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[Retrospective evaluation of pregnancy in women suffering from systemic lupus erythematosus (SLE)].

OBJECTIVES: Systemic lupus erythematosus (SLE) is an autoimmune disease predominantly affecting women of childbearing age, which may negatively influence the pregnancy course and outcomes. The objective of the study was to estimate the risk of fetal loss and prematurity in lupus patients. These data seem to be an important component of family counselling in lupus patients. DESIGN: The pregnancies' course and outcomes in lupus patients and in healthy controls were compared. MATERIAL AND METHODS: 47 lupus patients with pregnancies occurring after the diagnosis of SLE and 108 healthy controls were included in this study. The pregnancies were analysed in terms of delivery term and outcomes (live births/ miscarriage/ neonatal deaths/ congenital defects). The structured review and case histories analysis were used to gather the data. RESULTS: The percentage of spontaneous abortions (24.1%) and preterm deliveries (24.1%) were significantly higher in lupus patients when compared with healthy controls (9.3% and 5.3% respectively). CONCLUSIONS: The caution in pregnancy planning in lupus patients is necessary and the role of physicians' information in patients' awareness of fetal loss and prematurity is vital.

Abortion, Spontaneous↗

Comparison of renal disease severity and outcome in patients with primary antiphospholipid syndrome, antiphospholipid syndrome secondary to systemic lupus erythematosus (SLE) and SLE alone.

OBJECTIVE: To ascertain the clinical presentation, histopathology and outcome of renal involvement in patients with primary antiphospholipid syndrome (PAPS), antiphospholipid syndrome secondary to systemic lupus erythematosus (SAPS) and systemic lupus erythematosus alone. METHOD: A retrospective analysis was undertaken of 20 patients with PAPS, 25 patients with SAPS and 275 patients with systemic lupus erythematosus to ascertain the frequency and pattern of renal involvement. RESULTS: Renal involvement was found most frequently in patients with SAPS, in whom it occurred in 68% of patients. Renal disease was equally common in patients with PAPS and systemic lupus erythematosus alone where it was seen in 30% of patients. Patients with systemic lupus erythematosus most frequently presented with nephrotic syndrome due to glomerulonephritis, whereas those with PAPS and SAPS were more likely to present with hypertension and reduced glomerular filtration rate. No patients with PAPS developed end-stage renal failure compared with 5.9% of patients with SAPS and 16.9% of patients with systemic lupus erythematosus alone; 23.5% of patients with SAPS died compared with 15.7% of patients with systemic lupus erythematosus alone and no patients with PAPS. CONCLUSION: Renal involvement is a major feature of both PAPS and SAPS, where renal thrombosis frequently leads to reduced glomerular filtration rate and hypertension. One-third of patients with systemic lupus erythematosus alone develop glomerulonephritis leading to renal disease which most commonly presents with nephrotic syndrome. Patients with PAPS were less likely to develop end-stage renal failure or die during the follow-up period.

Adolescent↗

Heterogeneity and diversity of IgM and IgG lupus anticoagulants in an individual with systemic lupus erythematosus.

From one patient with systemic lupus erythematosus retaining lupus anticoagulant (LAC), we established 6 Epstein-Barr virus-transformed human B cell clones secreting antibodies that affect the coagulation assay. Two and 4 of the clones secreted IgM and IgG antibodies, respectively. Although all 6 antibodies displayed anticardiolipin activity in ELISA, the increased binding activity in the presence of beta 2-glycoprotein I was limited only to the IgG antibodies. Five antibodies (two IgM and three IgG) had LAC activity which prolonged the activated partial thromboplastin time (APTT), whereas one IgG antibody shortened the APTT. Two of the IgG producing clones had an identical Ig heavy chain gene rearrangement despite their opposite effects on the coagulation assay. These results demonstrated the heterogeneity of LACs and diversity among their physiological functions.

Adult↗

Major histocompatibility complex genes and susceptibility to systemic lupus erythematosus.

