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PubMed1993

Bullous systemic lupus erythematosus. Report of a case with lupus erythematosus cells in the dermis.

A 20-year-old black patient with bullous systemic lupus erythematosus developed papular and vesicular lesions on the extensor surfaces of the extremities. Histologically, subepidermal blisters and papillary neutrophilic abscesses with a striking number of lupus erythematosus cells were observed. No circulating anti-basal-membrane-zone antibodies were found. By Western immunoblotting, the patient's serum showed no reactivity against epidermal or dermal extracts.

Adult↗
PubMed1977

Problems in diagnosis and treatment of lupus psychosis. Report of a patient with systemic lupus erythematosus, Hashimoto's thyroiditis, and Sjögren's syndrome.

A 34-year-old woman with an organic psychosis was presumed to be suffering from corticosteroid psychosis because of prednisone treatment for urticaria. Lupus was found to be the cause of both urticaria and psychosis. The lupus psychosis was reversed when sufficiently high doses of steroids were given, in combination with immunosuppressant agents.

Adult↗
PubMed2000

Systemic lupus erythematosus in three ethnic groups. VI: Factors associated with fatigue within 5 years of criteria diagnosis. LUMINA Study Group. LUpus in MInority populations: NAture vs Nurture.

OBJECTIVE: To determine the frequency, degree and associated features of fatigue among Hispanic (H), African American (AA) and Caucasian (C) patients with recent onset (< or = 5 yr) systemic lupus erythematosus (SLE) at their baseline evaluation. METHODS: H (n = 69), AA (n = 83) and C (n = 71) patients from the LUMINA (LUpus in MInority populations: NAture vs Nurture) cohort were studied. Fatigue [Fatigue Severity Scale (FSS)] was defined as present if FSS score > or = 3.0. Variables from functional, clinical, sociodemographic, health behaviors, behavioral and psychological and immunogenetics domains were ascertained at study entry. Associations were examined using regression models. RESULTS: Eighty-six percent (85.7%) of patients reported having fatigue (82.6% H; 85.5% AA; 88.7% C); median FSS score, 5.3. Factors from the psychological and clinical domains were primarily associated with FSS; immunogenetic (HLA Class II phenotypes) features were not. Increased fatigue was strongly associated with decreasing function, both physical and mental. Variables associated with significantly greater degree of fatigue at baseline in the multivariable stepwise model in order of decreasing additional partial R2 explained included: abnormal illness-related behaviors, older age, higher self-reported pain, greater degree of helplessness, greater disease activity, Caucasian race, and lacking health insurance (model R2 = 37%). CONCLUSIONS: Fatigue is one of the most prevalent clinical manifestations of SLE across all ethnic groups. The perception of fatigue severity in SLE may be multifactorial in origin, including psychosocial factors and disease activity. If these prove causal, knowledge of their contribution may suggest therapeutic and/or behavioral interventions, which could ameliorate this pervasive and often incapacitating symptom of SLE.

Black or African American↗
PubMed2001

A case of systemic lupus erythematosus complicated with multicentric reticulohistiocytosis (MRH): successful treatment of MRH and lupus nephritis with cyclosporin A.

We document the first case of a patient who manifested systemic lupus erythematosus (SLE) complicated with multicentric reticulohistiocytosis (MRH). Neither intravenous steroid nor cyclophosphamide (CY) pulse therapies were fully effective against multiple MRH-related tumors that appeared on the left ankle joint and interphalangeal joints of both hands. In contrast, treatment with cyclosporin A (CyA) resulted in a marked regression of these nodules within one month, together with a complete remission of both MRH and lupus nephritis. We propose CyA as an alternative choice for the treatment of MRH.

Adult↗
PubMed2001

Reliability and validity of systemic lupus activity measure-revised (SLAM-R) for measuring clinical disease activity in systemic lupus erythematosus.

