Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 23 year old female presented with dyspnea on exertion and absent pulses in the left upper limb. She had prior history of two first trimester abortions and pre-eclampsia with premature delivery. A Doppler examination had revealed left subclavian and axillary artery thrombosis for which she had been given warfarin six months previously. She was admitted and investigated. Patient had low positive aCL IgG antibody, positive antibeta2gp1 antibody, negative lupus anticoagulant and negative ANA. Patient had cardiomegaly and her echocardiography showed severe aortic regurgitation, moderate mitral regurgitation and moderate pulmonary artery hypertension with poor ejection fraction with normal aortic root. A diagnosis of primary antiphospholipid antibody syndrome with valvular involvement with dilated cardiomyopathy was entertained. A CT angiogram of the aorta revealed narrowing and irregularity of the aorta and its multiple branches suggestive of type III Takayasu's arteritis. Temporal relationship suggests development of aorto-arteritis secondary to APS but simultaneous presence of both these disorders in this patient cannot be ruled out.
Lupus 2011 Dec
PMID:Is aorto-arteritis a manifestation of primary antiphospholipid antibody syndrome? 2184 94

Systemic lupus erythematosus (SLE) is an autoimmune disorder resulting in multiorgan inflammatory damage. The heart is frequently involved in SLE. The best known cardiac manifestations are pericarditis and Libman-Saks endocarditis. Severe valvular impairment is rather rare and occurs in few years and in advanced stage of the disease. In this study we report a case of a young women with SLE and heart failure due to mitral regurgitation as the first manifestation of the disease.
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PMID:[Severe mitral regurgitation as the first symptom of systemic lupus erythematosus in a young women required mitral valve replacement]. 2252 24

A 16 year old female patient with systemic lupus erythematosus presented to rheumatology clinic with a new I-II/VI honking-quality mitral regurgitation murmur. The patient was initially evaluated by transthoracic echocardiogram that revealed mitral valve regurgitation and a large band of tissue under the mitral valve leaflets. Blood cultures were obtained and were negative. Transesophageal echocardiogram provided better visualization of the lesion and showed the band of tissue involving most of the chordae of the posterior mitral leaflet. A diagnosis of Libman-Sacks endocarditis was made given the aseptic nature of the lesions and the patient's underlying lupus. Aggressive management of the lupus showed reduction of the mitral regurgitation and the size of the lesion. Libman-Sacks endocarditis is best evaluated by transesophageal echocardiogram.
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PMID:Libman-Sacks endocarditis in pediatric patient with systemic lupus erythematosus. 2278 45

Libman-Sacks endocarditis is rare in children and adolescents, more so as a first manifestation of systemic lupus erythematosus. Currently, sterile verrucous lesions of Libman-Sacks endocarditis are recognised as a cardiac manifestation of both systemic lupus erythematosus and antiphospholipid syndrome. They are clinically silent in a majority of the cases. The presence of antiphospholipid antibodies in systemic lupus erythematosus is associated with three times higher prevalence of mitral valve nodules and significant mitral regurgitation. We present the case of isolated mitral regurgitation with abnormal looking mitral valve, detected in early childhood, which deteriorated to a severe degree in the next decade and was diagnosed as Libman-Sacks endocarditis after surgical repair from histopathology. The full-blown clinical spectrum of systemic lupus erythematosus with antiphospholipid antibodies was observed several weeks after cardiac surgery. We discuss the atypical course of Libman-Sacks endocarditis with follow-up for 10 years, along with a review of the literature.
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PMID:Libman-Sacks endocarditis as the first manifestation of systemic lupus erythematosus in an adolescent, with a review of the literature. 2280 92

Non-bacterial endocarditis lesions associated with antiphospholipid antibodies (aPLs) in the absence of other criteria for antiphospholipid syndrome or systemic lupus erythematosus is termed an aPL-associated cardiac valve disease. Evidence regarding the management of this condition is sparse. A rare case is described of a 20-year-old female who presented with an incidental finding of 'vegetations on a heart valve'. Echocardiography revealed mitral valve leaflet thickening and echodensities with moderate mitral regurgitation. She had an elevated partial thromboplastin time that did not correct with a mixing study, and elevated levels of antiocardiolipin antibodies. Hence, a diagnosis of aPL-associated cardiac valve disease was made, and the patient commenced on warfarin, hydroxychloroquine, and a short course of oral prednisone. At one year after diagnosis the patient remained symptom-free, and follow up echocardiography revealed resolution of the vegetations with minimal mitral regurgitation. Further evidence is needed to guide the therapy of this rare condition.
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PMID:Antiphospholipid antibody-associated non-infective mitral valve endocarditis successfully treated with medical therapy. 2361 Sep 86

A 42 year-old woman was referred to our hospital with a history of fever and poor general status for the last 30 days. She presented tachycardia and a systolic apical murmur. Laboratory tests revealed leukocytosis of 13,100/mL, hemoglobin of 8.4g/dL and positive systemic lupus erythematosus antibodies (anti-Ro/SSA, anti-La/SSB, anticardiolipin, and antinuclear antibodies); blood culture was positive for Streptococcus gallolyticus. Three-dimensional transesophageal echocardiography was performed and revealed multiple mitral valve vegetations, with leaflet perforation and important mitral regurgitation, as well as large aortic vegetation, with cusp perforation and severe regurgitation. Additionally, a small vegetation was observed on the tricuspid valve, which presented moderate regurgitation. Threedimensional transesophageal echocardiography provides appropriate visualization of complications resulting from infectious endocarditis.
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PMID:Streptococcus gallolyticus infective endocarditis in a patient with systemic lupus erythematosus: a three-dimensional echocardiography evaluation. 2413 68

