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Libman-Sacks Disease


Presentation

  • Complete catalog of autoantibodies produced * Includes an outstanding review of imaging techniques for the diagnosis of lupus and its complications for each organ system * Includes CD-ROM of images excellent for teaching purposes and downloading into presentations[books.google.com]
  • Improve your interpretation of presenting symptoms with 39 new topics in the Differential Diagnosis section, and optimize patient care with 12 new tables in the Clinical Practice Guidelines section.[books.google.com]
  • We present a 30-year-old man with probable primary APS (with no history of thromboses) presenting with diffuse alveolar hemorrhage, an uncommon presentation.[journals.lww.com]
  • Among these, antinucleosome antibodies were present in 15 patients.[annalspc.com]
Noncompliance
  • Patients were excluded due to age 60 years, pregnancy, heart or brain disease unrelated to SLE, atrial fibrillation or flutter, cardiomyopathy, intracardiac thrombi, drug abuse, renal dysfunction, difficult venous access, self-withdrawal or noncompliance[imaging.onlinejacc.org]
Epilepsy
  • Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus.[scielo.br]
  • Other neuralgic presentations include epilepsy, transverse myelopathy, Guillain-Barrà syndrome and chorea.[stillsdisease.org]
Pathologist
  • Kradin is also an Associate Pathologist at Massachusetts General Hospital in the Immunopathology Unit. His techniques include tissue culture and immunocytochemistry, flow cytometry, Northern blotting and immunocytochemistry. Dr.[books.google.com]
  • Libman was at the time assistant pathologist at Mount Sinai hospital in New York. He was an eccentric generalist, who founded the cardiology department, and made important contributions in bacteriology and teaching.[patient.info]
Multiple Organ Dysfunction Syndrome
Failure to Thrive
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.com]
Heart Disease
  • Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare.[actamedindones.org]
  • Note also the thickened, shortened, and fused chordae tendineae that represent remote rheumatic heart disease.[library.med.utah.edu]
  • disease IV drug use immunosuppression prosthetic heart valve congenital heart disease Etiology Pathogenesis endothelial damage on the surface of the cardiac valve can cause a thrombus to form factors include turbulent blood flow that can damage endothelium[medbullets.com]
  • Disease Valvular heart disease is a well-recognized cardiac manifestation in APS and SLE.[phaonlineuniv.org]
Heart Murmur
  • 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]
  • Therefore, people with severe heart murmurs should speak to their doctor about taking an antibiotic before receiving dental work.[hopkinslupus.org]
  • murmur and Evidence of multiple systemic emboli Main differential diagnosis is Infective endocarditis - distinction is important Laboratory Exclude DIC: full blood count, prothrombin time, partial thromboplastin time, fibrinogen, thrombin time, D dimers[pathologyoutlines.com]
  • Also I'm not sure if the heart murmur was described, but this would be another way of ruling out stenosis over regurg This is how I think of why regurg stenosis: LSE buildup of gunk on leaflets, I shouldn't take much gunk buildup to WEIGH DOWN the leaflet[forums.studentdoctor.net]
  • A previously healthy 14-year-old girl was found to have a heart murmur when examined during an episode of upper respiratory tract infection.[annalspc.com]
Mitral Valve Prolapse
  • "Mitral valve prolapse and out-of-hospital sudden death: a case report and literature review.". Med Sci Law 50 (3): 164-7. PMID 21133272. 10.0 10.1 10.2 Virmani, R.; Atkinson, JB.; Forman, MB. (Aug 1988). "The pathology of mitral valve prolapse.".[librepathology.org]
  • With transcranial Doppler in 70 patients, 39% of 38 patients with versus none of 32 without antiphospholipid antibodies had microembolism associated with cerebrovascular ischemia and mitral valve prolapse (35) .[imaging.onlinejacc.org]
Purpura
  • Abstract A 42-year-old woman with antiphospholipid syndrome (APLS) secondary to systemic lupus erythematosus (SLE) complicated with thrombocytopenic purpura was successfully treated by mitral valve replacement with a mechanical prosthesis and tricuspid[jstage.jst.go.jp]
  • [Kawasaki] M30.8 Other conditions related to polyarteritis nodosa Polyangiitis overlap syndrome M31 Other necrotizing vasculopathies M31.0 Hypersensitivity angiitis Goodpasture's syndrome M31.1 Thrombotic microangiopathy Thrombotic thrombocytopenic purpura[apps.who.int]
  • D) Other Features Association of antiphospholipid antibodies with renal vein thrombosis, AddisonÂ’s disease, gut ischemia, Budd-Chiari syndrome, thrombocytopenia, autoimune hemolytic anemia, idiopathic thrombocytopenic purpura, cardiac valve abnormalities[stillsdisease.org]
Renal Insufficiency
  • The features these patients exhibited in common were an arthritis of varying duration, the subsequent development of albuminuria of rather marked degree, nephrotic edema, subsequent renal insufficiency and, finally, a fatal outcome with the pathologic[jamanetwork.com]
Homonymous Hemianopsia
  • Online Video 1A 18 year old female with SLE and acute homonymous hemianopsia, confusional state, and cognitive dysfunction.[imaging.onlinejacc.org]
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]

