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PubMed2004

Coronary artery aneurysm with systemic lupus erythematosus.

Coronary artery aneurysms rarely develop in patients with systemic lupus erythematosus. We herein describe a case of a right coronary artery aneurysm associated with systemic lupus erythematosus. A 49-year-old woman with known systemic lupus erythematosus presented with an acute myocardial infarction. A coronary artery aneurysm and thrombo-occlusion in the right coronary artery necessitated percutaneous coronary intervention. She experienced three myocardial infarctions during the following two months. A coronary artery dissection occurred during the percutaneous coronary intervention at the time of the last myocardial infarction, and emergency coronary artery bypass grafting was successfully performed. Difficulties in treatment with percutaneous coronary intervention and the fact that arteritis is a possible cause of a coronary artery aneurysm may indicate that surgical therapy, including coronary artery bypass grafting with or without the obliteration of an aneurysm, is the treatment of choice for a coronary artery aneurysm with systemic lupus erythematosus.

Aneurysm↗
PubMed1995

Musculoskeletal complications of systemic lupus erythematosus in the Hopkins Lupus Cohort: an update.

OBJECTIVE: To determine the frequency of musculoskeletal system damage among patients in the Hopkins Lupus Cohort, over time, and to determine predictors of musculoskeletal damage (including avascular necrosis of bone, osteoporosis, and fracture) in the same cohort. METHODS: Evaluation of the cohort visit database (from 1989 or at the time of entry into the cohort if later than 1989) for the 407 patients with systemic lupus erythematosus (SLE). Musculoskeletal damage was measured by the SLICC/ACR Damage Index. RESULTS: Total damage and musculoskeletal damage accumulated from the time of diagnosis of SLE, and musculoskeletal damage continued over time, with avascular necrosis of bone being the most common form. African-Americans and SLE patients of lower socioeconomic status were more likely to experience musculoskeletal damage. Use of corticosteroids was a major risk factor for both avascular necrosis of bone and osteoporosis. CONCLUSION: Musculoskeletal damage remains one of the major sequela of SLE. Reduction of the modifiable risk factors (especially prednisone dose) is the key to both primary and secondary prevention efforts.

Adult↗
PubMed2006

Systemic lupus erythematosus and pregnancy.

PURPOSE OF REVIEW: Pregnancy in patients with systemic lupus erythematosus is associated with a high risk of maternal disease exacerbation and adverse fetal outcome. This review summarizes recent published findings on lupus pregnancy. RECENT FINDINGS: The literature is in agreement that for most women with inactive and stable systemic lupus erythematosus, pregnancy is safe for both mother and fetus. The main risk factors for adverse pregnancy course and outcome are active disease, nephritis with proteinuria, hypertension, and maternal serum antibodies to SS-A/Ro, SS-B/La, cardiolipin, beta2-glycoprotein I, and lupus anticoagulant. Recent studies have broadened our understanding of the immunological mechanism underlying congenital heart block induced by anti-Ro/La antibodies. In addition, the approach to oral contraceptives has been modified on the basis of two well controlled studies suggesting that they do not cause exacerbation of inactive or mild disease. SUMMARY: Pregnancy in patients with systemic lupus erythematosus is safe and manageable provided the disease is stable. Patients should be evaluated before pregnancy for pregestational risk factors and be closely followed during pregnancy. In most cases of lupus flare during pregnancy, the disease can be safely managed.

Autoantigens↗
PubMed1980

Renal amyloidosis associated with systemic lupus erythematosus.

A renal biopsy of a patient who had systemic lupus erythematosus and proteinuria revealed the presence of both amyloidosis and lupus glomerulonephropathy. The association between secondary amyloidosis and systemic lupus erythematosus has only been reported in four previous cases. The significance of this association is discussed.

Adolescent↗
PubMed2006

[Terbinafine-induced Stevens-Johnson syndrome and aggravation of systemic lupus erythematosus].

