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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↗

PubMedDate not supplied

The selective value of computed tomography of the brain in cerebritis due to systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) and steroid effects on the brain were measured by computed tomography (CT). Of 14 patients with SLE cerebritis, 10 (71%) had marked cortical atrophy and 4 (29%) minimal atrophy. None were normal by CT. Controls included 22 patients with SLE without cerebritis receiving corticosteroids; this group had normal CT scans in 16 (73%) and minimal cortical atrophy in the remaining 6 (27%). Follow-up CT on 5 patients with cerebritis was unchanged. CT of the brain is a minimally invasive technique for documenting SLE cerebritis. CT may also help differentiate cerebritis from the neuropsychiatric side effects of corticosteroids.

Adolescent↗

PubMed1978

Diagnosis and management of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is not a rare disease. There are several common clinical signs which should alert the physician to a possible diagnosis of SLE and which should condition him to look for specific clinical and laboratory findings. In addition to simple screening tests, useful procedures include a search for antinuclear antibodies, lupus erythematosus (LE) cells, anti-DNA antibodies and low serum complement. Management is determined by the type of course encountered but most patients will do well under the care of their family physician.

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↗

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↗

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↗

PubMedDate not supplied

Tremor as an early manifestation of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) has been described by many authors as a multisystem disorder involving variable and protean clinical manifestations and with an unpredictable course. We report the case of a 68-year-old woman suffering from SLE in whom tremor appeared ten years before a clinical picture suggestive of SLE and which remained the only clinical neurological sign even during overt disease. Tremor and other SLE manifestations disappeared with corticosteroid therapy.

Adrenal Cortex Hormones↗

PubMed1993

Why so many women? Part 1. Systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder that predominantly affects women. While numerous factors may account for the etiology of SLE, the influence of gender-based sex hormones on the prevalence of SLE and autoimmune disorders among women is currently being evaluated.

Adult↗

PubMed1993

Tophaceous gout in young patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) and gout have been associated infrequently. We describe 3 young adults with SLE who developed tophaceous gout relatively early in the course of their disease. All were underexcretors of uric acid but were studied after the development of renal disease; 2 were treated with diuretics. In 2 cases, gout became obvious while lupus was quiescent.

Adult↗

PubMedDate not supplied

High levels of TH2 cytokine gene expression in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a clear imbalance in the network made up of different cytokines. However this statement has been derived from studies which have focused on the analysis of some specific cytokines and few have simultaneously analyzed those cytokines that could be involved in the pathogenesis of SLE. Therefore, we decided to analyze interleukin IL-1b, IL-2, IL-4, IL-6, IL-10, tumor necrosis factor-a (TNF-a) and gamma interferon (IFN-g) gene expression in peripheral blood mononuclear cells from 17 women with SLE and 10 normal females by a coupled reverse transcriptase-polymerase chain reaction technique. High gene expression of IL-4, IL-6, IL-10 and TNF-a was found in SLE patients as compared to normal subjects. The expression of IL-1b, IL-2 and IFN-g genes was low or undetectable. The resulting high level of cytokines with strong effect on proliferation and differentiation of B lymphocytes in SLE could be responsible for the characteristic B cell hyperactivity and autoantibody production seen in SLE.

Adolescent↗

PubMed1995

Inhibition of hematopoiesis by a plasma factor in a case of aplastic anemia associated with systemic lupus erythematosus.

Systemic Lupus Erythematosus (SLE) may be associated with inhibition of hematopoiesis mediated by antibodies, T-cells or both. A 41-year-old woman with a five-year history of SLE treated with prednisone was admitted to Cabrini Medical Center in New York. The patient complained of fever, chills, arthralgias, general malaise, weakness and dyspnea on exertion, and showed malar rash, pallor, and a systolic ejection murmur along the left sternal border. Admission work up included a CBC with evidence of moderate pancytopenia, a normal EKG, and a normal chest X-ray. The patient's anemia was symptomatic and required a transfusion of packed red blood cells (PRBC's). Bone marrow biopsy and aspiration revealed an aplastic marrow with few hypoplastic islands of hematopoietic elements. The patient was treated with plasmapheresis, achieving immediate progress towards recovery. Bone marrow culture studies (erythroid BFU-E, and myeloid CFU-GM) were done by incubating various titers of the patient's acute phase plasma with normal bone marrow cells. This was done to determine if the patient's plasma contained any hematopoietic inhibitory activity, as has been reported in other cases. Our experiments demonstrated marked inhibition of erymathropoiesis and myelopoiesis in vitro, when various titers of the patient's plasma were included in the culture media. Control plasma produced no inhibition. These studies support the hypothesis that a circulating antibody which inhibits hematopoiesis may be produced in SLE patients with aplastic anemia, and be responsible for it.

Adult↗

PubMed1996

[Septic arthritis caused by Salmonella enteritidis in systemic lupus erythematosus].

Systemic Lupus Erythematosus (SLE) is among the chronic diseases thought to predispose patients to severe Salmonella infections. However, arthritis and osteomyelitis due to this microorganisms are more frequently seen in patients with sickle-cell disease than SLE. We report two cases of SLE and osteoarticular infections by Salmonella enteritidis: A 36-years old woman with bilateral knee arthritis associated with femoral osteomyelitis and a 22-years-old woman who presented with left knee arthritis.

Adult↗

PubMed1996

[Renovascular hypertension associated with antiphospholipid antibodies in a woman with systemic lupus erythematosus].

