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PubMed1987

Lamin B autoantibodies in sera of certain patients with systemic lupus erythematosus.

Sera from four patients with systemic lupus erythematosus containing antibodies that yield nuclear rim staining of HEp-2 cells by indirect immunofluorescence were identified and characterized. Each serum contained autoantibodies reacting strongly with lamin B on western blots. One of the four sera displayed weaker reactivity with lamins A and C, while the other three displayed only minimal reactivity with lamins A and C. Titers of antilamin antibodies ranged from 1:1,250 to 1:36,250. Two of the sera also reacted at a dilution of 1:20 with cytoplasmic filaments of PTK-2 cells, suggesting that a small fraction of the autoantibodies in these sera may bind to alpha-helical domains of the lamins that are homologous to those of intermediate filaments. The majority of the antilamin antibodies in these patients' sera are specific for portions of the lamin B molecule that are not homologous to lamins A and C, however. The findings suggest that autoantibodies to the nuclear lamina may, in some instances, be responsible for a rim pattern in the fluorescent antinuclear antibody assay. In addition, autoantibodies to the nuclear lamina in sera of certain patients with systemic lupus erythematosus may be useful for defining the molecular structure and biological functions of lamin B, as well as for studying mechanisms of autoimmunity.

Antibody Specificity↗

PubMed1978

Oxygen-15 brain scanning for detection of cerebral involvement in systemic lupus erythematosus.

In 47 patients with systemic lupus erythematosus seen during fifty-one clinical episodes, oxygen-15, a short-lived gamma-emitting isotope, has been employed in a scannng technique to study cerebral oxygen utilisation and blood-flow. Abnormalities in regional distribution of oxygen utilisation and blood-flow were seen in twenty-three out of twenty-four instances of definite central-nervous-system disease, in fourteen out of fifteen instances of suspected C.N.S. lupus, and in ten out of twelve instances in which C.N.S. disease was not clinically apparent. The technique reflected remissions and relapses. It may prove valuable in diagnosis of subclinical cerebral disease, in monitoring of responses to therapy, and in study of the pathophysiology of cerebral lupus.

Adolescent↗

PubMed1995

Choroidopathy in a case of systemic lupus erythematosus.

Choroidopathy in association with systemic lupus erythematosus (SLE) has rarely been reported. We report a patient with SLE and choroidopathy manifested by bilateral multifocal, serous elevations of the neurosensory retina. Control of the systemic disease resulted in resolution of the serous detachment.

Adult↗

PubMed1979

Evidence for a therapeutic effect of plasmapheresis in patients with systemic lupus erythematosus.

Fourteen patients with active systemic lupus erythematosus (SLE) have been treated with plasmapheresis at a rate of two litres daily on three to four days per week, over a period of two to three weeks. Plasma was replaced isovolemically with either fresh frozen plasma or with human plasma protein fractions. Ten patients were receiving treatment with prednisone at the time of plasmapheresis, and four had received no prior treatment. Eight patients showed evidence of either clinical improvement or clinical and immunochemical improvement, at the time of plasmapheresis. In the three patients who showed high levels of circulating complexes before treatment, there was a sudden fall in the level of circulating immune complexes, which was quantitatively greater than could be explained by the amount removed. This suggests that in some patients with SLE, clearance of complexes by the mononuclear phagocytic system is initially blocked by high levels of circulating complexes and that one effect of plasmapheresis may be to relieve this blockade. Five patients showed a clinical response to plasmapheresis despite the fact that tests for immune complexes were negative. Three patients showed no response to plasmapheresis, and three were regarded as unevaluable. In a limited number of patients, who show a high level of circulating immune complexes, and whose condition is deteriorating despite treatment with corticosteroids, there may be an important therapeutic role for plasmapheresis.

Adolescent↗

PubMed2006

Dyslipoproteinemia during the active course of systemic lupus erythematosus in association with anti-double-stranded DNA (anti-dsDNA) antibodies.