Susceptibility to systemic lupus erythematosus is associated with major histocompatibility complex (MHC)--encoded genes. We have used nucleotide sequence analysis to better define the disease-associated MHC alleles. HLA-DR2, DQw1, and especially the rare allele DQ beta 1. AZH confer high relative risk (RR = 14) for lupus nephritis in a Caucasian population of patients. Pilot studies using historical controls suggest that these genes also confer a high risk in non-Caucasian ethnic groups (RR = 24-78). We have found that DR4 is significantly decreased in patients with lupus nephritis. Fifty percent of the patients with lupus nephritis had either the DQ beta 1.1, the DQ beta 1.AZH, or the DQ beta 1.9 alleles. These alleles share amino acid residues that have been predicted to be the contact points for antigen and the T cell receptor. These HLA alleles appear to have a direct role in the predisposition to lupus nephritis, whereas DR4 may have a "protective" effect.

Alleles↗

Pathology of cutaneous lupus erythematosus.

The only histologic finding common to all types of lupus erythematosus lesions is a perivascular lymphocytic infiltrate. The other histologic changes in lupus erythematosus are entirely dependent on the morphology and age of the individual lesions biopsied. We do not believe that the main clinical subsets of lupus erythematosus can be reliably diagnosed on the basis of histology alone. Some of the less common forms of lupus erythematosus have distinctive but not pathognomonic, histopathologic features. The pathogenesis of most of the histologic changes is relatively unknown, leaving room for speculation and future research.

Acute Disease↗

Sudden cardiac ischaemic death associated with systemic lupus erythematosus.

Association between systemic lupus erythematosus and coronary atherosclerosis has been reported only rarely in the medical literature. Sudden ischaemic cardiac death in such cases is an uncommon terminal event, particularly in young patients. The severity of coronary atherosclerosis in these cases may be similar to those seen in the classic coronary disease. A case of a 22-year-old female systemic lupus patient is presented in whom sudden death was found at autopsy to be due to extensive coronary atherosclerosis.

Adult↗

The nephritis of systemic lupus erythematosus--a review.

Systemic lupus erythematosus (SLE) is a disease of unknown aetiology. Its hallmark is anti-nucleic acid antibodies and it is accompanied by variable clinical, serologic, and histologic signs. Renal involvement in SLE has been documented for over 50 years; it represents one of the most serious complications of this systemic disorder. Although our understanding of the pathogenesis and natural history of lupus nephropathy is still inadequate, it is nevertheless more comprehensive than in most other forms of glomerulonephritis. This paper will review current concepts in the pathogenesis, histologic classification, clinical course, clinico-pathological correlation and therapeutic strategy of lupus nephropathy.

Antimalarials↗

Systemic lupus erythematosus in pregnancy.

Systemic lupus erythematosus is an inflammatory autoimmune disease that can affect multiple organ systems. The most popular theory regarding the origin of its clinical manifestations is that autoantibodies and circulating immune complexes become trapped in the capillaries of visceral structures.

Antiphospholipid Syndrome↗

Previous pregnancy outcome is an important determinant of subsequent pregnancy outcome in women with systemic lupus erythematosus.

Women with systemic lupus erythematosus (SLE) have increased adverse pregnancy outcomes. The reasons for these problems include maternal disease, clinical or serologic activity, medication use, and residual organ impairment from prior disease flares. In retrospective studies, pregnancy data are often treated cross-sectionally, with births rather than mothers as the unit of analysis. Multiple pregnancies from the same mother may be highly correlated with each other. In an unmatched retrospective study, the first two pregnancy outcomes in lupus patients with anticardiolipin antibody (anti-CL IgG or IgM isotype) (cases N = 47) and without anticardiolipin antibody (controls, N = 125) were assessed according to birth order. A good outcome was defined as a full-term (> 38 weeks) live birth without neonatal complications. All other pregnancy outcomes were considered adverse outcomes. Therapeutic abortions and ectopic or molar pregnancies were excluded. Both cases and controls with an adverse outcome in their first pregnancy had at least a 50% chance of another adverse outcome in their second pregnancy. Cases with a late miscarriage (fetal loss at 14 to 20 weeks' gestation) in their first pregnancy had the highest risk, 80%, of an adverse outcome in their second pregnancy. Both previous pregnancy loss and anti-CL antibody status should be considered in the analysis of pregnancy outcomes in women with SLE.

