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PubMed2003

Understanding and managing systemic lupus erythematosus (SLE).

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that many people know little about. Lupus can affect the skin, joints, kidney, heart, lungs, nervous system, blood and other organs or systems. For most people it is a relatively mild disease; for others it causes life-threatening problems. Social workers and related health professionals must learn to recognize how lupus affects individuals and their families' physical, psychological and social functioning in order to develop effective interventions for their patients. This article provides basic medical information about lupus and discusses strategies to help facilitate understanding, coping and management of this disease.

Adaptation, Psychological↗
PubMed1993

Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus.

In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.

Adolescent↗
PubMed1992

Prospective study of fluctuations of lupus anticoagulant activity and anticardiolipin antibody titre in patients with systemic lupus erythematosus.

Fluctuations of lupus anticoagulant activity and anticardiolipin antibody titres were studied in 53 patients with systemic lupus erythematosus (SLE). The median study time was 26 months with a median number of 12 samples. Lupus anticoagulant was measured by the kaolin clotting time (KCT) and dilute Russell viper venom time (dRVVT) assays; anticardiolipin antibodies were assayed by an enzyme linked immunosorbent assay (ELISA). Normal and increased KCTs or dRVVTs were seen during follow up in 13 and 12 patients, respectively. IgG anticardiolipin antibodies changed from negative to positive or positive to negative in 26 patients and IgM anticardiolipin antibodies in 16 patients. Disease activity and treatment with prednisone could account for these fluctuations in the kaolin clotting time (KCT) in 7 of 13 patients and in the dRVVT in 2 of 12 patients. Whole group analysis showed that the KCT, dRVVT, and IgM anticardiolipin antibodies were not associated with disease activity, in contrast with IgG anticardiolipin antibodies. During treatment with prednisone normal KCT and dRVVT results were obtained more easily than normal anticardiolipin antibody levels. It is recommended that lupus patients should not be classified as antiphospholipid antibody positive or negative on the basis of only one sample.

Adolescent↗
PubMed2006

Lupus erythematosus profundus in black South Africans.

BACKGROUND: Lupus erythematosus profundus (lupus panniculitis) is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue. There are only a few reported series of patients with this condition; none in individuals of African ancestry. The aim of the study was to evaluate clinical, histopathological and laboratory findings in black South African patients with lupus profundus. PATIENTS AND METHODS: Ten prospectively observed patients were studied. Skin biopsies were performed, and several laboratory tests routinely employed in patients with lupus erythematosus were carried out. All patients were followed up for at least a year and their response to treatment was assessed. RESULTS: All patients were females. Mean age at diagnosis was 28.5 years. Face was the most common site of involvement. In five patients, the periorbital edema was the initial manifestation. Only one patient had systemic lupus, and three patients also showed lesions of discoid lupus. In only 30% of the patients were the ANA titers greater than 1 : 80, and positivity in other lupus tests was rare and inconsistent. In general, patients responded to antimalarials and/or systemic corticosteroids. CONCLUSION: African patients with lupus profundus when compared with other series comprising Asian and white patients showed greater frequency of periorbital edema as the initial manifestation. Review of the literature disclosed that this subset of lupus profundus appears to have a more benign course.

