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]

• Anemia

  […] assoc w SLE Hemolytic anemia associated with systemic lupus erythematosus Hemolytic anemia with Systemic lupus erythematosus Inflammatory myopathy due to lupus Inflammatory myopathy due to SLE Lung disease with systemic lupus erythematosus Lupus erythematosus [icd9data.com]

  He was sent to Bethesda Naval Hospital where he tested positive for other auto-antibodies, and upon further evaluation was diagnosed with aplastic anemia. After conferring with Dr. [stillsdisease.org]

  The blood picture showed normocytic normochromic anemia. The erythrocyte sedimentation rate (ESR) was 89 mm/h and C-reactive protein was <5 mg/dl. Blood urea was 34 mg/dl and creatinine 0.7 mg/dl. [annalspc.com]

• Infertility

  Therefore, these drugs can induce reversible infertility. Cyclophosphamide can cause irreversible infertility through ovarian failure caused by the cumulative effect of high doses [ 10 ]. [ncbi.nlm.nih.gov]

  Cyclophosphamide (also known as Cytoxan), can lead to infertility by causing premature ovarian insufficiency (POI), the loss of normal function of one's ovaries prior to age forty.[49] Methotrexate can cause termination or deformity in fetuses and is [en.wikipedia.org]

• Surgical Procedure

  Common treatments include chemotherapy, radiation and surgical procedures. Photoradiation and hyperthermia are also used as treatment forms to kill or reduce malignant cells. [en.wikipedia.org]

  A careful choice of surgical procedure, i.e., valve repair or replacement with a mechanical valve or a bioprosthesis, is extremely important. Each procedure has advantages and disadvantages. [elsevier.pt]

• Dyspnea

  She underwent surgical aortic valve replacement and had resolution of her dyspnea as well as neurological recovery. [acc.org]

  Cardiac failure might develop secondary to valvular dysfunction (most commonly mitral regurgitation), leading to dyspnea, orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, and lethargy. [odlarmed.com]

  A 23-year-old man presented with complaints of exertional dyspnea, symmetrical arthritis, and facial exanthema. Auscultation revealed a loud systolic murmur (IV/VI) at the apex of the heart. [ahajournals.org]

  orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, lethargy Cerebrovascular embolism Focal weakness or numbness, visual loss, dysphasia, dysarthria, dysphagia, memory loss Systemic thromboembolism Pain, coldness and numbness of the peripheries [pathologyoutlines.com]

  Admitted for resting dyspnea and marked bloody sputum; at exploration she presented tachypnea, increased central venous pressure, bilateral rales up to the middle lung field, and grade IV/VI pansystolic mitral murmur. [revespcardiol.org]

• Orthopnea

  […] therapy Clinical features There are no pathognomonic signs and symptoms that allow for the confident diagnosis of NBTE Patients can present with: Cardiac failure Secondary to valvular dysfunction (most commonly mitral regurgitation), leading to dyspnea, orthopnea [pathologyoutlines.com]

  Cardiac failure might develop secondary to valvular dysfunction (most commonly mitral regurgitation), leading to dyspnea, orthopnea, paroxysmal nocturnal dyspnea, peripheral edema, and lethargy. [odlarmed.com]

• Exertional Dyspnea

  A 23-year-old man presented with complaints of exertional dyspnea, symmetrical arthritis, and facial exanthema. Auscultation revealed a loud systolic murmur (IV/VI) at the apex of the heart. [ahajournals.org]

  In July 2012, a 47-year-old woman was admitted to our cardiology department with exertional dyspnea, peripheral edema, and chest discomfort. [thij.org]

• Vomiting

  […] peripheral edema, lethargy Cerebrovascular embolism Focal weakness or numbness, visual loss, dysphasia, dysarthria, dysphagia, memory loss Systemic thromboembolism Pain, coldness and numbness of the peripheries, or acute abdominal syndromes with pain and vomiting [pathologyoutlines.com]

  Rarely, systemic thromboembolism can result in a wide spectrum of symptoms, including pain, coldness and numbness of the peripheries, or acute abdominal syndromes with pain and vomiting. [odlarmed.com]

  She denies fever, chills, headache, night sweats, weight loss, nausea, or vomiting. On further questioning, she stated that 21 years ago, she had inflammatory arthropathy, and was diagnosed with lupus by a rheumatologist. [academic.oup.com]

• Nausea

  She denies fever, chills, headache, night sweats, weight loss, nausea, or vomiting. On further questioning, she stated that 21 years ago, she had inflammatory arthropathy, and was diagnosed with lupus by a rheumatologist. [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]

• Erythema

  The patient had malar erythema, photosensitivity, discoid cutaneous lesions, symmetric polyarthritis, oral ulcers, and positive antinuclear factor titers (1:1024). [scielo.br]

  […] relationship of skin and systemic manifestations during disease activity.[128] The 19th century's research into lupus continued with the work of Sir William Osler who, in 1895, published the first of his three papers about the internal complications of erythema [en.wikipedia.org]

• Renal Injury

  injury adrenal suppression haematological abnormalities may have restrictive lung disease About Dr Chris Nickson An oslerphile emergency physician and intensivist suffering from a bad case of knowledge dipsosis. [lifeinthefastlane.com]

• 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]

• Confusion

  The terminology used in referring to these varied conditions is much confused and is by no means certain, but some relation among them at least seems possible. In searching for a useful clinical designation for the entire group W. [jamanetwork.com]

  Often confused with classic infective endocarditis during its early stages, NBTE can lead to valvular dysfunction, heart failure, and systemic embolization when unchecked. [ingentaconnect.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]

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]