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PubMedDate not supplied

Detecting cardiac valvular pathology in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is associated with multiple cardiac complications, including valvular damage and an increased risk of bacterial endocarditis. The purpose of this study was to evaluate prospectively a group of patients with SLE for the presence of valvular abnormalities in order to assess their candidacy for antibiotic prophylaxis prior to invasive dental procedures. Of the 43 participants, 19 (44%) had echocardiographic evidence of valvular pathology; however, only seven (16%) had a physical exam consistent with pathologic valve anatomy or function. Because of the high percentage of SLE patients with valvular abnormalities, and the poor sensitivity of the physical exam, referral to a cardiologist for echocardiography is suggested prior to invasive dental care for patients with SLE. If cardiac valvular pathology is detected, antibiotic prophylaxis should be considered.

Adolescent↗
PubMed1995

[Thyroid involvement in systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is a rare disease in childhood, and is characterized by widespread inflammation of blood vessels and connective tissue. Although the disease affects a number of different organs, thyroid involvement is not included in the classification criteria set of SLE. We describe two cases of irls with SLE who developed thyroiditis with goitre, thyroid autoantibodies, elevated serum TSH and decreased thyroid function tests. One patient had thyroiditis eighteen months before SLE was diagnosed and the other developed thyroiditis six months after the onset of SLE. Recent prospective studies have shown that thyroid involvement in SLE presenting either as hyper- or hypothyroidism is more common among children than adults. We therefore recommend that thyroid function tests should regularly be performed in juvenile SLE patients and, conversely, that child patients with Hashimoto's thyroiditis should be examined for symptoms and serology of SLE.

Adolescent↗
PubMed1986

The association between the lupus anticoagulant and cerebral infarction in systemic lupus erythematosus.

The lupus anticoagulant (LAC) is associated with the occurrence of thromboembolic complications. Assuming that thrombosis may underlie manifestations of the central nervous system (CNS) in patients with systemic lupus erythematosus (SLE), we studied 20 patients with SLE and CNS manifestations for the presence of LAC. In 8 patients (40%) including 4 with overt cerebral infarction, LAC was demonstrated. The 4 patients with LAC and cerebral infarction all had thrombocytopenia, 2 had a history of peripheral thrombosis, and one recurrent abortion. In the 4 LAC-positive patients without overt cerebral infarction, thrombocytopenia was present in 3, a history of thrombosis in 2, and fetal wastage in one. We conclude that LAC identifies within the CNS-SLE group a subpopulation of patients in whom CNS manifestations are caused by cerebral infarction. This subpopulation is further characterized by increased prevalence of thrombocytopenia, peripheral thrombosis and fetal wastage. A possible pathogenetic role of LAC may be related to a hypercoagulable state occurring in this subgroup of SLE patients.

Adolescent↗
PubMed2006

The epigenetic face of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an archetypical systemic, autoimmune inflammatory disease characterized by the production of autoantibodies to multiple nuclear Ags. Apoptotic defects and impaired removal of apoptotic cells contribute to an overload of autoantigens that become available to initiate an autoimmune response. Besides the well-recognized genetic susceptibility to SLE, epigenetic factors are important in the onset of the disease, as even monozygotic twins are usually discordant for the disease. Changes in DNA methylation and histone modifications, the major epigenetic marks, are a hallmark in genes that undergo epigenetic deregulation in disease. In SLE, global and gene-specific DNA methylation changes have been demonstrated to occur. Moreover, histone deacetylase inhibitors reverse the skewed expression of multiple genes involved in SLE. In the present study, we discuss the implications of epigenetic alterations in the development and progression of SLE and how epigenetic drugs constitute a promising source of therapy to treat this disease.

Animals↗
PubMed2006

[Current diagnosis of cutaneous lupus erythematosus].

Cutaneous lupus erythematosus (CLE) is a disease with different subtypes and the new classification system includes acute CLE (ACLE), subacute CLE (SCLE), chronic CLE (CCLE), and intermittent CLE (ICLE). The broad spectrum of skin involvement and the possibility of systemic organ manifestations at the beginning and during the course of CLE require specific diagnostic procedures. Clinical assessment of the cutaneous manifestations is necessary along with a detailed patient's history. The diagnosis of CLE is confirmed by histopathology and immunofluorescence microscopy. Selective laboratory screening and additional diagnostic procedures depending on clinical symptoms are recommended. Photoprovocation tests can be performed to assess photosensitivity in patients with CLE and to support the diagnosis. Recently, a scoring system for the activity of the cutaneous manifestations in CLE has been developed and is now evaluated in several clinical studies. In this review, the classification and the characteristic clinical criteria of the different CLE subtypes as well as the current diagnostic possibilities are emphasized.

