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PubMed2005

Quality of life in children with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) in children is a chronic multi-system disease with wide ranging effects on their quality of life (QOL). While SLE's impact on different arenas of life and well-being has been extensively examined in the adult population, its effect on children has not received adequate attention. This paper briefly discusses the multidimensional aspect of QOL, the biopsychosocial implications of SLE, factors complicating QOL measurement in the affected population, and the different generic and disease-specific scales used for measuring QOL and related constructs, and it also highlights the need for SLE-specific pediatric QOL instruments.

Adaptation, Physiological↗

PubMed2001

Middermal elastolysis in two patients with lupus erythematosus.

Despite lupus erythematosus (LE) being considered a "connective tissue disease," little has been written about the elastic fiber changes in the skin of affected patients. We report our histologic findings in two patients with unusual cutaneous lesions. Elastic fiber loss was noted, and scattered giant cells with elastic fiber phagocytosis were prominent in one patient. The findings are similar to those described for middermal elastolysis. Other authors have reported patients with LE and elastic fiber loss resembling anetoderma. We believe that a spectrum of elastic fiber changes can occur in patients with LE and may be induced by infiltrating lymphocytes and/or circulating antibodies.

Adult↗

PubMedDate not supplied

Partial C4 deficiency in two children with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE.

Adolescent↗

PubMed1999

Cardiac involvement in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE), a connective tissue disease characterized by the production of autoantibodies, can affect all organ systems. Cardiac involvement in patients with SLE has been described since the early 20th century. The manifestations are numerous and can involve all components of the heart, including the pericardium, conduction system, myocardium, valves, and coronary arteries. In recent years, echocardiography has yielded additional information about the heart in patients who have SLE with and without clinical cardiac involvement. Moreover, antiphospholipid antibodies have been linked to several cardiac manifestations in patients with SLE, including valvular abnormalities and possibly coronary artery disease. This updated, comprehensive review summarizes the new literature on SLE and the heart.

Antibodies, Antiphospholipid↗

PubMed1997

Recognition and management of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an inflammatory systemic disease that causes organ damage by the deposition of autoantibodies and complement activating immune complexes or by vascular occlusion due to procoagulant states associated with antiphospholipid antibodies. The vast majority of cases occur in women of childbearing age. SLE is diagnosed on the basis of its clinical manifestations and the demonstration of characteristic immunological phenomena, especially anti-nuclear antibodies. The prognosis in SLE has shown a distinct improvement over recent decades, the 5-year survival rate now approaching or exceeding 90%. The 15-year survival rate of 63 to 79%, on the other hand, underscores the need for further advances in diagnosis and treatment of the disease. Management of the disease includes regular monitoring of disease activity, avoidance of predisposing factors and close supervision of therapy. Drug therapy is guided by the activity and severity of the leading organ manifestations and ranges from nonsteroidal antirheumatic drugs to intensive treatment with cytotoxic agents. Corticosteroids remain irreplaceable for the control of acute flares. Antimalarials and azathioprine are important long term drugs for treating mild or moderate disease activity. Intravenous pulse cyclophosphamide is safer than other regimens and at least as effective as oral cyclophosphamide for severe lupus nephritis. It is also effective in the treatment of central nervous disease and of other organ-threatening manifestations. Recently, an intensified protocol which included cyclophosphamide induced long term treatment-free remission in 60% of patients. The toxicity of cyclophosphamide is considerable, but can be ameliorated by various measures. The value of several new immunosuppressants and other compounds remains to be determined.

Adrenal Cortex Hormones↗

PubMed2005

[Diagnostic problems and evaluation of disease activity in systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory disorder characterised by autoantibodies production. The diagnosis of disease requires evidence of 4 out of 11 clinical and/or laboratory symptoms proposed by American College of Rheumatology. For many years attention has been drawn to the problem of finding a valid and sensitive tool for measuring SLE activity and more than 60 different methods have been proposed. Unfortunately none of them has been generally accepted. Amongst the most frequently used are SLEDAI scale and its modifications, BILAG and SLAM. The systems have been validated in prospective studies, and their reproducibility, validity and sensitivity have been compared. At present they are widely used in clinical practice. In this review currently accepted methods of SLE activity evaluation are presented.