Antibodies, Anticardiolipin↗

Systemic lupus erythematosus and pregnancy.

Systemic lupus erythematosus (SLE) may improve, exacerbate or be unchanged during pregnancy. The activity of SLE at the time of conception has a major influence on pregnancy outcome: SLE patients with quiescent disease do well, while those with active, uncontrollable disease have a high abortion rate. There are specific autoantibodies that confer a high risk of abortion (e.g., IgG antiphospholipid antibodies). There are others, such as anti-Ro/SSA and anti-La/SSB, which confer a risk for neonatal lupus that includes lupus dermatitis, congenital complete heart block, thrombocytopenia and cholestatic jaundice, in order of their relative frequencies.

Abortion, Spontaneous↗

[ANF-negative subacute cutaneous lupus erythematosus with anti-Ro antibodies. A new subset of lupus with characteristic skin manifestations].

Recently a new subset of lupus erythematosus with distinct cutaneous lesions has been defined as subacute cutaneous lupus erythematosus. The salient clinical features are transient nonscarring widespread erythematosquamous or annular eruptions on the trunk and arms, cytopenia, musculo-skeletal symptoms and sometimes mild renal and cerebrovascular disease. Although more than one half of the patients have positive antinuclear factor tests, in a smaller proportion only antibodies against the predominantly cytoplasmic antigens Ro and sometimes La can be demonstrated. Due to the negative routine serologic findings the typical cutaneous lesions assume additional importance, because they frequently allow a clinical diagnosis even in the absence of more sophisticated immunologic procedures.

Antibodies, Antinuclear↗

[Lupus erythematosus in newborns].

Neonatal lupus erythematosus is an uncommon disease described mainly through case reports. In this paper the pathogenesis, epidemiology, skin and organ manifestations of the disease were presented. The necessity of co-operation between physicians of different specialisations in the prevention and treatment was underlined.

Humans↗

The spectrum of liver disease in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) have a 25-50% chance of developing abnormal liver tests in their lifetime. This percentage does not include unconjugated hyperbilirubinaemia due to haemolysis associated with SLE, or elevated aspartate-aminotransferase caused by SLE-associated myositis. The most common cause is drug-induced hepatitis, while mild, predominantly lobular-but sometimes also portal and periportal-hepatitis reflecting SLE activity is another possibility. Other liver disease in SLE can be related to thrombotic events, whether or not associated with the lupus anticoagulant, including Budd-Chiari syndrome and veno-occlusive disease. Other liver abnormalities have been more or less frequently associated with SLE, such as nodular regenerative hyperplasia, perihepatitis, and hepatic or splenic rupture. Also viral hepatitis, obstructive jaundice, autoimmune hepatitis, primary biliary cirrhosis, granulomatous hepatitis, cryptococcus infection of the liver, chronic hepatitis with IgA or IgD deficiency, porphyria or idiopathic portal hypertension co-existing with SLE have been described.

Humans↗

Ethnicity and mortality from systemic lupus erythematosus in the US.

OBJECTIVE: To study ethnic differences in mortality from systemic lupus erythematosus (lupus) in two large, population-based datasets. METHODS: We analysed the national death data (1979-98) from the National Center for Health Statistics (Hyattsville, Maryland, USA) and hospitalisation data (1993-2002) from the Nationwide Inpatient Sample (NIS), the largest hospitalisation database in the US. RESULTS: The overall, unadjusted, lupus mortality in the National Center for Health Statistics data was 4.6 per million, whereas the proportion of in-hospital mortality from the NIS was 2.9%. African-Americans had disproportionately higher mortality risk than Caucasians (all-cause mortality relative risk adjusted for age = 1.24 (women), 1.36 (men); lupus mortality relative risk = 3.91 (women), 2.40 (men)). Excess risk was found among in-hospital deaths (odds ratio adjusted for age = 1.4 (women), 1.3 (men)). Lupus death rates increased overall from 1979 to 98 (p<0.001). The proportional increase was greatest among African-Americans. Among Caucasian men, death rates declined significantly (p<0.001), but rates did not change substantially for African-American men. The African-American:Caucasian mortality ratio rose with time among men, but there was little change among women. In analyses of the NIS data adjusted for age, the in-hospital mortality risk decreased with time among Caucasian women (p<0.001). CONCLUSIONS: African-Americans with lupus have 2-3-fold higher lupus mortality risk than Caucasians. The magnitude of the risk disparity is disproportionately higher than the disparity in all-cause mortality. A lupus-specific biological factor, as opposed to socioeconomic and access-to-care factors, may be responsible for this phenomenon.