BACKGROUND: There have been published reports of induction and aggravation of subacute systemic lupus erythematosus of lupus by terbinafine. We report the case of female patient with systemic lupus erythematosus who, after treatment with terbinafine, presented a Stevens Johnson syndrome eruption, together with renal exacerbation of her connective tissue disease. OBSERVATION: A 25 - year-old woman was followed for 4 years for systemic lupus erythematosus was no renal involvement. The patient was stable under corticosteroids (20 mg/d) and chloroquine (200 mg/d). She was treated with terbinafine onychomycosis caused by Trichophyton rubrum. Seven days after trunk and limbs, with cheilitis affecting the mucous membrane and bilateral conjunctivitis. This rash was followed by epidermolysis involving 10% of the cutaneous area. Stevens-Johnson syndrome was diagnosed. Laboratory tests indicated massive hematuria and proteinuria, and the renal needle biopsy sample showed signs of class III lupus glomerulonephritis. The anti-histone antibodies were highly positive. The patient was treated with systemic corticosteroids (1 mg/kg/d), chloroquine was recommenced and cyclophosphamides were given in a bolus. The outcome of the patient's eruption and lupus was favorable. DISCUSSION: Although induction and worsening of lupus by terbinafine have already been reported, the present case differs through the concomitant occurrence of eruption on treatment with terbinafine and severe relapse of lupus, thus suggesting the involvement of a shared immunological mechanism.

Adult↗
PubMed2004

Systemic lupus erythematosus in Pakistan.

Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions in the world, with some clinical differences among different racial groups. Although data on the characteristics of SLE in Pakistan is scarce, it is not uncommon in the South East Asian region. The purpose of this study was, therefore, to delineate the clinical pattern and disease course in Pakistani patients with SLE and to compare it with international data on lupus patients. A total of 196 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatism Association admitted to the hospital between 1986 and 2001 were studied by means of a retrospective review of their records. Demographically, it was seen that SLE is a disease predominantly of females in their third decade, which is consistent with worldwide data. The mean age of presentation was 31 years (range 14-76) and the mean duration of follow up was 34 (4-179) months. Generally, there was less cutaneous (46%), arthritic (38%), serositis (22%) and renal involvement (33%) but more neuropsychiatric symptoms (26%) in our population. Eighty-six percent of patients were ANA positive, whereas anti dsDNA was positive in 74% of patients. Infections, renal involvement, seizures and thrombocytopenia were associated with poor prognosis (P < 0.05). This study is the first of its kind in Pakistan. The clinical and laboratory characteristics of SLE patients in our study place our population in the middle of a spectrum between the Caucasians and other Asian populations. It has shown that the clinical characteristics of SLE patients in this country may be different to those of its neighbors.

Adolescent↗
PubMed1990

The effect of ethnicity on major histocompatibility complex complement allotypes and extended haplotypes in patients with systemic lupus erythematosus.

Previous studies of patients with systemic lupus erythematosus (SLE) have shown an increased frequency of certain major histocompatibility complex (MHC) markers, including HLA-DR2, DR3, and C4AQ0 (C4A-null), in Caucasian patients. However, most of these studies were of randomly selected, unrelated patients; families were not included, and haplotypes were not determined. In order to define more accurately the possible role of MHC genes in lupus susceptibility, HLA-A, B, C, and DR, as well as BF, C2, C4A, C4B, and GLO, markers were determined in 62 Caucasian patients of known ethnic background, and in the members of their families. The distributions of extended haplotypes (fixed combinations of HLA-B, DR, and complotype alleles), fragments thereof, and individual alleles were determined in SLE patients and controls. The MHC distributions in all patients were compared with haplotypes in a normal Caucasian population. There were no statistically significant differences between the frequencies of any MHC marker, fragment, or extended haplotype in the patients compared with the controls. The patients were categorized into 2 groups of European ancestry (English/Irish; other Europeans), and each group was compared with a group of ethnically matched controls. There was a statistically significantly increased frequency of the alleles C4AQ0 and DR3 and the complotype SC01 in SLE patients of English/Irish descent as compared with ethnically matched controls. The increase in C4AQ0 and DR3 could be accounted for by the fact that they were part of the extended haplotype [HLA-B8;SC01;DR3] and/or its fragment (SC01;DR3). No increase in any MHC marker was observed in the other patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Complement System Proteins↗
PubMed2002

Myocarditis in systemic lupus erythematosus.