Diagnosis, Differential↗

PubMed2002

The neuropathology and pathogenesis of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease in which neuropsychiatric involvement occurs in about 50% of patients and carries a poor prognosis. Despite extensive research, the precise mechanisms of nervous tissue injury remain the least well understood. This article summarizes the important clinical neuropsychiatric features of SLE and, reviewing classical histopathological and more recent experimental studies, discusses theories concerning their presumed pathogenesis. The distinctive production of diverse autoantibodies seems to be related to defective clearance of apoptotic cells. Antibody-mediated neural cell injury and rheological disturbances represent the two principal suggested mechanisms of tissue injury. An interplay between these processes, underlying genetic factors, their modification by hormones, complicated by a number of secondary factors, may explain the wide spectrum of features encountered in this disease.

Autoantibodies↗

PubMed2005

Illness representations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic illness that usually follows a fluctuating course and, therefore, can be difficult to diagnose. Consequently, individuals with SLE often hold beliefs about the nature and course of the illness that are at odds with medical opinion. Clearly, a shared understanding of the illness would be beneficial to illness management. In this study, the authors used semistructured interviews and interpretative phenomenological analysis to explore the content of the illness representations held by 36 individuals diagnosed with SLE. They also identified the ways in which these illness representations were generated initially and attempted to determine whether changes occurred over time in these illness representations, as predicted by the self-regulatory model.

Adaptation, Psychological↗

PubMed1999

Degradation of tryptophan in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE), a progressive autoimmune disorder, is associated with chronic stimulation of various components of the immune system. Since cell-mediated immunity is also activated, we were interested to test for abnormalities in tryptophan metabolism in SLE which may result from activation of indoleamine 2,3-dioxygenase by cytokines released during the immune response. We measured serum tryptophan and kynurenine concentrations in 52 patients with SLE as well as serum neopterin as an indicator for the degree of immune activation. Compared to controls, we found significantly decreased tryptophan and increased kynurenine concentrations in SLE. The extent of tryptophan catabolism correlates with neopterin concentrations or with the disease activity index. Tryptophan depletion may be associated with neurologic/psychiatric disturbances in patients suffering from SLE.

Depression↗

PubMed1995

[Hematologic problems in systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) remains a disease of unknown origin, characterized by major alterations of both the cellular and the humoral arms of immunity. Hematological changes, including anaemia, leucopenia and thrombocytopenia, occur in more than one half of patients with this disease. Anaemia is the most common hematological abnormality seen in SLE. Its possible causes are anaemia of chronic disease (ACD), auto-immune haemolytic anaemia and hypoplastic anaemia. Leucopenia affects both granulocytic and lymphocytic lines and may be caused by autoantibodies. The influence of drugs, hypersplenism and marrow suppression are also possible. Thrombocytopenia occurs frequently and is almost invariably autoimmune. Patients with SLE are at increased risk of thrombosis. Haematological abnormalities in patients with this disease require careful long-term monitoring and prompt therapeutic intervention.

Autoimmune Diseases↗

PubMed1992

Primary total hip arthroplasty in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease affecting primarily young women. Osteonecrosis of the femoral head produces significant morbidity in these patients. The clinical and radiographic results of 33 total hip arthroplasties (THA) in 25 patients were studied. The Hospital for Special Surgery hip rating was good or excellent in all surviving index primary hip arthroplasties at a median follow-up of 47 months. Overall survival probability was 94.6% at 5 years and 81.8% at 9 years using survivorship analysis. Perioperative morbidity was minimal. Total hip arthroplasty using contemporary techniques and current management protocols can provide reliable functional improvement in patients with osteonecrosis secondary to SLE.

Adult↗

PubMed2001

A case of hypothermia combined with systemic lupus erythematosus.