Diagnosis, Differential↗
PubMed1985

Opera-glass deformity and tendon rupture in a patient with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a disease entity that frequently becomes clinically evident as a generalized arthritis. Despite this common presentation, it is widely accepted that the arthritis of SLE rarely produces severe joint deformity with barely 10% of the patients developing swan neck, boutumiere deformity or a Jaccoud-like arthropathy. An even lesser proportion of patients develop joint erosions and there have been several clinical reports of tendon rupture in patients suffering from the above condition. However, there is still a great deal of controversy concerning the aetiology of the tendon rupture in these patients and while some authors suggest that corticosteroid therapy influences the outcome of this syndrome, pathology studies were included in a small number of these case reports. The subject of the present case report is a patient with SLE and resorptive arthropathy who suffered repeated episodes of tendon ruptures. The local pathologic findings in this patient suggest that the basic disease process was the cause of the tendon ruptures.

Female↗
PubMed1999

Biologic agents and innovative interventional approaches in the management of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a difficult disease to study, having a variable disease course characterized by exacerbations and remissions. A variety of biologic agents are under investigation as potential treatments for SLE. These products are designed to specifically interfere with the following immunologic processes: T cell activation/T cell-B cell collaboration, production of anti-double-stranded DNA antibodies, deposition of anti-double-stranded DNA antibody complexes, complement activation, and immune complex deposition and cytokine activation and modulation. More aggressive interventions include gene therapy and stem cell transplantation. Immunomodulatory agents recently examined in patients with SLE include: thalidomide, AS101, 2' chlordeoxyadenosine, mycophenolate mofetil, and bindarit. Additional innovative pharmaceutical treatments include the mild androgen dehydroepiandrosterone, selective estrogen receptor modulators, and the prolactin inhibitor, bromocriptine. A variety of these biologic and pharmaceutical agents offer promise as potential therapies. Active participation in clinical trial efforts to develop international consensus regarding trial methodology and outcome measures are crucial to the development of these innovative therapies.

Animals↗
PubMed2003

B lymphocytes and systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterized by B cell hyperactivity in association with autoantibodies, most prominently those directed to components of the cell nucleus. The source of the antigens that drive B cell responses in SLE is unknown, although recent studies suggest mechanisms by which the self-antigens become immunogenic and stimulate responses. Among these mechanisms, abnormalities in the generation of apoptotic cells or their clearance may increase the availability of nuclear antigens to drive responses. In addition, autoantibody crossreactivity may promote induction of responses to disparate antigens, foreign and self, and enable a single autoantibody to cause disease by crossreactive binding. In addition to reflecting increased exposure to self-antigen, autoantibody responses in SLE may result from abnormalities in B cell signaling and regulation by cytokines. New approaches to therapy aim to abrogate autoantibody production by targeting specific steps in B cell activation, including blockade of T cell costimulation.

Antibodies, Antinuclear↗
PubMed2006

Autoantibody determination in the diagnosis of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease that usually develops in young women aged 18-50 years and is characterized by the presence of autoantibodies. Diagnosis is difficult as SLE is a great imitator of other diseases. When SLE is suspected clinically in a patient (involvement of two or more organ systems), an initial laboratory evaluation would be antinuclear antibody (ANA) testing. If ANA is negative, SLE is unlikely and results positive at less than 1:40 strongly argue against SLE. Other explanations for organ system involvement should be pursued. Results positive at greater than 1:40 may merit further evaluation for SLE and at times referral to a rheumatologist for a full SLE evaluation. While the American College of Rheumatology classification criteria for SLE are primarily a tool for research, they may be useful clinically, in that those patients fulfilling four or more criteria are highly likely to have SLE.

Antibodies, Antinuclear↗
PubMedDate not supplied

Imaging findings in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an unusually complex autoimmune disease that is encountered in every radiology subspecialty because of its multisystem involvement and the wide age range of affected patients. There are no universally accepted diagnostic imaging criteria for SLE, and in fact, many SLE patients present with systemic findings and laboratory abnormalities and do not require imaging. Nevertheless, radiology plays an ancillary role in the diagnosis and management of this often insidious disease, and knowledge of the spectrum of radiologic findings in SLE and its complications is crucial for proper image interpretation. Imaging is often performed in patients with a known diagnosis of SLE to determine the extent and severity of disease, which depend on the extent of organ involvement, and to monitor complications. In addition, imaging may be important in selected patients with diseases such as pneumonia who present with atypical symptoms due to immunosuppressive therapy.