Adult↗
PubMed1998

Increased risk of malignancy in patients with systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus is a chronic, multisystem, autoimmune disorder that primarily affects women. Morbidity and mortality have improved for lupus patients during the last 15 years. An increased risk of malignancy in patients with lupus has been shown in some, but not all studies. The purpose of this study was to ascertain cancer risk in lupus patients by linking two disease registries. METHODS: Participants in the Chicago Lupus Cohort included 616 women with lupus who were residents of Cook County, Illinois. They were seen during 1985-1995 at 4 University, inner city, and suburban inpatient and outpatient clinics in Chicago. Malignancies occurring in these subjects during the study interval, 1985-1995, were identified from the Illinois State Cancer Registry by matching name, birthdate, and social security number. Standardized incidence ratios (SIRs) were estimated for all malignancies in this cohort of lupus patients using age, gender, and all race or race-stratified specific cancer incidence data from Cook County, Illinois. RESULTS: The registry linkage study with the Illinois State Cancer Registry documented that 30 women with lupus had a malignancy. The expected number of malignancies for women in the lupus cohort was 15.0. There were 8 cases of breast cancer and 4 each of lung and cervical cancer. In the remaining 14 women, 12 different types of cancers were noted. The SIR and 95% confidence interval (CI) for malignancy for all women with lupus in the study were 2.0 (1.4, 2.9) and lung cancer was the only individual cancer increased in all women--SIR and 95% CI were 3.1 (1.3, 7.9). In the analysis stratified by race, the risk of malignancy (SIR and 95% CI) was increased in Caucasian women, 2.3 (1.4, 3.9). Breast cancer was the only individual cancer increased in Caucasian women with lupus with an SIR and 95% CI of 2.9 (1.4, 6.4). CONCLUSIONS: Lupus patients have an increased risk of malignancy. Breast, lung, and gynecological malignancies were the most common malignancies observed in the cohort and breast cancer was significantly increased in Caucasian women.

Adult↗
PubMed2002

Cutaneous lupus erythematosus: a review.

This article will review and update information about the pathogenesis, clinical presentation, diagnosis, and treatment of cutaneous lupus erythematosus. Lupus erythematosus (LE) can present as a skin eruption, with or without systemic disease. Cutaneous LE is subdivided into chronic cutaneous LE, subacute cutaneous LE and acute LE. The prevalence of systemic lupus erythematosus (SLE) is 17-48/100,000 population worldwide. Skin disease is one of the most frequent clinical complaints of patients suffering from SLE. It has been found to occur in up to 70% of patients during the course of the disease. The most frequent mucocutaneous manifestations of SLE are malar rash (40%), alopecia (24%), and oral ulcers (19%). It has been suggested that risk factors that are more likely to signal transition of cutaneous into systemic LE are high ANA titers (> 1:320) and the presence of arthralgias. CLE patients who exhibit these symptoms should be monitored closely, since they may be at increased risk to develop SLE.

Animals↗
PubMed2005

Proinflammatory cytokine (IL-1beta, IL-6, IL-12, IL-18 and TNF-alpha) levels in sera of patients with subacute cutaneous lupus erythematosus (SCLE).

Subacute cutaneous lupus erythematosus (SCLE) is a subset of lupus erythematosus that identifies patients with clinically recognized erythematous, nonscarring lesions, photosensitivity and serologic abnormalities. Anti-Ro (SS-A) antibodies are considered to be a typical immunopathologic marker of SCLE. Autoimmune diseases have been also characterized by the disturbances in the cytokine network. The aim of this study was to compare the concentrations of proinflammatory cytokines (IL-1beta, IL-6, IL-12, IL-18 and TNF-alpha) in serum of ANA-positive (antibody against nuclear antigen) and ANA-negative patients with SCLE. Sera samples were collected from 15 patients with SCLE (9 ANA-positive and 6 ANA-negative ones). The preliminary identification of autoantibodies as well as their titers was determined on HEp-2 cells using IIF method. Western blotting (EUROIMMUN) was applied to verify the results of IIF. Proinflammatory cytokine concentrations in the patients' sera samples were determined by enzyme-linked immunosorbent assay (ELISA) (Bender MedSystems). The levels of IL-12 were higher in ANA-positive patients than in ANA-negative subgroups [median (interquartile range), 330 pg/ml (128-708 pg/ml) versus 39.4 pg/ml (31.25-80 pg/ml)]. Similar differences were observed in the level of IL-18 [median (interquartile range), 508.4 pg/ml (180-1222 pg/ml) versus 100.5 pg/ml (78.1-154 pg/ml)]. The differences in TNF-alpha levels between the groups of ANA-positive and ANA-negative patients were at the verge of statistical significance, p<0.05. The sera levels of IL-1beta and IL-6 were low and of no significant difference concerning the ANA-positive and ANA-negative subgroups. Since serum levels of IL-12 and IL-18 were higher in ANA-positive patients than in ANA-negative patients, these cytokines might play an important role in the inflammatory process in SCLE.

