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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a Chinese woman who developed severe heart failure 3 years from the onset of systemic lupus erythematosus (SLE). Endomyocardial biopsy confirmed lupus myocarditis, with focal infiltrates of small lymphocytes and some polymorphic neutrophils. The conventional treatment for cardiac failure plus oral prednisolone failed to bring clinical and echocardiographical improvement until the addition of intravenous (i.v.) 'pulse' cyclophosphamide. Three weeks after i.v. cyclophosphamide treatment, there was significant improvement of her heart failure symptoms with improvement in the ejection fraction from 19% to 63%.
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PMID:Intravenous cyclophosphamide improves cardiac dysfunction in lupus myocarditis. 1469 Jan 45

In patients receiving immunosuppressive therapies, Strongyloides stercoralis can cause a life-threatening septic shock, with multi-organ failure and infestation. Strongyloides hyper-infection should be considered in any immunosuppressed patient who has been exposed to the parasite, even if it is many years since that exposure occurred. Delayed eosinophilia may be a feature and treatment with high doses of anthelmintics may be required. An interesting case of S. stercoralis hyper-infection was recently observed at the Royal Darwin Hospital in tropical, northern Australia. The patient was an 18-year-old female with lupus glomerulonephritis, who was receiving immunosuppression in the form of corticosteroids and pulse cyclophosphamide. The characteristics and intensive-care management of this case, including the use of granulocyte-colony stimulating factor and high-dose ivermectin, are described. The patient, who survived, appears to represent the first reported case of S. stercoralis hyper-infection with suspected myocarditis.
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PMID:Strongyloides hyper-infection: a case for awareness. 1475 98

Clinically important myocarditis is an unusual feature in patients with systemic lupus erythematosus (SLE). We report three consecutive lupus patients over a 1 year period who developed severe left ventricular dysfunction in the absence of coronary artery disease or hypertensive cardiomyopathy. Two of them had clinical and biological flare of the disease whereas the lupus was quiescent in the latter. Two of them had positive IgG anticardiolipin antibodies. High dose steroids were given in two patients; one of them also required cyclophosphamide on account of diffuse proliferative glomerulonephritis. Left ventricular function improved quickly and markedly in these two patients; one of them had recurrence of severe myocarditis at intervals of 6 years and was each time responsive to steroids. Lupus cardiomyopathy, a rare event in the course of SLE, can be related to the disease even in the absence of coronary artery disease or hypertensive cardiomyopathy. It may be improved by steroids and immunosuppressive therapy. Literature concerning this cardiac manifestation in lupus is reviewed.
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PMID:Specific cardiomyopathy in lupus patients: report of three cases. 1476 25

Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.
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PMID:Heart disease in systemic lupus erythematosus: diagnosis and management. 1522 37

Reduction of pathological autoantibodies may be useful in the treatment of systemic lupus erythematosus (SLE). On the other hand clinically manifested myocarditis in SLE, though uncommon, may be life-threatening and its pathogenesis has been ascribed to autoimmunity. The aim of this study is to present a rare case of a patient with severe lupus myocarditis, where immunoadsorption (IA) was evaluated as rescue therapy. A case of SLE with initial manifestation of myocarditis is reported in a 29-year-old male who presented with arthritis, fever, lymphadenopathy, joint swelling and morning stiffness. Laboratory evaluation revealed increased antinuclear antibody (ANA), slightly decreased complement and positive anticoagulant panel. From the above clinical and laboratory features, criteria of SLE seemed applicable. During his hospitalization, the patient developed pericardial effusion and cardiogenic shock. Although pericardiotomy was performed and was treated with immunosuppressive agents, plasmapheresis and supported with current medications, his clinical condition remained critical with an ejection fraction of 20%. At this point it was decided to receive IA onto staphylococcal protein A. After 6 sessions with IA and concomitant immunosuppression, the patient responded well, his condition improved and was dismissed with an ejection fraction of 50%. Fulminant lupus myocarditis is a severe and rare situation lacking a satisfying specific therapy available today. In our presented case, IA in addition to immunosuppressive therapy was beneficial. Considering the benefits of our case and the current knowledge, it might be useful to clarify the open question in scale pilot studies.
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PMID:Immunoadsorption in lupus myocarditis. 1527 78

The purpose of this prospective, pilot study was to determine whether differences in myocardial T2 relaxivity can be identified among active systemic lupus erythematosus (SLE) patients with clinically suspected SLE myocarditis, other active SLE patients, inactive SLE patients and age and gender matched controls. Eleven consecutive female patients (six with active SLE and five with inactive SLE), and five age, gender and race matched healthy controls underwent imaging with echocardiography and cardiac magnetic resonance imaging (MRI). Echocardiographic measurements included left ventricular end diastolic (LVEDV) and end systolic volumes (LVESV), and mass (LVM) (all indexed to body mass); ejection fraction and cardiac output. The cardiac MRI measurement was the T2 relaxation time (an index of soft tissue signal, with higher levels suggestive of increased tissue water content). Patients with active SLE had significantly higher LVEDV and LVM than inactive SLE patients and healthy controls, and significantly larger LVESV than healthy controls. Myocardial T2 relaxation times were significantly higher in patients with active SLE compared to those with inactive SLE and to healthy controls, and remained higher even after excluding the two active SLE patients who had clinical myocarditis. The four active SLE patients who underwent repeat cardiac imaging studies after clinical improvement showed normalization of these myocardial abnormalities. The conclusion was that active SLE patients demonstrate abnormalities in myocardial structure manifested by high myocardial T2 relaxation times that normalized after clinical improvement in disease activity. These findings suggest that T2 relaxation values are a sensitive indicator of myocardial disease in patients with SLE and that myocardial T2 relaxation abnormality frequently occur in patients with active SLE, even in the absence of myocardial involvement by clinical criteria.
Lupus 2005
PMID:Cardiac magnetic resonance imaging abnormalities in systemic lupus erythematosus: a preliminary report. 1575 18