Although clinical manifestations of myocarditis in systemic lupus erythematosus are uncommon, noninvasive cardiac testing may detect subclinical cases. The pathogenesis of myocarditis in systemic lupus erythematosus has been ascribed to many factors, including autoimmunity, medications, and coexisting diseases. Lupus myocarditis merits urgent clinical attention because of the likely progression to arrhythmias, conduction disturbances and heart block, dilated cardiomyopathy, and heart failure. Endomyocardial biopsy can be used to identify the underlying inflammatory histopathology. Usual therapy includes high-dose corticosteroids, in addition to standard cardiac medications.

Humans↗
PubMed1992

Immunofluorescence microscopy of healthy skin from patients with systemic lupus erythematosus: more than just the lupus band.

Many papers have been published on the lupus band in systemic lupus erythematosus (SLE), but little information exists on the possible diagnostic value of the lupus band and other microscopic immunofluorescence phenomena found in clinically normal skin of patients with SLE. In a study of 297 subjects (66 patients with SLE, 81 patients with other forms of LE, and 150 patients with other systemic connective tissue disorders) it was found that: (a) granular deposits of IgA, IgG, and IgM in the basal membrane zone and in the deeper blood vessels were more common in patients with SLE than in the other two groups; (b) depending on the clinical differential diagnosis, IgA and IgG deposits at the epidermal basal membrane can be specific for SLE; (c) using logistic regression analysis sets of variables can be selected with a high potential to discriminate between SLE and the other groups; and (d) immunofluorescence variables do not duplicate the information for the diagnosis of SLE given by the American Rheumatism Association (ARA) criteria or other laboratory methods. From these results, it is concluded that immunofluorescence microscopy of clinically normal skin is a valuable diagnostic method which should be reconsidered as a potential criterion for the diagnosis of SLE in the next evaluation of the ARA criteria.

Adolescent↗
PubMed1988

Major histocompatibility complex associations with systemic lupus erythematosus.

This study focused on clinical subsets within systemic lupus erythematosus (SLE) in order to identify more homogeneous patient groups in which to define disease susceptibility gene(s). Analysis of the major histocompatibility complex gene products and genes with major histocompatibility complex class II and class III locus-specific probes and oligonucleotide probes for selected human leukocyte antigen DQ-beta alleles showed significant increases of human leukocyte antigen DR2 and the rare DQ-beta allele DR2-DQw1.AZH in the lupus nephritis patients compared with lupus patients without renal disease (relative risk = 8.3). C4A null was detected in one third of all of the SLE patients. In two thirds of the C4A null patients this was due to a DR3-associated C4A gene deletion. The remaining third may have a regulatory defect and this was DR2-associated. DR4 was significantly decreased in the nephritis patients in comparison with the non-renal SLE patients (relative risk = 0.3). A novel DQ-beta gene has been sequenced from two SLE patients that has not been observed in the normal population. Potential implications of these findings are discussed.

Complement C2↗
PubMed1993

Psychiatric disorder in Malaysians with systemic lupus erythematosus.

All 79 patients who attended a University Systemic Lupus Erythematosus (SLE) Clinic over a 6 month period were assessed using the Clinical Interview Schedule for psychiatric disorder. Using the ICD-9 Classification, 40 were found to have psychiatric disorder, 26 having depressive neurosis, six anxiety neurosis, five endogenous depression and three dementia. The group with psychiatric disorder had significantly poor family support as well as lack of a confidant compared to the group without psychiatric disorder (P < 0.01). There was no difference between the group with psychiatric disorder and those without psychiatric disorder in terms of age, duration of illness, ethnicity and severity of SLE. Psychiatric disorder is common affecting more than half the subjects and depression was the most frequent diagnosis.

