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Infective Endocarditis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
In nonbacterial thrombotic endocarditis (NBTE), thrombotic vegetations that do not contain microorganisms develop on heart valves. They are found in 1–2% of autopsies, are usually larger than the 1–2 mm diameter vegetations found, for example, in acute rheumatic fever and are most frequently attached at the line of closure. They develop during the terminal phase of a patient's life and are usually incidental findings. However, vegetations may be present for some time prior to death and act as a source of thromboemboli that may cause clinical symptoms and signs. The incidence of visceral emboli in NBTE has been reported to be as high as 40%. Because NBTE is usually a condition found in terminally ill patients with widespread malignant disease, the clinical features suggesting emboli are often missed or ignored. These sterile vegetations are often associated with malignancies, systemic lupus erythematosus (SLE), and antiphospholipid antibody syndrome (APS). Other terms used to describe these vegetations include marantic and verrucous endocarditis. The first time this form of endocarditis was described was in 1924 by Emanuel Libman and Benjamin Sacks. Libman-Sacks endocarditis most commonly affects the mitral and aortic valves, but other valves may also be involved. When large, they can be complicated by embolic cerebrovascular disease, peripheral arterial embolism and by superimposed infective endocarditis.15
Cardiology
Published in Fazal-I-Akbar Danish, Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Culture-negative endocarditis:1 Infective: aBrucella spp.bChlamydia spp.cCoxiella burnetti.dLegionella spp.eMycoplasma spp.fTropheryma whippelii.g Fungi/histoplasmosis.2 Non-infective: a SLE (Libman–Sacks’ endocarditis).b Marantic endocarditis.
Endocarditis
Published in Andrew R. Houghton, MAKING SENSE of Echocardiography, 2013
Non-infective endocarditis occurring in systemic lupus erythematosus is called Libman–Sacks endocarditis (also known as ‘verrucous’ endocarditis), and in this condition, the vegetations mainly consist of immune complexes and mononuclear cells. The mitral and aortic valves are most commonly affected, although just about any part of the endocardium can be involved. The vegetations are usually small, irregular and immobile (compared with the vegetations in infective endocarditis). Libman–Sacks endocarditis is usually asymptomatic, but can present with valvular regurgitation or, less commonly, stenosis. There is also a risk of embolization, although this is uncommon.
Active NET formation in Libman–Sacks endocarditis without antiphospholipid antibodies: A dramatic onset of systemic lupus erythematosus
Published in Autoimmunity, 2018
Daniel Appelgren, Charlotte Dahle, Jasmin Knopf, Rostyslav Bilyy, Volodymyr Vovk, Pia C. Sundgren, Anders A. Bengtsson, Jonas Wetterö, Luis E. Muñoz, Martin Herrmann, Anders Höög, Christopher Sjöwall
In this case report, we describe a young female with some years of prodromal symptoms (including episodic migraine, thrombosis, hypothyreosis and arthralgia) before the prompt and dramatic onset of SLE with cardiac and CNS involvement. This case of Libman–Sacks endocarditis is very rare since it is aPL seronegative. We consistently received negative results for the classical APS tests: LA (dRVVT) as well as aCL and anti-β2GPI antibodies of IgG, IgM and IgA isotypes. In addition, IgG anti-PS/PT antibodies suggestive to be highly associated with APS were negative [28,29]. The hypothesis of alternative, and possibly simultaneous, disease-driving mechanisms behind the SLE manifestations was the cause for our devoted interest in this case.
Treatment failure of direct oral anticoagulants in anti-phospholipid syndrome
Published in Scandinavian Journal of Rheumatology, 2018
SJA Johnsen, MB Lauvsnes, R Omdal
We describe three patients with APS who developed thromboembolic events while being treated with a DOAC at an adequate dose. One of these had a possible Libman–Sacks endocarditis, representing an additional element of risk for thromboembolism. One patient was triple aPL positive, representing an increased thrombotic risk (3). The other two patients had a lower risk for new events, according to this risk stratification.