New clinical scales for semiquantitating disease activity in systemic lupus erythematosus (SLE) are widely used in research. They are reliable and valid measures. One of the original scales, the Systemic Lupus Activity Measure (SLAM), has been modified based on experience with it in multi-observer studies and training of individuals in its use. We tested the psychometric properties of the revised SLAM (SLAM-R). SLAM-R was tested on 30 SLE patients, who fulfilled 1997 revised ACR criteria and were selected to represent a range of disease activity. The patients were evaluated independently by two physicians, who studied the instruction booklet and who had never used SLAM-R, on two occasions 2-4 weeks apart. At the first visit, the physician's global assessment of activity using visual analog scale, anti-dsDNA Ab, C3 and C4 were checked for construct validity. The psychometric properties were analyzed with nested analysis of variance and Pearson's correlation coefficient using SAS. All patients were female, the median age was 31 (15-52) y, and the mean score of SLAM-R was 10.5 +/- 5.3 (3-26). Estimates of reliability were 0.78 of inter-rater, 0.61 of inter-visit, 0.76 of physician 1 between visits, and 0.56 of physician 2 between visits. Among subcategories except 'Eye,' the 'Gastrointestinal' category had the highest (0.96) and the 'Neuromotor' category had the lowest inter-rater reliability (0.50). With respect to construct validity, the correlation of SLAM-R scores with the disease activity variables except C4 was high and statistically significant. In conclusion, the SLAM-R is reliable and valid for measuring clinical disease activity in SLE.

Adolescent↗
PubMed2005

Mortality and cancer incidence in Korean patients with systemic lupus erythematosus: results from the Hanyang lupus cohort in Seoul, Korea.

The rates of mortality and cancer have not been compared between Korean systemic lupus erythematosus (SLE) patients and the general population. The objective of this study was to compare the observed mortality and cancer incidence rates of SLE patients with the expected age-, sex- and calendar-matched incidence rates of a comparable general control population. This study included 466 SLE patients who were in the Hanyang Lupus Cohort, Seoul and we excluded the males from the analyses because the number of males was too small to expect any incidence of cancer or death. We used the national female mortality rates from 1992 to 2001 as recorded by the Korean National Statistical Office as the standard mortality rates, and the Seoul Cancer Registry data as the standard cancer incidence rate. Ten of the females in our study died, giving a crude mortality rate of 596.0/100 000 person-years (PY) and a standardized mortality ratio (SMR) of 3.02. The crude cancer incidence was 179.3/100 000 PYand the standard incidence ratio was 1.04. These results demonstrate that the SMR is significantly higher in SLE patients than in the general population, whereas the incidence of cancer is the same in these two groups.

Adolescent↗
PubMed1999

Systemic lupus erythematosus in three ethnic groups: III. A comparison of characteristics early in the natural history of the LUMINA cohort. LUpus in MInority populations: NAture vs. Nurture.

AIM: To determine and contrast the socioeconomic-demographic and clinical features of patients with recent onset (< or =5 y) systemic lupus erythematosus (SLE) from three ethnic groups, Hispanic, African-American and Caucasian (H, AA, C). SUBJECTS AND METHODS: SLE cases (American College of Rheumatology criteria) (incident (n = 56), prevalent (n = 173)), were enrolled in a longitudinal study at The University of Alabama at Birmingham, The University of Texas-Houston Health Science Center and The University of Texas Medical Branch at Galveston. Socioeconomic-demographic, clinical, immunological, behavioral and psychological data were obtained using validated instruments and standard laboratory techniques, and compared. RESULTS: 70 H, 88 AA and 71 C SLE patients constitute this cohort. H and AA patients were younger and of lower socioeconomic-demographic status. They also had evidence of more frequent organ system involvement (renal, cardiovascular), more auto-antibodies, more active disease (after adjusting for discrepant socioeconomic-demographic features), lower levels of social support and more abnormal illness-related behaviors (more in H than in AA). H also were more likely to have an abrupt disease onset; C were more likely to be on antimalarials but less likely to be on corticosteroids. H, AA, and C used health care resources comparably. They had similar levels of pain and physical and mental functioning after adjusting for age, disease duration, income, education, social support, illness-related behaviors, and Systemic Lupus Activity Measure or SLAM scores. CONCLUSIONS: H and AA patients have more active SLE, at an earlier age of onset, and a less favorable socioeconomic-demographic structure (worse among the H than AA) which predispose them to a less favorable natural history.

