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Systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a complex multisystem autoimmune disease of unknown aetiology that is subject to period of exacerbation and remission. Treatments aimed at controlling disease activity remain primarily by immunosuppression, the prescribing of which is dependent on the development of any multisystem complications. With the establishment of dedicated lupus clinics using a multidisciplinary team approach, prognosis is better and treatment options continue to improve.

Female↗

An unusual presentation of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem connective tissue disease caused by the damage of tissues and cells by pathogenic auto-antibodies and immune complexes. A 27-year-old female presented with chronic diarrhoea was diagnosed as intestinal tuberculosis. But further evaluation diagnosed it a case of SLE and diarrhoea subsided with treatment. The case is reported because of its atypical presentation.

Adult↗

Aortic aneurysm in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is frequently associated with cardiovascular manifestations, but rarely complicated with aortic disease. We report a 28-year-old female patient with a 14-year history of SLE and a 3-year history of hypertension. She had suffered from palpitation and chest tightness for 1 month before admission. Heart echo showed thoracic to low abdominal level with low flow. A computed tomography (CT) scan confirmed aneurysms of the descending thoracic and upper abdominal aorta, down to the renal level. Diagnosis of aortic aneurysm should be considered in patients with SLE, especially those who have a history of hypertension, prolonged steroid use, palpitation and chest pain. Current imaging modalities, such as cardiac echo, CT and magnetic resonance angiography may provide earlier detection of subclinical disease, which may aid in preventing these fatal complications. It is important to control hypertension aggressively in patients with SLE. In addition to decreasing steroid doses, early use of immunosuppressive agents and accurate noninvasive image modalities may allow us to prevent severe damage to the aorta and avoid the fatal complications.

Adrenal Cortex Hormones↗

Analysis of the apolipoprotein(a) size polymorphism in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is characterized by an increased incidence of vascular disease which is only partially explained by traditional risk factors. Previous reports suggested that the level of lipoprotein(a) [Lp(a)], a particle linked to atherothrombotic disorders, is increased in patients with SLE. However, whether there are any differences in the distribution of apolipoprotein(a) [apo(a)] phenotypes between SLE patients and healthy controls remain to be determined. To address this issue, Lp(a) levels and apo(a) isoform size were analyzed in a total of 54 patients with SLE and in 108 age- and gender-matched healthy controls. SLE patients showed Lp(a) levels [median (interquartile range): 25.3 (6.5-51.0) vs. 9.5 (4.6-25.9) mg/dl, P=0.0109)] and a percentage of subjects with at least one small-sized apo(a) isoform (< or =25 K-IV repeats) significantly higher than controls (44.44% vs. 25.92%, P=0.0277). Multiple regression analysis adjusting for age, gender, disease duration, kidney involvement, the presence of active disease, as well as the carriage of at least one small apo(a) isoform revealed that only small apo(a) phenotypes were significant predictors of Lp(a) levels in SLE patients (P=0.0001). We conclude that genetic factors related to apo(a) size are a major determinant of elevated Lp(a) levels in patients with SLE. As small apo(a) phenotypes have been related to adverse vascular effects, it is feasible that small apo(a) isoforms may be a useful biological marker in the assessment of vascular risk in patients with SLE.

Adult↗

Systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease that exhibits a wide range of clinical symptoms, including skin rashes, oral ulcers, arthritis, pulmonary pleuritis, anemia and seizures. Medical imaging plays a role in assessing the extent of disease, neurological involvement, treatment complications and disease progression. This article describes the known risk factors for SLE and SLE diagnosis and treatment.

Humans↗

Assessment of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is the archetypal autoimmune disease given its complex clinical and molecular manifestations. Like the other rheumatic diseases, appropriate management is critically dependent upon the proper assessment of disease activity, organ damage, and quality of life. Here, we describe the components of the comprehensive assessment of SLE, including accurate physical and laboratory diagnosis, monitoring of disease activity, recording of accumulated organ morbidity, and integration of these with the patient's own perceptions of health status and quality of life. In doing so, we will review the most appropriate laboratory tests and indices currently used in standard clinical care and in clinical research.