Dyslipoproteinemia is common in lupus patients. In this study, we investigated the pattern of dyslipoproteinemia in the course of active systemic lupus erythematosus (SLE) in possible association with anti-double-stranded DNA (anti-dsDNA) antibodies. Forty-six lupus patients under 45 years old who fulfilled the American College of Rheumatology revised criteria for the classification of SLE were selected. The exclusion criteria were renal failure, nephrotic syndrome, thyroid or liver disease, diabetes mellitus, obesity, pregnancy and taking drugs that induce dyslipidemia. Disease activity was measured by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Comparison of the lipid profiles, between active and inactive groups determined high levels of serum TG and VLDL and low levels of serum HDL in active group in comparison with inactive group (P < 0.05). The results indicated that the levels of TG and VLDL were significantly elevated in the patients with positive anti-dsDNA (P < 0.05). Although, the mean of serum HDL levels was also lower in patients with positive anti-dsDNA, the difference was not significant. This pattern of dyslipoproteinemia in active SLE may be associated with the autoimmune mechanisms especially in relation to the presence of anti-dsDNA antibodies.

Adolescent↗

PubMed1996

Acute myocardial infarction in a young woman with systemic lupus erythematosus.

A young woman was diagnosed with systemic lupus erythematosus at the age of 7 years and incurred an acute myocardial infarction at the age of 17 years. Her risk factors for coronary artery disease include hypertension, hypercholesterolemia, a relatively long disease duration, a fairly active disease as evidenced by the history of nephrotic syndrome and other organ system involvement, and a long history of prednisone use. It is difficult to determine the etiology of this patient's acute myocardial infarction without coronary artery histopathology, but aspects of her presentation (a history of virulent systemic lupus erythematosus, and the angiographic findings of ectasia and aneurysm) suggest that coronary arteritis was the etiology of her accelerated coronary artery disease and subsequent myocardial infarction. Acute myocardial infarction is an uncommon occurrence in premenopausal women less than 30 years old.35 These patients are typically found to have an associated systemic disease such as diabetes mellitus or familial hypercholesterolemia. Systemic lupus erythematosus is a less common systemic disease associated with premature coronary artery disease. Mechanisms of acute coronary syndromes in these patients include accelerated atherosclerosis, active coronary vasculitis, and/or vasospasm with superimposed thrombosis.

Adolescent↗

PubMed2002

Functional melanonychia due to involvement of the nail matrix in systemic lupus erythematosus.

Longitudinal melanonychia is an unusual finding in systemic lupus erythematosus. We report the case of a patient of Arabic extraction with cutaneous lesions of lupus erythematosus on the cheek and chest and longitudinal melanonychia affecting several fingers. Results of histologic examination of the skin and direct immunofluorescence analysis confirmed the diagnosis of systemic lupus erythematosus. The distal matrix revealed changes suggestive of systemic lupus erythematosus accompanied by increased activity of melanocytes but without melanocytic hyperplasia. In this case, longitudinal melanonychia associated with systemic lupus erythematosus was of functional origin.

Adult↗

PubMed2001

Epidemiology of systemic lupus erythematosus.

Tracking the epidemiology of systemic lupus erythematosus is problematic because the diagnosis can be elusive. Systematic review of cases is also difficult because of the varying paths these patients take through the medical provider system. In general, 7 women are stricken with the disease for every man, with an 11:1 female-to-male ratio during the childbearing years. African-Americans are disproportionately afflicted, and evidence is at least suggesting a higher prevalence among other ethnic groups including Hispanics, Asians, and native Americans. Difficulties with defining the patient population, along with minimal education for physicians, may contribute to suboptimal treatment, especially in the critical early stages of disease.

Diagnosis, Differential↗

PubMedDate not supplied

Comparison of tests for the lupus anticoagulant and antiphospholipid antibodies in systemic lupus erythematosus.

A variety of laboratory assays are used to screen for the presence of the lupus anticoagulant. Six commonly used coagulation tests, and the ELISA assay for antiphospholipid antibody using three different substrate phospholipids, have been evaluated in 110 patients with systemic lupus erythematosus or lupus-like disease. One or more coagulation assays was abnormal in 41% (45/110) of the patients. No individual test detected more than 78% of these abnormalities, indicating that a single phospholipid based coagulation test cannot be used to screen for a possible lupus anticoagulant. A combination of Actin FSL, DTTA and DRVVT detected all the abnormalities. The most sensitive two-test combination was Actin FSL and DRVVT. Approximately half (56%) of the patients with a positive clotting test had an abnormal antiphospholipid antibody assay. A similar proportion (58%) of the aPL positive patients had a prolonged coagulation test. The marked discordance between the coagulation assays and a positive antiphospholipid antibody test further complicates the laboratory definition of this abnormality, at least in patients with systemic lupus erythematosus.