Giant cell myocarditis (GCM) is a rare form of myocarditis with a median survival of less than one year. It has been reported to occur in patients with various underlying autoimmune diseases; however, no cases of GCM have been described in patients with clear evidence of underlying systemic lupus erythematosus (SLE). The presentation of GCM may mimic that of lupus myocarditis, including an initial response to immunosuppression. Despite initial clinical similarities, lupus myocarditis and GCM are histologically distinct entities with dramatic differences in prognosis. We report herein a patient with a longstanding history of SLE, who presented acutely with myocarditis, responded well to initial immunosuppression and then subsequently died of progressive heart failure that was found to be due to GCM. Endomyocardial biopsy can help define diagnosis and prognosis of lupus patients presenting with myocarditis, and early referral for cardiac transplantation should be considered in patients diagnosed with GCM.
Lupus 2005
PMID:Giant cell myocarditis: a rare cardiovascular manifestation in a patient with systemic lupus erythematosus. 1575 23

Mercury (Hg) has long been recognized as a neurotoxicant; however, recent work in animal models has implicated Hg as an immunotoxicant. In particular, Hg has been shown to induce autoimmune disease in susceptible animals with effects including overproduction of specific autoantibodies and pathophysiologic signs of lupus-like disease. However, these effects are only observed at high doses of Hg that are above the levels to which humans would be exposed through contaminated fish consumption. While there is presently no evidence to suggest that Hg induces frank autoimmune disease in humans, a recent epidemiological study has demonstrated a link between occupational Hg exposure and lupus. In our studies, we have tested the hypothesis that Hg does not cause autoimmune disease directly, but rather that it may interact with triggering events, such as genetic predisposition, exposure to antigens, or infection, to exacerbate disease. Treatment of mice that are not susceptible to Hg-induced autoimmune disease with very low doses and short term exposures of inorganic Hg (20-200 microg/kg) exacerbates disease and accelerates mortality in the graft versus host disease model of chronic lupus in C57Bl/6 x DBA/2 mice. Furthermore, low dose Hg exposure increases the severity and prevalence of experimental autoimmune myocarditis (induced by immunization with cardiac myosin peptide in adjuvant) in A/J mice. To test our hypothesis further, we examined sera from Amazonian populations exposed to Hg through small-scale gold mining, with and without current or past malaria infection. We found significantly increased prevalence of antinuclear and antinucleolar antibodies and a positive interaction between Hg and malaria. These results suggest a new model for Hg immunotoxicity, as a co-factor in autoimmune disease, increasing the risks and severity of clinical disease in the presence of other triggering events, either genetic or acquired.
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PMID:Mercury and autoimmunity: implications for occupational and environmental health. 1602 90

Criteria of organ-specific autoimmunity are fulfilled in a subset of patients with myocarditis/dilated cardiomyopathy (DCM). In particular, circulating heart-reactive autoantibodies are found in patients and symptom-free relatives. These autoantibodies are directed against multiple antigens, some of which are expressed in the heart (organ-specific), others in heart and some skeletal muscle fibres (partially-heart specific) or in heart and skeletal muscle (muscle-specific). Distinct autoantibodies have different frequency in disease and normal controls. Different techniques detect one or more antibodies, thus they cannot be used interchangeably for screening. It is unknown whether the same patients produce more antibodies or different patient groups develop autoimmunity to distinct antigens. IgG antibodies, shown to be cardiac and disease-specific for myocarditis/DCM, can be used as autoimmune markers for identifying patients in whom immunosuppression may be beneficial and relatives at risk. Some autoantibodies may also have a functional role, but further work is needed.
Lupus 2005
PMID:Autoimmune myocarditis and dilated cardiomyopathy: focus on cardiac autoantibodies. 1621 60

The clinical cardiac manifestations most frequently reported in idiopathic inflammatory myopathies, myositis, are congestive heart failure, conduction abnormalities, that may lead to complete heart block and coronary artery disease. Although clinically overt cardiac involvement is rarely reported in myositis patients, subclinical manifestations are frequently observed and are predominated by conduction abnormalities and arrhythmias detected by ECG. Furthermore, cardiovascular manifestations constitute a major cause of death in myositis, thus cardiac involvement maybe overlooked in these patients. Also children with juvenile dermatomyositis may develop cardiac involvement although the frequency seems to be low. The underlying pathophysiologic mechanisms that may cause cardiac manifestations could involve myocarditis and coronary artery disease as well as involvement of the small vessels of the myocardium. In patients with mixed connective tissue disease (MCTD) clinically significant cardiac involvement is also rare, the most frequently reported manifestations being pericarditis and pulmonary hypertension, the latter often attributable to small vessel disease, and often a prognostic unfavourable manifestation.
Lupus 2005
PMID:Cardiac involvement in autoimmune myositis and mixed connective tissue disease. 1621 72


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