We present a rare case of a young female patient with lupus-negative Libman-Sacks endocarditis complicated by CAPS. [ncbi.nlm.nih.gov]

Systemic thromboembolism can manifest as peripheral arterial embolism presenting as cold limbs or mesenteric ischemia presenting as severe abdominal pain. [statpearls.com]

• Camping

  Camps M.T. et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002; 46: 1019-1027 Asherson R.A. Cervera R. [thrombosisresearch.com]

• Cyanotic Congenital Heart Disease

  Cardiac manifestations include coronary by-pass graft and angioplasty occlusions, cardiomyopathy, cyanotic congenital heart disease, intracardiac thrombus and complications of cardiovascular surgery. [thrombosisresearch.com]

• Respiratory Distress

  The diagnosis is challenging especially if there is no history of APLS and should be suspected in patients presenting with acute renal failure and the respiratory distress syndrome, diffuse alveolar haemorrhage, encephalopathy, and adrenal haemorrhage [academic.oup.com]

• Heart Disease

  This volume is a must-have for every cardiologist needing to be fully current on recent advances in structural heart disease and its associated disorders. [books.google.com]

  Antiphospholipid antibodies, valvular heart disease, and systemic lupus erythematosus. Am. J. Med. 1989; 86: 633-634 Straaton K.V. Chatham W.W. Smith S.H. Koopman W.J. Clinically significant valvular heart disease in systemic lupus erythematosuse. [thrombosisresearch.com]

  Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare. [actamedindones.org]

  Despite this, these patients have a greater incidence of stroke, peripheral embolism, heart failure, infective endocarditis, and death as compared with patients with no valvular heart disease 9. [scielo.br]

  Clinically significant valvular heart disease in systemic lupus erythematosus. Am J Med. 1989; 86: 407-412 Cervera R. Font J. Pare C. et al. Cardiac disease in systemic lupus erythematosus: prospective study of 70 patients. [amjmed.com]

• Heart Murmur

  The second question came down to a left-sided systolic murmur. [forums.studentdoctor.net]

  Fever and a heart murmur are sometimes present. Blood cultures Echocardiography Noninfective endocarditis should be suspected when chronically ill patients develop symptoms suggesting arterial embolism. [merckmanuals.com]

  Visits to the dentist offer opportunities for bacteria to sneak into the blood and land on a heart valve. Therefore, people with severe heart murmurs should speak to their doctor about taking an antibiotic before receiving dental work. [hopkinslupus.org]

  Heart Endocarditis Noninfective endocarditis Topic Completed: 1 April 2014 Revised: 4 March 2019 Copyright: 2014-2019, PathologyOutlines.com, Inc. [pathologyoutlines.com]

  Infective endocarditis[edit] Infective endocarditis is an infection of the inner surface of the heart, usually the valves.[3] Symptoms may include fever, small areas of bleeding into the skin, heart murmur, feeling tired, and low red blood cells.[3] Complications [en.wikipedia.org]

• Systolic Murmur

  Auscultation revealed a loud systolic murmur (IV/VI) at the apex of the heart. [ahajournals.org]

  The second question came down to a left-sided systolic murmur. [forums.studentdoctor.net]

  The following cardiac murmurs might be heard: Ejection systolic murmur (crescendo decrescendo): This is most commonly the result of a hyperdynamic state due to associated conditions and can indicate aortic valve thickening with or without stenosis. [odlarmed.com]

  On cardiac auscultation, she had a grade II systolic murmur in the apex area. Electrocardiography (ECG) showed sinus rhythm. Lung auscultation was unremarkable. Abdominal examination did not reveal organomegaly. [cardiovascimaging.portal.tools]

  The prominent finding on cardiovascular examination was a grade 3/6 ejection systolic murmur at the right upper sternal border conducted to the carotids. Chest radiograph showed a cardiothoracic ratio of 0.55. [annalspc.com]

• Macula

  Fundoscopic examination showed a pale retina with a cherry-red macula (Figure 1) and a diagnosis of right central retinal artery occlusion was made. [revportcardiol.org]

• Suggestibility

  These findings suggested that multiorgan failure was caused by systemic thrombosis in the small vessels but not by vasculitis. Figure 6. [ahajournals.org]

  All of the above are suggestive as significant for lupus, with secondary APLS. [academic.oup.com]

  However, data suggests a correlation between Libman–Sacks endocarditis and an increased risk for embolic cerebrovascular disease in SLE patients. [ncbi.nlm.nih.gov]

• Seizure

  The purpose of this study is to determine whether Libman-Sacks endocarditis (inflammation of the heart valves) is the cause of neuropsychiatric manifestations (stroke, transient ischemic attacks, cognitive dysfunction, seizures, acute confusional state [clinicaltrials.gov]

  […] thrombocytopaenia coagulopathy U+E CXR ECHO CT/MRI DIAGNOSTIC CRITERIA (require >4) malar rash (butterfly) discoid rash photosensitivity oral ulcers non-erosive arthritis serositis: pleuritis, pericarditis renal: proteinuria, casts central nervous: seizures [lifeinthefastlane.com]