Disease Progression↗

Systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a syndrome commonly affecting young women. The clinical manifestations are extremely varied and any major organ of the body may be involved. Misdiagnosis is not uncommon in early SLE where symptoms and signs may be few. Auto-antibodies to DNA, RNA and other cell nucleus antigens are frequently present. Circulating immune complexes may deposit in major organs, causing inflammation and tissue damage by a number of mechanisms. The lupus disease is marked by exacerbations and remissions. Management is dependent on accurate assessment of clinical activity and severity. Patient education and co-operation in management affect outcome of the disease. With good management, the ten year survival may exceed 90%.

Female↗

Painless massive ascites and hypoalbuminemia as the major manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is frequently associated with ascites, but rarely without proteinuria. We report a 10-year-old girl with distended, non-tender abdomen with shifting dullness and no pitting edema in the lower legs before admission. Facial rash had appeared 1-2 weeks before admission and became more prominent 3 days prior to admission. Hypoalbuminemia with hypertriglycemia (but no proteinuria or diarrhea) was noticed. The antinuclear antibody titer was 1:2560 (speckle type) and the anti-double-stranded DNA was 1:160. Abdominal echo revealed no cirrhosis change or venous obstruction. Chest X-ray and electrocardiogram revealed no cardiomegaly or pericardial effusion. The serum prealbumin was low on admission day 5, but the liver function tests were within normal range. We deduced that the hypoalbuminemia in SLE without nephritis may be secondary to mesenteric vascular leakage. SLE may present with initial manifestation of painless massive ascites. Careful utilization of history taking, chest X-ray, electrocardiogram, cardiac and abdominal echo, urinary analysis and serum prealbumin is helpful in decision-making while assessing such patients.

Ascites↗

[Clinical features of Pneumocystis pneumonia in patients with systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is often associated with various opportunistic infections, particularly during treatment with corticosteroids or immunosuppressants. We studied the clinical characteristics of 15 patients with SLE who underwent diagnostic bronchoalveolar lavage (BAL) and compared 6 patients with confirmed Pneumocystis pneumonia (PcP+), with 9 patients without Pneumocystis pneumonia (PcP-). The serum concentrations of beta-D-glucan and KL-6 were significantly higher in PcP+ than in PcP- patients, whereas serum LDH was similar in both groups. The serum concentrations of complement, a marker of SLE activity, and of IgG did not predict the presence of PcP. In all patients, the overall cell and lymphocyte counts were increased in the BAL fluid, without any significant difference between the PcP+ and PcP- groups. Ground-glass opacities on chest computed tomography, and oxygenation impairment (PaO2/FiO2<200Torr) were more common in PcP+ than PcP- patients. We concluded that, in patients with SLE, serum beta-D-glucan and KL-6 might be useful in the diagnosis of PcP, particularly when severe hypoxemia precludes BAL.

Adult↗

Interferon inhibitor in the blood of patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) patients at advanced stages of the disease have an interferon inhibitor in the blood circulation. This inhibitor can block antiviral activity of all three types of human interferons and can significantly reduce the synthesis of interferon alpha by the treated lymphocytes obtained from normal healthy individuals. Available evidence suggests that inhibitor activity is neither because of the antibody to interferon nor due to high level of protease-like activity in the plasma. The inhibitor has also been shown to be effective in eliminating the interferon-mediated enhancement of natural killer cell activity. Interferon inhibitory activity was not detected in any of the sera taken from normal healthy individuals. Identification and characterization of interferon inhibitor has direct bearing upon effective utilization of interferons in the clinic.

Animals↗

Liver lymphoma in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) has been associated with an increased incidence of lymphoma. We describe the occurrence of hepatic lymphoma, which was likely primary in origin, in a patient with SLE and discuss the etiologic and diagnostic implications.

Aged↗

Pedal manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with numerous pedal manifestations. Since pedal manifestations are often its first presentation, it is important that the podiatrist knows how to recognize the disease. This article discusses the manifestations, diagnosis, and treatment of SLE as they relate to podiatry.

