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After 3 years of treatment with azathioprine and prednisone, immunoblastic lymphoma appeared in a patient with systemic lupus erythematosus. An increased incidence of immunological aberrations and malignancies was found in her family and their relation to the pathogenesis of this patient's diseases is discussed.  相似文献   

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Systemic lupus erythematosus. A prospective analysis.   总被引:16,自引:6,他引:10       下载免费PDF全文
The spectrum of organ involvement in 50 patients with systemic lupus erythematosus has been assessed in a prospective study. All patients were admitted to hospital electively for 2 days and a complete clinical and laboratory assessment protocol completed. Subsequent hospital admissions depended on clinical status. The overall mean observation period was 29 months. Widespread multisystem involvement was found in every patient. Subclinical abnormalities of respiratory and cerebral function were common even in patients in clinical remission. A more conservative approach than has been generally recommended was used for the management of systemic lupus erythematosus and is supported by the estimated 5-year survival of 98%.  相似文献   

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Systemic lupus erythematosus in Zimbabwe.   总被引:3,自引:3,他引:0       下载免费PDF全文
Systemic lupus erythematosus (SLE) was diagnosed in 31 black Zimbabweans over a six year period. Renal involvement (71%) was more common and photosensitivity (16%) and serositis (23%) less common than in the United States. Lymphopenia (48%) was the commonest haematological abnormality. Unusual complications included subarachnoid haemorrhage, cardiac rhythm disturbance, portal and superior mesenteric vein thrombosis, and a non-Hodgkin lymphoma. Tuberculosis was a common differential diagnosis that was difficult to exclude. Nine patients (29%) died within one year of diagnosis. SLE is being recognised more commonly in Zimbabwe.  相似文献   

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Systemic lupus erythematosus in childhood.   总被引:2,自引:1,他引:2       下载免费PDF全文
The clinical and serological features have been analysed retrospectively in 42 patients with an onset of systemic lupus erythematosus (SLE) up to 16 years of age. Thirty-seven (88.1%) were female and 5 (11.9%) male. The mean age of onset was 12.3 years (range 7-16); 11 patients were 10 years or under. The mean duration of disease from diagnosis was 7.1 years (range 6 months-25 years). There were 6 deaths, 3 from infection, 2 from renal failure, and 1 from heart failure. Survival was calculated both from the date of onset and from the date of diagnosis. With the latter the estimated overall survival at 5 years was 82.6% and at 10 years 76.1%. The survival for patients with lupus nephritis was 59.5% at 5 years and 47.6% at 10 years. These data suggests that SLE in childhood is not necessarily associated with a poor prognosis, though renal involvement is still serious. There appeared to be no major differences between prepubertal, adolescent, and adult SLE with respect to clinical and serological findings.  相似文献   

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Pregnancy occurring in patients with diagnosed and controlled SLE will be associated with a flare of disease in 60 per cent of cases, which is not significantly different from flares in nonpregnant patients. Signs and symptoms of active SLE should be carefully evaluated and treated with steroids according to severity and organ systems involved. When pregnancy occurs with inactive kidney disease there is a 10 per cent rate of reactivation and SLE kidney disease may appear for the first time during pregnancy in 6.8 per cent of patients. These rates are similar in the control group. There will be a significantly increased abortion rate which cannot be improved with maternal treatment. There will also be a high prematurity rate and an increased number of newborns with intrauterine malnutrition that are associated with active maternal disease. The following points are important when caring for a pregnant SLE patient: 1. Maintain maternal disease inactive throughout gestation. 2. Monitor growth and development of fetus. 3. Monitor for fetal distress. 4. Interrupt pregnancy when fetal distress is diagnosed. 5. A neonatal intensive care unit should be available at the time of delivery. The short-term prognosis is good with no maternal mortality and there is no long-term deleterious influence of pregnancy on the evolution of SLE.  相似文献   

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This review covers major advances in clinical issues related to systemic lupus erythematosus (SLE) published between 1995 and 2000. The classification criteria for both SLE and antiphospholipid syndrome (APS) have been updated, and up to 19 different subsets of neuropsychiatric lupus have been defined. New epidemiological data show that the incidence of new cases and the survival of patients with SLE are both increasing. Several randomised controlled trials have defined the role of cyclophosphamide, methotrexate, antimalarials, and hormonal treatment in the management of SLE. New data are available for drugs such as ciclosporin and thalidomide. Finally, several new treatments for severe refractory cases, such as mycophenolate mofetil and stem-cell transplantation, are being increasingly used. New data also refer to management of thrombosis in APS and high-risk pregnancies in women with SLE or APS.  相似文献   

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Kneitz C  Goebeler M  Tony HP 《Der Internist》2003,44(12):1557-71; quiz 1572-3
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Otte A  Weiner SM 《Lancet》2001,358(9281):586-587
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Systemic lupus erythematosus (SLE) is one of the most diverse autoimmune diseases as it may affect any organ in the body and display a broad spectrum of clinical and immunological manifestations. Epidemiological studies have identified marked differences in the prevalence and course of SLE between genders, and across different ages, races and geographic locations. Methodological differences between studies may account for some of the disparity seen. Additionally, some insights into possible environmental risk factors for SLE have also been provided. As this condition is relatively uncommon, multifactorial, and largely influenced by genetic predisposition, it is inherently difficult to confirm or exclude infectious or environmental contributors to its etiology. Movement of people between communities and defining specific exposures can also be problematic. Despite these limitations, ongoing observation of SLE cohorts in multiple countries and settings, along with large international cooperative efforts in recent years, have helped clarify the risks of SLE in various groups and have defined marked differences in the worldwide occurrence of the disease.  相似文献   

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D'Cruz DP  Khamashta MA  Hughes GR 《Lancet》2007,369(9561):587-596
Systemic lupus erythematosus is an autoimmune connective-tissue disorder with a wide range of clinical features, which predominantly affects women, especially from certain ethnic groups. Diagnosis is based on clinical assessment supported by investigations, including the finding of autoantibodies. Treatments range from antimalarial agents to corticosteroids and immunosuppressive agents. This Seminar draws attention to advances in the epidemiology, genetics, cardiovascular risks, lupus nephritis, CNS disease, the antiphospholipid syndrome, assessment of disease activity and damage, and pregnancy related and quality of life issues. New therapeutic approaches, such as biological agents and mycophenolate mofetil, will also be discussed.  相似文献   

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Systemic lupus erythematosus (SLE) is a typical autoimmune disease that leads to multiple organ damage. For over half a century, SLE has been treated mainly with nonspecific glucocorticoids and immunosuppressants, and the development of molecular target drugs with few adverse reactions is awaited. The treatment goal is remission without systemic symptoms or organ damage. An anti-B-cell activating factor antibody belimumab and an anti-type I interferon receptor antibody anifrolumab are used for patients with active SLE who respond poorly to standard of cares. Additionally, as many susceptibility genes for SLE are associated with signal transduction of dendritic and B cells, cytokines and signaling molecules that bridge the innate and adaptive immune systems are the current focus of attention. Promising approaches include the development of a Janus kinase inhibitors targeting tyrosine kinase deucravacitinib, plasmacytoid dendritic cell-targeted drugs, proteasome inhibitors (e.g., iberdomide), type II anti-CD20 antibody, and obinutuzumab.  相似文献   

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