Systemic lupus erythematosus (SLE) patients, especially those with antiphospholipid antibodies, have a high incidence of arterial and venous thrombotic manifestations. However, renovascular hypertension (RVH) has been rarely reported in these patients. We describe here a 49-year-old female with antiphospholipid antibodies, complicated with RVH and presenting with sudden onset of severe hypertension, headache and nausea. She had experienced phlebitis and arterial thrombosis of the right leg. At the age of 38 years, she was diagnosed as SLE and steroid therapy was started, but she had poor drug compliance and irregularly visited our clinic. On admission, hypertension was recognized and abdominal bruit was audible on physical examination. Serological findings were compatible with SLE. She was also found to have IgG anti-cardiolipin antibody and lupus anticoagulant. Peripheral plasma renin activity (PRA) was elevated, and captopril test showed hyper-response of PRA with lowering of blood pressure. Renal echography and scintigram showed a small and poorly perfused right kidney. Selective angiography demonstrated a severe stenosis of the right renal artery at origin. A stenosis at the origin of both the superior mesenteric artery (SMA) and celiac trunk was also detected. Percutaneous transluminal angioplasty was performed, achieving successful dilatation of the right renal artery and SMA, whereas the attempt to insert the catheter into the celiac trunk was unsuccessful. After this procedure, abdominal bruit has not been audible. Following the initiation of steroid pulse therapy combined with heparin and dipyridamole, her blood pressure was gradually depressed and the test for lupus anticoagulant became negative. Therefore, RVH of this patient is thought to be associated with antiphospholipid antibodies.

Antibodies, Antiphospholipid↗

PubMed1998

The genetics of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a disease characterised by diverse clinical manifestations and the presence of multiple autoantibodies. There are multiple aetiological factors involved in its pathogenesis. Genetic factors do play an important role and the major histocompatibility complex has been studied extensively and many human leukocyte antigen (HLA) associations have been reported. Twin and familial lupus studies confirm the importance of genetic factors in the development of SLE. Reported HLA associations range from that of HLA-DR3 in Caucasians to HLA-DR2 in Chinese, Japanese and American Blacks. These associations however may only represent linkage disequilibrium and not the actual susceptibility genes. Other non-major histocompatibility complex genes have also been reported to play important roles in the pathogenesis of lupus. The advent of molecular biological techniques has advanced the understanding of susceptibility genes in many diseases. The use of microsatellite genome scanning to study multiplex lupus families has yielded a wealth of information on clusters of susceptibility genes. The identification of these genes will be an important advance in the understanding of this complex disease.

Animals↗

PubMed2004

Gene expression profiling in the study of the pathogenesis of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a complex pathogenesis involving multiple genetic and environmental contributions. DNA microarray technology has recently been applied to unravel some of this complexity through genomewide profiling. Early studies using microarray analysis of peripheral blood mononuclear cells (PBMCs) from SLE patients revealed dysregulation of inflammatory cytokines, chemokines, and immune response-related genes, as well as genes involved in apoptosis, signal transduction, and the cell cycle. More recently, using arrays containing many more genes, 4 independent research groups have found that interferon (IFN)-regulated genes are highly overexpressed in the peripheral blood and kidney glomeruli of SLE patients, supporting a crucial role for interferon in SLE. Future studies focusing on target tissues or organs in lupus, including the kidney, may further contribute to our understanding of lupus pathogenesis while providing new targets for therapy.

Animals↗

PubMed1997

[Neuropsychiatric symptoms and findings in systemic lupus erythematosus ].

Systemic lupus erythematosus (SLE) frequently involves the central nervous system. The clinical presentation is highly variable and heterogeneous. The involvement of the central nervous system is often reflected in minor or major neuropsychiatric symptoms. We describe three cases of this neuropsychiatric lupus erythematosus. A variety of laboratory tests are essential in order to establish the correct diagnosis of neuropsychiatric SLE. We discuss the usefulness of magnetic resonance imaging in both a diagnostic setting and as a tool for improving the understanding of the pathogenesis of neuropsychiatric SLE.

Adult↗

PubMed1996

[Nephrotic syndrome in systemic lupus erythematosus].

Systemic lupus erythematosus as one of the most important connective tissue diseases represents an outstanding disease commonly associated with nephrotic syndrome. The author reviews important facts relating to ethology and pathogenesis of the disease emphasising the renal involvement. Personal observations concerning systemic lupus erythematosus as an ethological factor of nephrotic syndrome as well as relevant diagnostic and therapeutic procedures are presented.

Humans↗

PubMed2006

Systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which is autoimmune in origin and is characterized by the presence of autoantibodies directed against nuclear antigens. It is a multi-system disease, and patients can present in vastly different ways. Prevalence varies with ethnicity, but is estimated to be about 1 per 1000 overall with a female to male ratio of 10:1. The clinical heterogeneity of this disease mirrors its complex aetiopathogenesis, which highlights the importance of genetic factors and individual susceptibility to environmental factors. SLE can affect every organ in the body. The most common manifestations include rash, arthritis and fatigue. At the more severe end of the spectrum, SLE can cause nephritis, neurological problems, anaemia and thrombocytopaenia. Over 90% of patients with SLE have positive anti-nuclear antibodies (ANA). Significant titres are accepted to be of 1:80 or greater. SLE is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life-threatening, but often incapacitating day to day symptoms. Hydroxychloroquine and non-steroidal anti-inflammatory drugs are used for milder disease; corticosteroids and immunosuppressive therapies are generally reserved for major organ involvement; anti-CD20 monoclonal antibody is now used in patients with severe disease who has not responded to conventional treatments. Despite enormous improvements in prognosis since the introduction of corticosteroids and immunosuppressive drugs, SLE continues to have a significant impact on the mortality and morbidity of those affected.

Adult↗