammatory and anti-thrombotic therapy may be an effective therapy in SLE patients with Libman-Sacks endocarditis and associated acute CVD.Methods and materials17 SLE patients with Libman-Sacks endocarditis detected by two-and-three-dimensional transesophageal echocardiography (TEE) and complicated with acute CVD [stroke/TIA, focal brain injury on MRI, or cognitive dysfunction] were treated with conventional anti-inflammatory and anti-thrombotic therapy for a median of 6 months and then underwent repeat TEE, transcranial Doppler, brain MRI, and neurocognitive testing for re-assessment of Libman-Sacks endocarditis and CVD.ResultsValve vegetations decreased in number, diameter, and area (all p ≤0.01); associated valve regurgitation significantly improved (p = 0.04), and valve thickening did not progress (p = 0.56). In 13 (76%) patients, valve vegetations or valve regurgitation resolved or improved in number and size or by ≥1 degree, respectively, as compared to 4 (24%) patients in whom vegetations or valve regurgitation persisted unchanged or increased in size or by ≥1 degree (p = 0.03). Also, cerebromicroembolism, lobar and global gray and white matter cerebral perfusion, ischemic brain lesion load, and neurocognitive dysfunction resolved or significantly improved (all p ≤0.04).ConclusionThese preliminary data suggest that combined conventional anti-inflammatory and antithrombotic therapy may be an effective treatment for Libman-Sacks endocarditis and its associated CVD and may obviate the need for high-risk valve surgery.

Project description:BackgroundLibman-Sacks endocarditis, characterized by Libman-Sacks vegetations, is common in patients with systemic lupus erythematosus and is commonly complicated with embolic cerebrovascular disease. Thus, accurate detection of Libman-Sacks vegetations may lead to early therapy and prevention of their associated complications. Although two-dimensional (2D) transesophageal echocardiography (TEE) has high diagnostic value for detection of Libman-Sacks vegetations, three-dimensional (3D) TEE may allow improved detection, characterization, and clinical correlations of Libman-Sacks vegetations.MethodsTwenty-nine patients with systemic lupus erythematosus (27 women; mean age, 34 ± 12 years) prospectively underwent 40 paired 3D and 2D transesophageal echocardiographic studies and assessment of cerebrovascular disease manifested as acute clinical neurologic syndromes, neurocognitive dysfunction, or focal brain injury on magnetic resonance imaging. Initial and repeat studies in patients were intermixed in a blinded manner with paired studies from healthy controls, deidentified, coded, and independently interpreted by experienced observers unaware of patients' clinical and imaging data.ResultsThe results of 3D TEE compared with 2D TEE were more often positive for mitral or aortic valve vegetations, and 3D TEE detected more vegetations per study and determined larger sizes of vegetations (P ≤ .03 for all). Also, 3D TEE detected more vegetations on the anterior mitral leaflet, anterolateral and posteromedial scallops, and ventricular side or both atrial and ventricular sides of the leaflets (P < .05 for all). In addition, 3D TEE detected more vegetations on the aortic valve left and noncoronary cusps, coronary cusps' tips and margins, and aortic side or both aortic and ventricular sides of the cusps (P ≤ .01 for all). Furthermore, 3D TEE more often detected associated mitral or aortic valve commissural fusion (P = .002). Finally, 3D TEE detected more vegetations in patients with cerebrovascular disease (P = .01).ConclusionsThree-dimensional TEE provides clinically relevant additive information that complements 2D TEE for the detection, characterization, and association with cerebrovascular disease of Libman-Sacks endocarditis.

Dataset Information

Immunosuppressive therapy to reduce mitral regurgitation in Libman-Sacks endocarditis: a case report.

Background

Case summary

Discussion

Publications

Immunosuppressive therapy to reduce mitral regurgitation in Libman-Sacks endocarditis: a case report.

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