Adolescent↗
PubMed1989

Impaired accessory cell function of monocytes in systemic lupus erythematosus.

We studied the ability of monocytes from systemic lupus erythematosus (SLE) patients to act as accessory cells in an in vitro antibody response. Depletion of monocytes from peripheral blood mononuclear cells of normal subjects caused markedly diminished immunoglobulin (Ig) production but that of SLE did not. In order to evaluate the accessory cell activity of monocytes in in vitro immunoglobulin (Ig) production, we designed an accessory cell index of monocytes in the antibody responses. The studies revealed that Ig synthesis of isologous lymphocytes, mixed with monocytes from patients with active lupus nephritis, was significantly lower than those mixed with monocytes from healthy subjects. Moreover, the addition of graded numbers of monocytes of the former to unseparated peripheral blood mononuclear cells did not alter in vitro antibody production. These results indicate that monocytes from active SLE patients have an impared accessory cell function in an in vitro antibody response with no stimulating effect on suppressor cell activity.

Antibody-Producing Cells↗
PubMed2005

Systemic lupus erythematosus in Europe at the change of the millennium: lessons from the "Euro-Lupus Project".

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium--the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Age of Onset↗
PubMed1992

Third International Conference on Systemic Lupus Erythematosus.

A Fourth International Conference on Systemic Lupus Erythematosus will be held in Jerusalem in March 1995. We look forward to a better understanding of the molecular, genetic, immunologic, and clinical aspects of the disease and trust that this knowledge will result in improved treatment.

Animals↗
PubMed2001

Characterization of a prolactin gene polymorphism and its associations with systemic lupus erythematosus.

OBJECTIVE: Hyperprolactinemia is associated with systemic lupus erythematosus (SLE), but the mechanism is unknown. Prolactin is expressed in T lymphocytes and is under the control of an alternative promoter region. We characterized a G/T single-nucleotide polymorphism (SNP) at position -1149 of this promoter and assessed its prevalence in patients with SLE. METHODS: Electrophoretic mobility shift assays (EMSAs) were performed to determine DNA protein complex formation in the prolactin promoter. Transient transfection of reporter gene constructs containing the G/T promoter alleles into the Jurkat T cell line were used to determine transcription activity. Peripheral blood lymphocytes (PBLs) were treated in vitro with phytohemagglutinin (PHA) to determine levels of prolactin messenger RNA (mRNA). RESULTS: EMSAs indicated that binding of a GATA-related transcription factor was altered by the G/T SNP at position -1149. Transient transfection studies in Jurkat cells showed that the G allele consistently produced higher promoter activity. PHA treatment of PBLs in vitro induced a greater increment of prolactin mRNA from patients with the GG(-1149) genotype than from those with the TT(-1149) genotype. Disease association studies in a cohort of SLE patients demonstrated an increased frequency of the prolactin -1149 G allele compared with control subjects. CONCLUSION: We found a functionally significant polymorphism that alters prolactin promoter activity and mRNA levels in the lymphocytes. Altered local prolactin production by immune cells may contribute to disease progression by affecting T cell function.

Alleles↗
PubMed1993

Improved or fatal acute disseminated intravascular coagulation in systemic lupus erythematosus.

To analyze the outcome of systemic lupus erythematosus (SLE) associated with acute disseminated intravascular coagulation (DIC) and also to clarify the clinical factor(s) contributing to the outcome, we retrospectively investigated 120 SLE patients treated between 1981 and 1991. Eight of these patients (6.7%) developed acute DIC; four recovered and the other four died within 2 weeks of onset. Infection preceded acute DIC in all these patients. Acute DIC associated with atypical pneumonia was always fatal, while the patients with pharyngitis or urinary tract infection survived when they were treated adequately. Comparison of the dead and surviving groups revealed that the activity of SLE before the onset of DIC, the severity of DIC, and the treatment given for DIC and the coexistent infection were not significantly related to a fatal outcome. However, severe infection such as atypical pneumonia in patients with secondary immunodeficiency was likely to be fatal irrespective of the presence of DIC.

Acute Disease↗