Adolescent↗

Chimerism in systemic lupus erythematosus--three hypotheses.

Systemic lupus erythematosus (SLE) is an immune-mediated disease characterized by the presence of autoantibodies and a wide array of clinical symptoms. Despite intensive research, the aetiology of SLE is still unknown and is probably multifactorial. Both genetic and environmental factors have been associated with SLE, but these factors alone are insufficient to explain the onset of SLE. Recently, it has been suggested that chimerism plays a role in the pathogenesis of autoimmune diseases, including SLE. Chimerism indicates the presence of cells from one individual in another individual. In an experimental mouse model, the injection of chimeric cells induces a lupus-like disease. In addition, chimerism is found more often in kidneys of women with SLE than in healthy controls. There are several mechanisms by which chimeric cells could be involved in the pathogenesis of SLE. In this review, three hypotheses on the role of chimerism in SLE are discussed. The first two hypotheses describe the possibilities that chimeric cells induce either a graft-vs-host reaction in the host (comparable with reactions seen after bone marrow transplantation) or a host-vs-graft reaction (comparable with reactions seen after solid organ transplantation). The third hypothesis discusses the possible beneficial role chimeric cells may play in repair mechanisms due to their stem cell-like properties. This review provides insights into the mechanisms by which chimerism may be involved in SLE and proposes several lines of inquiry to further investigate chimerism in SLE.

Animals↗

Autoantibodies in systemic lupus erythematosus.

Autoantibodies in systemic lupus erythematosus (SLE) are targeted predominantly to intracellular nucleoprotein particles. Following analysis of the quantitative and qualitative properties of the humoral immune response as well as identification of the major particles recognized as antigens, current studies are addressing the following questions: How is tolerance lost? What accounts for the selection of the antigens? What perpetuates the autoimmune response in SLE?

Animals↗

Cytomegalovirus-induced flare of systemic lupus erythematosus.

Exacerbations of systemic lupus erythematosus (SLE) may be brought about by a number of factors, including stress, surgery, or infection. By interacting with immunologic mediators or by producing immune complexes, infections may exacerbate underlying autoimmune diseases such as SLE. This illustrative case report describes a woman who had SLE for 20 years who contracted cytomegalovirus mononucleosis that induced an exacerbation of her SLE.

Cytomegalovirus Infections↗

Burkitt's lymphoma presenting as ileocaecal intussusception in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) are reported to have an increased risk of malignancy, especially lymphoproliferative disorders. We decribe the occurrence of ileocaecal intussusception secondary to Burkitt's lymphoma in a patient with SLE. A 23-year-old woman, who had been diagnosed with SLE 2 years ago, developed intermittent abdominal pain with a palpable mass. Computed tomography and a double-contrast barium enema showed a lobulated mass with intussusception at the ileocaecal junction. Right hemicolectomy and splenectomy was performed after histopathological examinations on colonoscopic biopsy revealed Burkitt's lymphoma. Fourteen months after chemotherapy, there is no evidence of recurrence of the Burkitt's lymphoma. When a patient with SLE has abdominal complaints, besides serositis, lupus enteritis such as peptic ulcer disease, mesenteric vasculitis with or without complications and pancreatitis, we have to consider intussusception secondary to gastrointestinal lymphoma as one of the differential diagnoses. Therefore, we should thoroughly investigate patients with SLE presenting with abdominal pain and not simply consider it afeature of lupus enteritis until other causes have been ruled out.

Adult↗