Cell Line↗
PubMed2003

Autoantibodies against mannose-binding lectin in systemic lupus erythematosus.

In systemic lupus erythematosus (SLE), autoantibodies directed against complement components of the classical pathway, especially against C1q, are associated with severe disease and are of prognostic value for flares of lupus nephritis. Mannose-binding lectin (MBL), the recognition unit of the MBL pathway of complement activation, has structural similarities to C1q. Deficiencies of MBL have been shown to predispose to the development of SLE and to influence the course of the disease. We hypothesized that the presence of autoantibodies to MBL, analogous to autoantibodies to C1q in patients with SLE, may contribute to disease development. The occurrence of anti-MBL autoantibodies was assessed by enzyme-linked immunosorbent assay (ELISA) of 68 serum samples from 20 patients with SLE and in serum from 70 healthy controls. Levels of antibodies directed against MBL were significantly higher in patients with SLE compared to healthy subjects. No significant difference was found between patients with active disease compared to those with inactive disease. While the occurrence of anti-C1q autoantibodies was associated with renal involvement, no such relationship was found for anti-MBL autoantibodies. A significant correlation was found between anti-MBL and anti-C1q antibody levels. The level of anti-MBL antibodies was negatively correlated with MBL-complex activity of circulating MBL. Anti-MBL autoantibodies were of the immunoglobulin G (IgG) isotype and the binding site of IgG anti-MBL was located in the F(ab')2 portion. We conclude that anti-MBL are present in sera from SLE patients and influence the functional activity of MBL.

Adult↗
PubMedDate not supplied

[Spectral analysis of the electroencephalogram in children with systemic lupus erythematosus].

INTRODUCTION: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects different systems and organs, including the central nervous system (CNS). It has been suggested that about 40% of the cases of neuropsychiatric lupus (NPSLE) develop before SLE is diagnosed or at the same time it is being carried out, and 63% appear during the first year following diagnosis. AIMS: The aim of this study was to check the hypothesis that the electroencephalogram (EEG) may be sensitive to the damage to the CNS in children with SLE in whom there is still no clinical evidence of NPSLE. PATIENTS AND METHODS: EEG recordings were performed in 30 children with a diagnosis of SLE with or without signs of a neuropsychiatric syndrome. The results of the EEG were evaluated visually and analysed quantitatively. RESULTS: The visual inspection of the EEG showed the presence of alterations in 44.5% of the children with SLE and in 76.9% of those with NPSLE. There were significant differences in Student's t test (p = 0.0055) between the two groups for the analysis of the broadband spectral measurements. The narrow band analysis revealed a significant increase in the theta and delta frequencies in children with SLE as compared to standard values, whereas in children with NPSLE significant differences were found in the fast bands in frontal regions. CONCLUSIONS: Spectral analysis of the narrow band could help to confirm diagnoses of NPSLE, while anomalies in the slow bands could be an early marker of damage to the CNS although there are still no symptoms of the disease.

Adolescent↗
PubMed2003

[Drug-induced lupus erythematosus].

Drug-induced lupus is a syndrome which share symptoms and laboratory characteristics with the idiopathic systemic lupus erythematosus (SLE). The list of medications implicated as etiologic agents in drug-induced lupus continues to grow. The terms used for this condition are lupus-like syndrome, drug-induced lupus erythematosus (DILE) and drug related lupus. More than 80 drugs have been associated with DILE. The first case of DILE was reported in 1945 and associated with sulfadiazin. In 1953 it was reported that DILE was related to the use of hydralazine. Drugs responsible for the development of DILE can divided into three groups, but the list of these drugs is quite long because new drugs are included yearly in the list. The syndrome is characterised by arthralgia, myalgia, pleurisy, rash and fever in association with antinuclear antibodies in the serum. Recognition of DILE is important because it usually reverts within a few weeks after stopping the drug.