Foot Diseases↗

Autoantibodies in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease in which the predominant autoantibodies are antinuclear antibodies (ANA) reactive with DNA and histones, and antibodies reactive with the non-histone extractable nuclear antigens (ENA), Sm and Ro. Racial differences demonstrable in predisposition to SLE are also evident in the prevalence of autoantibodies, the frequency of anti-Sm and anti-Ro being 2-4 times higher in Asians with SLE than in Caucasians with SLE. Autoantibodies have also played a role in the classification of lupus and the recognition of multisystem autoimmune diseases that fail to meet the classical criteria for the identification of patients with SLE but appear to be variants of lupus. Anti-Ro is a diagnostic marker for subacute cutaneous lupus, anti-(U1)RNP a marker for mixed connective tissue disease, antibodies to phospholipids a marker for the syndrome comprising stroke, fetal wastage, thromboembolism and thrombocytopenia and antibodies to histones a marker for lupus induced by the drugs hydralazine and procainamide. There is still no unaminity on whether these antibodies play an integral role in the disease process or whether they are "epiphenomena". The challenge for research in the 1980s is the understanding of the relationship of these antibodies to the pathogenesis of SLE.

Antibodies, Antinuclear↗

Diminished response to an inhibitory signal in lymphocytes from patients with systemic lupus erythematosus.

Systemic lupus erythematosus is an autoimmune disease characterized by B-cell hyperactivity, resulting in polyclonal hypergammaglobulinaemia. One mechanism potentially resulting in excessive immunoglobulin synthesis is a diminished response to inhibitory signals. To test this hypothesis, anti-IgG antisera was used to inhibit pokeweed mitogen activation of cultured lymphocytes from lupus patients and controls. Inhibition of IgG secretion by B cells from lupus patients required more than 75 times as much anti-IgG as normal controls (P less than 0.005), indicating that lupus lymphocytes are hyporesponsive to this inhibitory signal. Similar studies with DR3+ controls demonstrated that diminished responsiveness to anti-IgG inhibition may in part be associated with the HLA-DR3 allele. Defects in this inhibitory mechanism may play a role in the B-cell hyperactivity observed in lupus.

Antibodies, Anti-Idiotypic↗

Endomyocardial biopsy in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) has numerous cardiovascular manifestations. Myocarditis is present in more than 50% of patients with SLE at autopsy, but it may be silent clinically during life. Percutaneous endomyocardial biopsy performed in 4 patients with SLE was found helpful in establishing the diagnosis and in determining the extent of the inflammatory myocarditis. The findings aided the regulation of therapy, particularly the administration of immunosuppressive drugs.

Adult↗

Animal models of human systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a human autoimmune disease of unknown etiology. Clinical, serologic, immunologic, and pathologic findings are highly variable in different patients and at different times in the same patient. Murine and canine animal models of SLE have been found with clinicopathologic abnormalities resembling those observed in humans. Each animal model has unique characteristics; taken together they reflect the spectrum of disease in human SLE.Investigations in the animals have suggested that genetic, hormonal, immunologic, viral, and other environmental factors contribute to and modify the expression of disease. Where analogous studies are available for humans, the same factors have been found to modify disease expression in a similar fashion. Together, these studies have helped to clarify the multifactorial basis for SLE.The best characterized abnormalities are immunologic. These include excessive B cell function with the formation of large amounts of autoantibodies, and T cell abnormalities which include defects in T cell regulatory function as well as certain T cell effector functions.The animal models of SLE also serve as convenient test subjects for newer therapeutic modalities. It is hoped that further study of the animal models will provide a more rational approach to therapeutic modulation of disease in humans with SLE.

Animals↗

Recent advances in the immunopathogenesis of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disease having definite etiologic associations with ethnic, genetic, viral and immunologic factors. Its pathologic hallmark, vasculitis, is currently felt to be the end result of an immune-complex mechanism. Several clinical and serologic variants of SLE are recognized including discoid lupus erythematosus (DLE), mixed connective tissue disease (MCTD) and drug-induced equivalents-such as procainamide-induced lupus (PIL). The distinguishing features of these variants as well as their prognosis and therapy are discussed in relation to recent developments in the immunopathogenesis of SLE.

Animals↗

Hemolytic anemia and thrombocytopenic purpura: two related subsets of systemic lupus erythematosus.

Systemic lupus erythematosus patients who develop hemolytic anemia or thrombocytopenic purpura differ from other lupus patients and are similar enough to be considered two related subsets with a more benign course. Thirty-one lupus patients with either or both these hemocytopenias were found to be significantly younger, more often males, and had less frequent fever, polyarthritis, serositis, cutaneous vasculitis, nephropathy, neurologic manifestations, and persistent hypocomplementemia than 62 lupus patients without any of these hemocytopenias. They also had lower index scores of overall disease severity and required less treatment. It seems important to subdivide lupus patients in subsets for therapeutic and prognostic purposes.

Adolescent↗