This study was designed to assess cardiac abnormalities in patients with systemic lupus erythematosus (SLE) by echocardiography. It was an analytic type of cross sectional study, conducted in lupus clinic, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2008 to June 2009. Fifty lupus patients, diagnosed on the basis of ACR criteria, without cardiovascular symptoms, were enrolled in the study and were evaluated by standard echocardiography with color Doppler. SLEDAI was applied for assessment of disease activity. Out of 50 patients 80% had abnormal echocardiographic findings. Pericardial thickening was found in 38% patients, pericardial effusion 20%, diastolic dysfunction 72%, hypokinesia of ventricular wall 8%, overall valvular abnormalities 20%, commonest being aortic regurgitation (12%), followed by mitral regurgitation (8%), and 6% had pulmonary hypertension. Males (100%) were more vulnerable to cardiac involvement than females (68.2%) and later age of disease onset (31-40 years) was associated with higher (87.5%) chance of echo abnormalities. The differences, however, were not statistically significant (p>0.05). There was significant relationship between disease duration and cardiac abnormalities (p<0.01). Active disease (80.08%) was associated with higher frequency of cardiac involvement than disease in remission (62.50%) but the result was not statistically significant (p=0.151). Cardiac abnormalities are very common in lupus patients even when clinically asymptomatic from cardiac aspect. Echocardiography is an excellent non-invasive tool for cardiac evaluation. These observations emphasize a need for further assessment of early intervention to reduce subsequent cardiac morbidity and mortality among the lupus patients.
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PMID:Echocardiographic assessment of cardiac involvement in systemic lupus erythematosus patients. 2429 5

A rare form of vascular disease in systemic lupus erythematosus (SLE), lupus vasculopathy is characterized by necrosis and accumulation of immunoglobulins (IGs) and complements in the wall of arterioles and small arteries resulting in luminal narrowing. Lupus vasculopathy often accompanies lupus nephritis and portends a poor prognosis. Although there is general agreement on the treatment of lupus nephritis, effective treatment strategies for lupus vasculopathy remain to be defined. We report a 20-year-old woman with SLE who presented with generalized tonic-clonic seizure. Her immunosuppressive regimen consisted of mycophenolate mofetil, prednisone and hydroxychloroquine. On physical examination, she was Cushingoid in appearance and hypertensive. Laboratory tests indicated renal disease. Coagulation studies disclosed de novo lupus anticoagulant. Magnetic resonance imaging of the brain demonstrated acute focal cerebral hemorrhage. Echocardiography revealed reduced ejection fraction and severe mitral regurgitation. Despite high-dose glucocorticoids and mycophenolate mofetil, renal function remained poor. Kidney biopsy demonstrated lupus vasculopathy and glomerulonephritis. Plasma exchange therapy and intravenous cyclophosphamide were administered. Over the ensuing four weeks, renal function improved, complement levels increased, autoantibody titers decreased and lupus anticoagulant disappeared. In conclusion, lupus vasculopathy can occur in SLE despite a heavy immunosuppressive regimen. Antiphospholipid antibodies might be involved in the pathogenesis of lupus vasculopathy. Plasma exchange therapy in conjunction with intravenous cyclophosphamide may represent an effective treatment strategy for lupus vasculopathy.
Lupus 2014 Apr
PMID:Lupus vasculopathy: Diagnostic, pathogenetic and therapeutic considerations. 2489 38

We present a series of echocardiography images to demonstrate the myocardial response to a high dose of prednisone. A young woman with systemic lupus erythematosus (SLE) associated with interventricular septal hypertrophy exhibited a high pressure gradient between the ascending aorta and left ventricular outflow tract as well as significant systolic anterior motion (SAM) and mitral regurgitation (MR) during high-dose prednisone treatment. However, the pressure gradient decreased dramatically and the MR disappeared rapidly when the dose of prednisone was reduced. To the best of our knowledge, this is the only adult case of myocardial hypertrophy that is assumed to be related to prednisone use.
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PMID:Prednisone induced two-way myocardial development in a patient with systemic lupus erythematosus. 2478 58

A 53-year-old woman with systemic lupus erythematosus and antiphospholipid syndrome presented with central nervous system (CNS) lupus and vegetation of the mitral and aortic valves. Her CNS lupus was relieved with methylprednisolone pulse therapy; however, her mitral regurgitation worsened, and she developed acute decompensated heart failure. The mitral and aortic valves were replaced with mechanical heart valves. Microscopic examination of the excised valves showed no bacterial invasion, and Libman-Sacks (LS) endocarditis of both valves was confirmed. This was a case of LS endocarditis with clear vegetation that spread over the mitral and aortic valves.
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PMID:Double-valve replacement for mitral and aortic regurgitation in a Patient with Libman-Sacks endocarditis. 2513 Jan 8


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