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

Ishizu, Kenichi; Isotani, Akihiro; Yamaji, Kyohei; Ando, Kenji

doi:10.1093/ehjcr/ytz133

Cited by 6 publications

(10 citation statements)

References 15 publications

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“…It may present as infective endocarditis with symptoms of heart failure due to valvular regurgitation and thromboembolic events [6]. LSE is typically situated in the tip, middle, or the base of the posterior mitral leaflets and this can be confused with infective endocarditis vegetation [7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

“…A high index of suspicion for LSE in the presence of multiorgan disease is warranted as early recognition of LSE could have an impact on its management and reduce morbidity and mortality [ 8 ]. According to pathology reports from valvular specimens, the underlying mechanism for LSE showed evidence of inflammation and cell degeneration with fibrin deposition [ 10 - 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…Management of LSE and the usefulness of immunosuppressive for valvular regurgitation were not described in detail before in the pediatric age group but some evidence in the adult population [ 8 , 12 - 13 ]. For instance, Ishizu et al [ 10 ] reported a dramatic improvement after using immunosuppressive medication in LSE with severe mitral regurgitation. However, there was a dramatic improvement in this case after proper SLE management and mitral regurgitation almost resolved in about four months from the initial presentation.…”

Section: Discussionmentioning

confidence: 99%

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All Endocarditis Is Not Infective: Libman-Sacks Endocarditis

Riyami¹,

Joshi²,

Senaidi³

et al. 2022

Cureus

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Libman-Sacks endocarditis (LSE) is an uncommon disorder that might be confused with infective endocarditis. It is one of the systemic lupus erythematosus (SLE) manifestations that could present with heart failure. We report a 12-year-old girl who presented with a history of shortness of breath, joint pain for four weeks, and fever for about one week. On examination, she was pale, edematous, and febrile. Her cardiac exam revealed a pan-systolic murmur of mitral regurgitation, harsh, grade 3/6 best heard at the apex. She was diagnosed with systemic lupus erythematosus with lupus nephritis and carditis. Her echocardiography revealed severe mitral regurgitation with nodular thickening of the valve in keeping with a diagnosis of LSE. After appropriate management of her underlying disorder using immunosuppressive, we saw a dramatic clinical improvement and her heart failure symptoms resolved. This case proves that SLE can have significant cardiac involvement and a proper evaluation would help in overall management and prognosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

All Endocarditis Is Not Infective: Libman-Sacks Endocarditis

Riyami¹,

Joshi²,

Senaidi³

et al. 2022

Cureus

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“…Antiphospholipid syndrome is a non-inflammatory systemic autoimmune disease with an incidence of five new cases/100,000 persons per year and a prevalence of 40–50 cases/100,000 persons ( 3 ) and is more common in young women ( 4 ). “Definite” APS must have recurrent arteriovenous thrombosis and/or morbid pregnancy (habitual abortion, or stillbirth in the middle and late stages) as its main clinical manifestations ( 5 ).…”

Section: Discussionmentioning

confidence: 99%

Case report: Mitral valve replacement for Libman-Sacks endocarditis and cerebral embolism of primary antiphospholipid syndrome

Chen

2022

Front. Cardiovasc. Med.

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Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent arteriovenous thrombosis and/or morbid pregnancy. Valve involvement is the most common cardiac manifestation of APS, with lesions characterized by valve thickening and vegetations known as Libman-Sacks endocarditis (LSE). This report discussed a rare case of a 26-year-old young woman diagnosed with primary APS with multiple cerebral infarctions and right middle cerebral artery occlusion that occured 3 years ago. During the investigation, transthoracic echocardiography (TTE) revealed vegetations on both leaflets of the mitral valve with mild to moderate mitral regurgitation. One year following corticosteroid and anticoagulant treatment, mitral valve fibrosis and moderate to severe regurgitation were noted, after which mitral mechanical valve replacement was finally performed. Accordingly, this report suggests that LSE occurrence should be alerted during the examination of APS patients especially in those with cerebrovascular disease. Furthermore, establishing an early diagnosis and conducting close follow-ups are necessary for its timely intervention and treatment.

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“…In a prospective study of 232 SLE patients treated with hydroxychloroquine, chloroquine, or both for a median of 52 months, a significant protective effect against thrombosis was demonstrated (HR 0.28, 95% CI 0.08–0.90) [ 31 ]. Finally, few isolated case reports have shown that immunosuppressive therapy combined with anti-thrombotic and hydroxychloroquine therapy resolve or improve Libman-Sacks endocarditis and its associated CVD [ 32 – 34 ].…”

Section: Discussionmentioning

confidence: 99%

Libman-Sacks endocarditis and associated cerebrovascular disease: The role of medical therapy

et al. 2021

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Background Libman-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. Objective To 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 materials 17 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. Results Valve 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). Conclusion These 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.

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

Cited by 6 publications

References 15 publications

All Endocarditis Is Not Infective: Libman-Sacks Endocarditis

All Endocarditis Is Not Infective: Libman-Sacks Endocarditis

Case report: Mitral valve replacement for Libman-Sacks endocarditis and cerebral embolism of primary antiphospholipid syndrome

Libman-Sacks endocarditis and associated cerebrovascular disease: The role of medical therapy

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