Antibodies, Antinuclear↗
PubMed2001

Prolactin in murine systemic lupus erythematosus.

Murine systemic lupus erythematosus (SLE) manifests several autoimmune perturbations that resemble human SLE, including cytokine aberrations, lymphoproliferation, hypergammaglobulinemia, autoantibody formation, and immune complex glomerulonephritis. In multiple studies, elevated serum prolactin concentrations (hyperprolactinemia) stimulated appearance or progression of murine lupus. Autoimmune disease acceleration by prolactin appears to be accentuated by estrogen stimulation of prolactin secretion and independent of immunosuppressive effects of androgens such as testosterone. Conversely, suppression of serum prolactin concentrations by bromocriptine inhibits development of murine SLE. These data clearly support the concept that prolactin is immunostimulatory in autoimmune disease and that the therapeutic goal of lowering serum prolactin concentrations may be beneficial to patients. Further utilization of murine SLE models will facilitate dissection of the actions and interactions of prolactin with estrogen, progesterone and testosterone and lead to a better understanding of hormonal immunomodulation and therapy of autoimmune disease.

Animals↗
PubMedDate not supplied

Phenotypic and functional analysis of natural killer cells in systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) manifested with multiple autoantibodies production and glomerulonephritis, is the best example of systemic autoimmune diseases. To further elucidate the role of cytokines and the potential involvement of natural killer cells (NK cells) in the pathogenesis of lupus, phenotypic analysis of peripheral blood mononuclear cells (PBMC), NK cells cytotoxicity and cytokines production pattern of SLE patients and normal controls were examined. In addition, the effect of a variety of cytokines on anti-dsDNA antibodies production was also investigated. Our results showed that: (a) there was an increased percentage of memory T cells and decreased percentage of NK cells in SLE patients when compared to normal controls (p < 0.05); (b) a decreased production of cytokines like gamma-IFN in mitogen-stimulated PBMCs was also noted in SLE patients; (c) cytolytic activity of NK cells was markedly reduced in SLE patients (p < 0.05); (d) spontaneous secretion of IgG anti-dsDNA antibodies by B cells isolated from SLE patients could be inhibited by gamma-IFN, but not by IL-2, IL-4 and IL-5. These data suggested that decreased functions of NK cells and related type 1 T helper cells be closely related to the immune dysregulation and autoantibodies production in SLE.

Adolescent↗
PubMed2004

The study of the participation of basement membrane zone antibodies in the formation of the lupus band in systemic lupus erythematosus.

BACKGROUND: The lupus band test (LBT) is an important auxiliary method in the diagnosis of systemic lupus erythematosus (SLE), but the mechanism of its formation is still unknown. There are many kinds of autoantibodies, such as basement membrane zone autoantibodies (BMZ-Abs), in patients with SLE. AIM: To detect whether skin BMZ-Abs participated in the formation of the lupus band in SLE. Methods Immunoglobulin G (IgG)-type BMZ-Abs in 15 SLE patients were detected by means of immunofluorescence (IF), immunoblotting (IB), and immunoelectron microscopy (IEM). RESULTS: Direct immunofluorescence (DIF) on salt-split skin showed epidermal fluorescence in 12 of the 15 SLE patients. Two of the 12 patients also showed dermal fluorescence. Indirect immunofluorescence (IIF) on salt-split skin revealed that, in 12 of the 15 (80%) sera, antibodies were bound to the epidermal roof of the salt-split skin. IB showed that, in 14 SLE sera, autoantibodies reacted to 230, 200, 180, 130, and 97 kDa epidermal extracts and 75 kDa dermal extracts. Direct (DIEM) and indirect (IIEM) immunoelectron microscopy showed that gold particles were directed to every region of the BMZ, including hemidesmosomes, lamina lucida, lamina densa, and sublamina densa. CONCLUSIONS: BMZ-Abs in SLE sera may participate in the formation of the lupus band.