Adolescent↗
PubMed2002

Treatment options for juvenile-onset systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an inflammatory chronic disease characterized by the presence of activated helper T-cells that induce a B-cell response, resulting in the secretion of pathogenic autoantibodies and the formation of immune complexes. SLE in children is a disease of low prevalence with a wide range of clinical manifestations, which means that the number of randomized controlled studies are few and usually involve a small number of patients. In recent years, new therapeutic agents have appeared and the role of older treatments has been clarified. Many of these treatments are designed to reduce inflammation. The spectrum is broad and ranges from traditional nonsteroidal anti-inflammatory drugs (NSAIDs) to cytotoxic agents that have anti-inflammatory effects. The current treatment of children or adults depends on the clinical expression of the disease. Minor manifestations usually respond to the administration of NSAIDs, low doses of corticosteroids, hydroxychloroquine, or methotrexate. Thalidomide could be used for refractory skin lesions. Major manifestations can endanger the patient's life and require early, aggressive treatment. Kidney disease and other manifestations have been related to the formation or deposit of tissular immune complexes. Therefore, for years the main aim of treatment has been to suppress the immune response. The immunosuppressant treatments used in children with SLE include high doses of corticosteroids, azathioprine, methotrexate, cyclosporine, and cyclophosphamide. Several combinations of medications have been used to obtain a rapid remission or to reduce the risk of toxicity of prolonged administration of cytotoxic agents. Intravenous gamma-globulin has been successfully used in the treatment of lupus nephritis, vasculitis, and acute thrombocytopenia. In spite of numerous published studies, the use of these drugs is still controversial. The immunosuppression achieved with these treatments is nonspecific, not always effective, and associated with significant toxicities; the most significant being growth retardation, accelerated atherosclerosis and severe infectious complications. The purpose of new biological therapies is to achieve specific immunosuppression, which makes it possible to design more effective and less toxic therapeutic strategies. Mycophenolate mofetil is a promising alternative in patients who do not respond to high doses of cyclophosphamide or azathioprine. Some recently developed monoclonal antibodies such as anti-CD40L or anti-IL-10, or other molecules such as LJP394 may prove useful in the near future. Finally, stem cell transplantation may be proposed in patients with severe juvenile-onset SLE who do not respond to any treatment.

Adrenal Cortex Hormones↗
PubMed1989

[Acute massive mediastinal hemorrhage three weeks after mitral valve replacement in a patient with systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is one of the most common autoimmune diseases and patients suffering from this disease often died of massive hemorrhage. We report the case of a patient who died of acute massive hemorrhage three weeks after mitral valve replacement. The patient, a 42 year-old woman, had been diagnosed as having valvular heart disease at the age of 10. She underwent mitral commissurotomy at the ages of 18 and 32. SLE was diagnosed 8 years previously and corticosteroid therapy was initiated. The patient was experiencing exertional dyspnea again 1 year ago and mitral valve replacement was performed for recurrent stenosis. The postoperative course seemed to be uneventful, but on the 21st postoperative day, acute massive mediastinal hemorrhage occurred, and the patient eventually died of septicemia. Massive hemorrhage in SLE patients usually occur in the central nervous system or alimentary tract. However, bleeding can occur anywhere, so great care must be taken in regulating anticoagulant therapy.

Adult↗
PubMed2007

Enhanced oxidative status but not corresponding elevated antioxidative status by anticardiolipin antibody and disease activity in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is associated with premature atherosclerosis, and an increased susceptibility of plasma lipids and lipoproteins to oxidation could at least in part promote atherogenesis. The objective of this study was to identify the effects of anticardiolipin antibody (aCL) and disease activity on oxidative and antioxidative status in patients with SLE. In this study, patients in SLE/aCL+ group and in SLE disease activity index >3 group had significantly higher thiobarbituric acid reactive substance (TBARS) levels, titers of autoantibodies against oxidized low-density lipoprotein (ox-LDL), and glutathione peroxidase activity than that of healthy controls. However, only TBARS and titers of autoantibodies against ox-LDL but not antioxidant enzyme activities were significantly different between SLE subgroups. Thus, enhanced oxidative status but not corresponding elevated antioxidative status by the presence of aCL and active disease activity make potent antioxidant therapy valuable for these SLE patients in preventing oxidative damage.

Adult↗
PubMed2004

Trace elements and some extracellular antioxidant proteins levels in serum of patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic progressive autoimmune disorder with a wide spectrum of clinical and immunological abnormalities. In this study, we aimed to investigate the levels of serum zinc (Zn), copper (Cu), magnesium (Mg), manganese (Mn), iron (Fe), ceruloplasmin (Cp), transferrin (Trf), and albumin (Alb) in SLE and whether it is related to the severity of the clinical condition of this chronic disease. Cp and Cu levels were higher, while Trf, Alb, Zn, Mg, Mn, and Fe levels were lower in serum of patients with SLE (n = 27) compared with healthy controls (n = 20). The mechanisms by which these alterations occur in certain inflammatory conditions need to be elucidated. It is also obscure whether these alterations are a cause or a consequence of the inflammation. As a conclusion, alterations in the levels of the parameters in SLE may not be a reason for, but in fact a consequence of the disease itself.

Adolescent↗
PubMed2004

Stem cell transplantation in systemic lupus erythematosus.

Systemic lupus erythematosus is a heterogeneous, multisystem disease responsive to treatment with corticosteroids and immune suppressives. Many patients fail to achieve treatment-free remissions, and their long-term outcomes remain poor owing to the development of vital organ failure, cumulative drug toxicity and an increased risk of cardiovascular disease and malignancy. Haematopoietic stem cell transplantation (HSCT) offers the potential to improve long-term outcome in those with a poor prognosis. Preliminary phase II and registry studies have usually employed non-myeloablative conditioning with positive CD34 cell selection. They have highlighted the potential efficacy and dangers of HSCT. Patient selection is important but complex, and the influence of HSCT on long-term outcome is unknown. Disease relapse occurs in up to one-third of patients after HSCT, but the consequences of relapse and the role of remission-maintenance strategies are unknown. With the availability of other alternative therapies in refractory disease, there needs to be a clear demonstration of the benefits of HSCT from current randomized trials.

Bone Marrow↗