  Online Videos 5 A, D, E, F 50 year old female with SLE with an acute transient ischemic attack, progressive memory loss, past stroke and seizure disorder. [imaging.onlinejacc.org]

  She denies history of rash, photosensitivity, renal problems, seizures, pleurisy, pericarditis, or anaemia. She admits occasional canker sores sometimes and mild thinning of the hair. On arrival, the patient was haemodynamically stable. [academic.oup.com]

A hypercoagulable workup should also be obtained in each case suspected of LS endocarditis including lupus anticoagulant and antiphospholipid antibodies. However, the primary evaluation for LSE is by echocardiography. [statpearls.com]

Autoimmune workup was negative. Hematological workup showed an isolated lupus anticoagulant with normal anti-cardiolipin antibodies. Repeat lupus anticoagulant a few days later was negative. [ncbi.nlm.nih.gov]

• Leukocytosis

  Given the fever, leukocytosis and suspected cardioembolic phenomenon causing infarctions in the brain, there was concern for bacterial endocarditis. [ncbi.nlm.nih.gov]

Improvements in imaging modalities, particularly in echocardiography, have allowed better estimation of the prevalence of the disease, but further investigation is still needed into its pathogenesis, treatment, and association with APS. [ucdavis.pure.elsevier.com]

Treatment / Management The treatment for patients with LS endocarditis is not well established, partially due to the scarcity of information on the natural history of the disease in untreated patients. [statpearls.com]

Four patients were on treatment with steroids, 2 were on hydroxychloroquine, and 2 were treatment-naive. One patient was treated with Cyclophosphamide, Rituximab, and plasmapheresis for pulmonary hemorrhage. [acrabstracts.org]

Although valvular dysfunction is rare, when it occurs, treatment with steroids and MMF may reduce the lesions and postpone the need for valve replacement. [revistanefrologia.com]

Optimal treatment, including adequate aggressive anticoagulation therapy and specific treatment for heart failure, may play a pivotal role in reducing the severity of valve dysfunction in these patients. [ncbi.nlm.nih.gov]

Prognosis The prognosis of LS endocarditis has not been fully studied and there continues to be a need for further studies to better define prognosis. From clinical observations, the prognosis of LS is considered poor. [statpearls.com]

Prognosis The prognosis of LS endocarditis requires further study, and there continues to be a need for additional research to better define prognosis. From clinical observations, the prognosis of LS is considered poor. [ncbi.nlm.nih.gov]

The diagnosis of LS generally gets delayed as we look for other etiologies of patients presenting illness (thromboembolic event). [statpearls.com]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. [hon.ch]

In this article Epidemiology History Examination Investigations Management Complications Prognosis Historical Libman-Sacks Endocarditis In this article Epidemiology History Examination Investigations Management Complications Prognosis Historical Synonyms [patient.info]

Epidemiologic, twin, and human leukocyte antigen data suggest a strong genetic contribution to the etiology of SLE, but the exact cause is unknown. [pedsinreview.aappublications.org]

"Current Cancer Epidemiology". Journal of Epidemiology and Global Health. 9 (4): 217–222. doi:10.2991/jegh.k.191008.001. PMC 7310786. PMID 31854162. ^ a b Bender E (September 2014). "Epidemiology: The dominant malignancy". [en.wikipedia.org]

Epidemiology In patients with SLE, the reported prevalence of LS vegetations are 1 in 10 patients. [1] LS endocarditis most commonly affects patients between 40 to 80 years of age. [3] A significant correlation was found between LS endocarditis and SLE [statpearls.com]

In order to provide the latest and most sophisticated treatment, cardiology physicians must possess current knowledge of a vast amount of translational research in the pathophysiology of structural heart disease and its associated disorders as well as [books.google.com]

[…] endocarditis was higher in patients with underlying malignancy compared to the general population on autopsy (1.25% versus 0.2%). [4] The highest incidence of LS endocarditis in this study was observed in patients with pancreatic adenocarcinoma. [4] Pathophysiology [statpearls.com]

This activity describes the pathophysiology, evaluation, and management of Libman-Sacks endocarditis and highlights the role of the interprofessional team in the management of this condition. [ncbi.nlm.nih.gov]

In case of SLE with APLS, in which anticoagulation and antiplatelet therapy is required to prevent the thromboembolic event and thrombocytopenic purpura, after valve replacement, strict management of anticoagulation plays an essential role to prevent [jstage.jst.go.jp]

Anticoagulation should be considered as secondary prevention for thromboembolic phenomena in patients who have had a thromboembolic event. [statpearls.com]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

Conclusions: Once a diagnosis of APS is established, primary or secondary stroke prevention is imperative. [n.neurology.org]

It is also associated with low platelet or blood elements that prevent bleeding. [stillsdisease.org]