Autoantibodies↗
PubMed1996

[Cytokines in systemic lupus erythematosus].

In systemic lupus erythematosus (SLE) is a disease characterized by B cell hyperactivity, autoantibody production and immune complex deposition in vital organs. To explain the mechanisms responsible for immune dysregulation in SLE cytokines have received increasing attention. This review has discussed a number of cytokines which appear to be involved in lupus pathogenesis. Recent studies have shown that disease activity and the main symptoms of SLE are associated with increasing serum levels of cytokines such as interleukin-(IL)-1, IL-2, IL-6, interferon-gamma (IFN-gamma) and tumor necrosis factor-alpha (THF-alpha). Constitutive expression and in vitro induction of specific cytokines are also aberrant in SLE. The presence of IL-1, IL-6 and IFN-gamma in involved kidneys suggests that they have local pathogenic effects. Moreover IFN-gamma, IL-6 and IL-1 modulate spontaneous IgG production by SLE mononuclear cells. During the next several years, the exact role of these cytokine in the pathogenesis of lupus become more fully elucidated.

Animals↗
PubMedDate not supplied

[Neonatal lupus erythematosus].

The neonatal lupus erythematosus syndrome, first described by McCuistion and Schoch in 1954, is associated with characteristic skin lesions and congenital heart block in the new-born, and the presence of Ro-(SSA), La-(SSB), or RNP antibodies in mothers and infants. A transplacental transference of maternal autoantibodies is discussed as possible pathophysiologic mechanism in neonatal lupus. The symptoms, the onset, and recently published pathogenetic concepts are reviewed.

Antibodies, Antinuclear↗
PubMed1996

Necrotizing lymphadenitis associated with systemic lupus erythematosus.

OBJECTIVE: Systemic lupus erythematosus (SLE) may have protean manifestations, including necrotizing lymphadenitis. After describing an illustrative case, we discuss the incidence, clinical features, and pathologic findings of SLE-associated necrotizing lymphadenitis. METHODS: A case of SLE associated with necrotizing lymphadenitis is reported. The patient's clinical presentation, course, and response to therapy is detailed. The literature on lupus lymphadenitis is reviewed. RESULTS: A young man who presented with a febrile illness characterized by multifocal necrotizing lymphadenitis is described. Glomerulonephritis, meningo-encephalitis, pericarditis, and hemolytic anemia evolved. The diagnosis of SLE was based on the clinical features, positive antinuclear antibody (ANA), and characteristic renal biopsy. High dose corticosteroids and cyclophosphamide induced a complete remission. In recent series from the literature the prevalence of lymphadenopathy was 12% to 59% of patients with SLE. The most common nodal groups involved were cervical (43%), mesenteric (21%), axillary (18%), and inguinal (17%). Lymph node pathology was characterized by paracortical foci of necrosis and infiltration by histiocytes, lymphocytes, plasma cells, and immunoblasts. The hematoxylin body, an amorphic aggregate of basophilic material, was pathognomonic of lupus lymphadenitis. The necrotizing lymphadenitis of SLE is pathologically similar to Kikuchi-Fujumoto disease (KFD), a distinctive, self-limited form of necrotizing lymphadenitis. The pathologic and clinical literature support a close link between SLE and KFD. CONCLUSIONS: SLE can be complicated by necrotizing lymphadenitis, with distinctive pathologic features. Lupus lymphadenitis and KFD share some common clinical and pathologic features, supporting a relationship between the disorders.

Adult↗