Libman-Sacks endocarditis

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Classification according to ICD-10
M32.1 + Systemic lupus erythematosus with involvement of organs or organ systems
I39 * Endocarditis and valvular disease in diseases classified elsewhere
ICD-10 online (WHO version 2019)

The Libman-Sacks endocarditis is a disease of the heart . It is a special form of abacterial endocarditis that can be found in systemic lupus erythematosus (SLE). Their name goes back to the American internists Emanuel Libman (1872-1946) and Benjamin Sacks, who first described this form of endocarditis in 1924 in four patients at the Mount Sinai Hospital in New York as atypical verrucous endocarditis (atypical verrucous endocarditis).

Large vegetations on the heart valves , which are mostly fibrotic or consist of proliferating endothelial cells , myocytes and mononuclear inflammatory cells , are typical . Most of the vegetation is small and hemodynamically insignificant. The chordae tendineae can also have a strong thickening. There is a strong propensity for localized inflammatory infiltrates. It is not uncommon for pleurisy or pericarditis to occur. However, valve insufficiency up to heart failure develops in 10% of patients .

Usually the left heart valves are affected, most commonly the mitral valve , followed by the aortic valve . Using transthoracic ultrasound, signs of Libman-Sacks endocarditis are found in 7–11% of all patients with SLE, while transesophageal ultrasound shows changes in up to 43% of all SLE patients. In autopsies of patients who died prematurely from SLE, the rate of endocarditic changes was 13–65%.

Risk factors for endocarditis in SLE patients are high disease activity, the presence of lupus nephritis and the presence of antiphospholipid antibodies (three-fold increased risk).

The optimal therapy for Libman-Sacks endocarditis is unknown. A reduced prevalence in SLE patients and the increased observation of healed lesions after the advent of cortisone therapy in the 1970s suggested that it was efficient. However, cohort studies have shown no connection between immunosuppressive or cytotoxic medication and a change in the occurrence or form of endocarditis. Individual reports also described worsening with cortisone therapy. Surgical interventions are associated with a high risk of complications .

literature

Individual evidence

  1. ^ Emanuel Libman, Benjamin Sacks: A hitherto undescribed form of valvular and mural endocarditis . Arch Intern Med 1924; 33: 701-37