Systemic lupus erythematosus(SLE) is a prototypic autoimmune disease affecting various organ systems. Hypothermia is a rare manifestation of SLE. We experienced a case of SLE combined with hypothermia. A 36-year-old woman, who had been diagnosed as SLE 3 days before admission, admitted complaining of mental confusion. After admission, her body temperature, initially 36.1 degrees C, became 32.6 degrees C. Her core body temperature was less than 35.0 degrees C. Despite of warming with heating lamp and blankets, her core temperature did not reach 35.0 degrees C during 18 hours. Ten days later, her temperature exceeded 36.0 degrees C.

Adrenal Cortex Hormones↗

PubMed1992

[An update on the clinical and pathogenetic value of a lymphocyte subpopulation study in systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is an entity with multiple clinical signs due to a basic immunoregulation disorder with an unbalance between humoral and cellular components of the immunological response. Its course of evolution is characterized by frequent exacerbations and remissions and the search for new activity markers of the disease is an ongoing activity. During the past years, phenotypical studies of the immunological system cells have been incorporated to the effort of establishing clinical-immunological correlations and obtaining objective parameters useful for the clinical and therapeutical management of a disease as variable as the SLE. Several disorders among lymphocitary populations have been described in the SLE; however, the published data are sometimes controversial, given the clinical and serological heterogenicity of the disease.

B-Lymphocytes↗

PubMed1991

Immunoglobulin heavy chain constant-region gene polymorphism in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is associated with immunoglobulin allotypes in several ethnic groups. In this study, we investigated the association of a Bst EII immunoglobulin heavy chain constant-region gene restriction fragment length polymorphism with SLE in patients from the US and Mexico. A 3-kb restriction fragment was observed with significantly decreased frequency in randomly selected Mexican SLE patients and in Mexican SLE patients in multiplex families. However, no such association was observed in SLE patients from the US. Thus, the absence of a 3.0-kb Bst EII restriction fragment from immunoglobulin heavy chain constant-region genes provides a marker for SLE in Mexican individuals.

Blotting, Southern↗

PubMed2006

Roles of genetic variations in signalling/immunoregulatory molecules in susceptibility to systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a complex genetic basis that includes many susceptibility genes on multiple chromosomes. As complex human diseases like SLE involve multiple, interacting genetic and environmental determinants, identifying genes for complex traits is challenging and has had limited success so far. However, recent advances in genetic resources and technology have been providing new tools to identify the novel pathways or the sequence variants that contribute to autoimmune diseases. During the past several years, several new candidate genes have been implicated in development of SLE though association studies. In this article we describe an overview of the latest findings in the genetics of SLE, especially focusing on the genetic variations in the signalling or immunoregulatory molecules including CD28 and IRF family members.

Animals↗

PubMedDate not supplied

The bite of a wolf: systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown etiology that occurs in people who have an aberrant immune system; it affects most major organ systems. People with SLE vacillate between periods of remission and exacerbation, with symptoms of fatigue and chronic pain, and experience disturbances in self-concept. SLE no longer has the high mortality rate of past years due to improved diagnostic tests that allow for earlier detection and treatment. SLE is a complex disease that affects the individual and the family. Rehabilitation nurses should use a holistic approach to treat altered functional ability and help individuals adjust to lifestyle changes associated with chronic illness and/or disability. Nurses with specialized rehabilitation knowledge and skill can help individuals with SLE realize and reach their optimal level of functioning, and thus improve their quality of life.

Female↗

PubMedDate not supplied

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↗

PubMed1983

[Biological diagnosis of systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) is a polymorphic disease of unknown origin, characterized by many stigmata of autoimmunization which allow a biological diagnosis. Besides non-specific inflammatory disorders, considerable alterations of humoral and cellular immunity are found. The hallmark of the disease is the presence of anti-double-stranded-desoxyribonucleic acid (anti-ds DNA) antibodies, detected by the Farr radioimmunologic method or the crithidia luciliae immunofluorescence technique, both of which are nearly specific for SLE. The clinical significance of other types of anti-nuclear antibodies is unclear. Serial evaluations of antinuclear antibodies, complement fractions, circulating immune complexes, and, above all, immune complexes bound to target organs (kidneys, skin, synovial membranes) are needed to follow the course of the disease. The demonstration of abnormal suppressor T-cell activity has led to current pathogenic hypotheses and ongoing therapeutic trials with immunoregulating drugs.

Biopsy↗