Adolescent↗

PubMed1995

Systemic lupus erythematosus. Recognizing its various presentations.

Systemic lupus erythematosus (SLE) is a multisystem disease that predominantly affects women. Probable causative factors include genetic predisposition, complement deficiencies, persistence of antigen, drugs, and environmental factors. The widely varying presentations of SLE include skin, musculoskeletal, cardiovascular, renal, pulmonary, gastrointestinal, neuropsychiatric, systemic, hematologic, and immunologic manifestations. Milder symptoms can be managed with nonsteroidal anti-inflammatory drugs and antimalarial agents. Corticosteroids and cytotoxic drugs are given in more severe disease.

Female↗

PubMed2006

A comparison of damage accrual across different calendar periods in systemic lupus erythematosus patients.

Therapeutic approaches in systemic lupus erythematosus (SLE) have evolved over the last few decades, but their impact on prevention of organ damage is unknown. The objective of this study was to compare new cumulative damage in SLE patients across different calendar periods. Patients from a large SLE cohort were divided into two subcohorts; the first diagnosed and followed between 1978 and 1988 (cohort #1, n=100) and the second between 1989 and 1999 (cohort #2, n=51). Initial Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) scores, and changes in scores over the observation intervals, were compared for the two groups. Logistic regression estimated adjusted odds ratios (OR) comparing damage accrual between the two cohorts. Medication exposures were noted. Baseline characteristics were similar between the two groups. At first assessment, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.79 (95% CI 0.82, 3.88) for cohort #1 versus cohort #2. At the end of the observation interval, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.22 (0.58, 2.55) for cohort #1 versus cohort #2. The adjusted OR for accruing damage over the observation interval in cohort #1 versus cohort #2 was 0.94 (0.39, 2.44). Increased medication exposure was evident for cohort #2 compared to cohort #1. Despite increased therapeutic measures used for patients in more recent periods, our data do not establish a clear difference in damage accrual. This emphasizes the need for strategies to effectively treat lupus-specific manifestations, while minimizing side effects and comorbidities.

Adrenal Cortex Hormones↗

PubMed1994

Aspergillus pneumonia successfully treated with itraconazole in a patient with systemic lupus erythematosus.

The case of a patient with systemic lupus erythematosus who developed pneumonia caused by Aspergillus fumigatus is described. She was treated with itraconazole 200 mg twice daily, with a rapid response. After a follow-up period of 5 years no recurrence of the infection has been detected. To our knowledge this is the first report on the use of itraconazole in patients with systemic lupus erythematosus. Considering the poor prognosis associated with Aspergillus pneumonia in patients with systemic lupus erythematosus, we believe that itraconazole constitutes a safe and effective alternative to amphotericin B in these patients.

Adult↗

PubMed1975

Serum factors causing impaired macrophage function in systemic lupus erythematosus.

Leukocytes from patients with systemic lupus erythematosus (SLE) and healthy controls were cultured for 4 days in either SLE serum or control serum. The remaining monolayer of monocyte-derived macrophages was allowed to ingest yeast cells. Macrophages (from SLE patients or controls) incubated in SLE sera presented impaired phagocytic activity and glass adherence compared with cells grown in control sera. SLE sera contain one or more factors that impair macrophage function; IgG-containing immune complexes may be one such factor.

Adult↗

PubMed2003

The genetics of human systemic lupus erythematosus.

Genetic dissection of human systemic lupus erythematosus (SLE) has been under intense studies during the past decade. Although the complexity inherent to polygenic, multifactorial diseases is challenging, several new insights have been obtained in the past several years using linkage and association studies of families containing SLE patients as well as case-control studies of populations. In addition, recent advances in our understanding of the human genome and emerging technology have been providing new tools in analyses of complex traits, such as SLE. An overview of our current understanding of the genetic basis of SLE and a brief review of findings of linkage and association studies are described here.