Workup

  • 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]
Albuminuria
  • The features these patients exhibited in common were an arthritis of varying duration, the subsequent development of albuminuria of rather marked degree, nephrotic edema, subsequent renal insufficiency and, finally, a fatal outcome with the pathologic[jamanetwork.com]
Candida
  • Introduction Clinical definition inflammation of the heart valve, typically secondary to infection Epidemiology location mitral valve tricuspid valve tricuspid valve disease is associated with intravenous (IV) drug use Staphylococcus aureus, Pseudomonas, Candida[medbullets.com]
White Matter Lesions
  • In a study of 14 patients, focal white matter lesions and cerebral infarcts on pre-mortem MRI correlated highly with old and acute cerebral infarcts and microthromboemboli on histopathology (10) .[imaging.onlinejacc.org]
Gliosis
  • (G and H) (H&E 40 and 100 , respectively) Old deeper (white matter) infarct with liquefactive necrosis, residual macrophages, and gliosis (arrows) . (I) (H&E 100 ) Large cerebral vessel with fibrin thrombi (arrow) .[imaging.onlinejacc.org]

Treatment

  • Home Vol 49, No 2 (2017) Ginanjar Autoimmune Disease with Cardiac Valves Involvement: Libman-Sacks Endocarditis Abstract This case study aim to evaluate the response of steroid treatment for autoimmune endocarditis.[actamedindones.org]
  • Cardiologia 18(4): 225-233, 1951 Therapeutic value of partial substitutive transfusions in the treatment of malignant lupus erythematosus visceritis (Libman-Sacks disease) .[eurekamag.com]
  • This edition focuses on evidence-based findings, treatment consensuses, and practical clinical information.[books.google.com]
  • Focus on the development, function, and treatment of a wide range of disease entities with the text’s clear, logical, organ-system approach.[books.google.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

  • […] contributing authors—twenty of them new to this edition—provide comprehensive coverage of every aspect of cutaneous and systemic lupus erythematosus, including definitions, pathogenesis, autoantibodies, clinical and laboratory features, management, prognosis[books.google.com]
  • 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]

Etiology

  • It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system.[hon.ch]
  • . * Comprehensive current coverage of basic models of etiology and pathogenesis * Complete catalog of autoantibodies produced * Includes an outstanding review of imaging techniques for the diagnosis of lupus and its complications for each organ system[books.google.com]
  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]

Epidemiology

  • 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]
  • […] valve surface, rich in fibrin and platelet aggregates but devoid of inflammation or infective organisms Terminology Also called marantic endocarditis Diagrams / tables Images hosted on other servers: Differences between NBTE and infective endocarditis Epidemiology[pathologyoutlines.com]
  • 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]
  • Introduction Clinical definition inflammation of the heart valve, typically secondary to infection Epidemiology location mitral valve tricuspid valve tricuspid valve disease is associated with intravenous (IV) drug use Staphylococcus aureus, Pseudomonas[medbullets.com]
Sex distribution
Age distribution

Pathophysiology

  • […] endocarditis Diagrams / tables Images hosted on other servers: Differences between NBTE and infective endocarditis Epidemiology NBTE is uncommon but underestimated as symptoms are often attributed to other diseases Sites Cardiac valves and endocardial surface Pathophysiology[pathologyoutlines.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]

Prevention

  • 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]
  • 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]

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