Animals↗

PubMed1983

Pregnancy in patients with systemic lupus erythematosus.

This retrospective study of 31 patients with systemic lupus erythematosus during 38 pregnancies shows a spontaneous or missed abortion rate of 7.9%, elective abortion rate of 10.5%, and a perinatal mortality rate of 12.9%. There was one maternal death 5 weeks post partum. If the onset of systemic lupus erythematosus during pregnancy included nephritis or significant thrombocytopenia, the mothers were acutely ill. All of the perinatal mortality occurred in these patients. Management of systemic lupus erythematosus during pregnancy need not differ from that in the nonpregnant state. However, immunosuppressive therapy should not be diminished or discontinued during pregnancy. Clinical parameters, renal function studies, and hematologic information were far more useful than immunologic laboratory data in assessing the course of systemic lupus erythematosus during pregnancy and indicating alterations in treatment. Antepartum fetal surveillance is advised. The timing of and route of delivery must be individualized, and systemic lupus erythematosus in and of itself is not an indication for delivery by cesarean section.

Abortion, Missed↗

PubMed1995

Systemic and cerebral vasculitis coexisting with disseminated coagulopathy in systemic lupus erythematosus associated with antiphospholipid syndrome.

Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are closely related, but each has it own characteristic vasculopathy: vasculitis in SLE and thrombosis in APS, and either may be a serious or life threatening complication. When a patient has SLE associated with APS, systemic and cerebral vasculitis may coexist with disseminated coagulopathy. We report this complication in a 60-year-old woman who died from stroke and myocardial infarction, an occurrence seldom documented in the literature.

Antiphospholipid Syndrome↗

PubMed1987

Perspectives in pediatric systemic lupus erythematosus.

As in the adult, systemic lupus erythematosus is a disorder with many manifestations and a number of subgroups, but in any personal series the total is usually small so that these are not always obvious. There is thus an urgent need for multicenter collection of data. This is important not only for appraisal of different aspects of the disease, particularly central nervous system involvement but also to look at prognosis. Assessment of therapy on the different aspects of this disease also requires multicenter cooperation.

Autoantibodies↗

PubMed1978

Neurocytotoxic antibodies in serum of patients with systemic lupus erythematosus.

Sera from patients with systemic lupus erythematosus (SLE) have been tested for antibody to a human neuronal cell line, SK-N-SH, derived from a metastatic neuroblastoma. With a complement-dependent (51)Cr-release cytotoxicity assay, 75% of SLE sera had antineuronal activity mediated by IgM antibody. Most of the sera containing this IgM neurocytotoxic antibody were also cytotoxic to the human glial cell lines A-172 and U-118MG. The sera did not mediate complement-dependent (51)Cr release when tested against normal human fibroblasts or peripheral blood lymphocytes. IgG antineuronal antibody was detected in 17% of SLE sera by an antibody-dependent, cell-mediated cytotoxicity assay with SK-N-SH cells as targets. The relationship of IgM and IgG antineuronal antibodies to the antilymphocyte antibodies present in SLE sera was evaluated by a series of crossabsorption experiments using SK-N-SH cells to remove neuronal antibodies and WI-L2 (human lymphoblasts) to remove antilymphocyte antibodies. Most of the complement-dependent neurocytotoxicity was not removed by multiple lymphoblast absorptions, although the WI-L2 cells readily removed lymphocytotoxic activity as assayed on normal lymphocytes. Absorption with SK-N-SH cells removed most, but not all, of the lymphocytotoxic antibody. Thus, although lymphocytotoxic antibodies reactive with membrane antigens shared by lymphocytes and brain may constitute a subset of the antibodies to neural cells, most of the antineuronal activity in SLE serum is directed at other cell surface antigens expressed on neuronal and glial cells. Should they gain access to the brain, these antibodies have the potential to produce neuropathology, but their presence in the nervous system of patients with the neuropsychiatric manifestations of SLE is yet to be documented.

Antibodies↗