Adolescent↗
PubMed1977

Lupus erythematosus cells in pleural effusion: the initial manifestation of procainamide-induced lupus erythematosus.

A 63-year-old man developed an asymptomatic pleural effusion following the administration of 500 gm of procainamide hydrochloride over a six-month period. The diagnosis was initially suggested by the finding of lupus erythematosus cells in the pleural fluid. Lupus erythematosus cells and antinuclear antibodies appeared in the blood two months later and remained for a period of six months. The diagnosis was corroborated by the presence of antibodies to denatured DNA, but not to native DNA.

Antibodies, Antinuclear↗
PubMed2005

Abetimus sodium: a new therapy for delaying the time to, and reducing the incidence of, renal flare and/or major systemic lupus erythematosus flares in patients with systemic lupus erythematosus who have a history of renal disease.

The safety and efficacy of abetimus sodium (abetimus) has been evaluated in 13 controlled and uncontrolled clinical trials involving > 800 patients or subjects. The two pivotal trials enrolled a total of 547 patients with systemic lupus erythematosus (SLE) who had a history of lupus nephritis. Evidence of clinical effectiveness of abetimus comes from analyses of data from patients with SLE and high-affinity antibodies to the DNA epitope of abetimus at baseline; a retrospective subgroup in the pivotal Phase II/III LJP-394-90-05 trial (90-05 trial) and the intent-to-treat population in the Phase III LJP-394-90-09 trial (90-09 trial). These studies enrolled SLE patients who had experienced prior renal manifestations of their disease and had elevated anti-dsDNA antibodies at baseline by Farr assay. Both were long-term studies, with a mean duration of treatment participation of 371 days in 90-05 and 310 days in 90-09 for the population of patients with high-affinity antibodies at baseline. The 90-05 and 90-09 studies, as well as all other clinical studies of abetimus, consistently showed that treatment with abetimus resulted in durable and persistent reductions in anti-dsDNA antibodies in SLE patients. Treatment with abetimus was associated with statistically significant decreases in anti-dsDNA antibody levels from baseline compared with placebo in both the 90-09 and 90-05 trials. Positive trends were noted for the incidence of renal flares and major SLE flares in patients treated with abetimus. Abetimus appeared to be well tolerated for treatment periods of < or = 22 months.

Clinical Trials as Topic↗
PubMed2002

Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity.

Pulmonary hypertension (PH) sometimes occurs in patients with systemic lupus erythematosus (SLE). We report a case of 51-year-old-woman with PH associated with SLE. She had been diagnosed as SLE on the basis of pericardial effusion, hematological disorder, positive antinuclear antibody, and hypocomplementemia. Despite minimal lupus activity, she had marked elevation of pulmonary arterial pressure (101/53 mmHg) and decreased cardiac index (1.5 l/min/m2). Symptoms related to PH were progressive under treatment with oral corticosteroids, oxygen, calcium antagonists, and warfarin. After 17 months of epoprostenol treatment, she died of pulmonary infarction. SLE-associated PH is often severe and progressive even in association with minimal activity.

Anticoagulants↗
PubMedDate not supplied

Systemic lupus erythematosus presenting with pericardial tamponade and lupus pneumonitis.

A previously-well 37-year-old woman presented with pericardial tamponade and pulmonary infiltrates. Systemic lupus erythematosus was diagnosed. Pericardiocentesis and systemic treatment with prednisolone produced dramatic improvement with no recurrence. The patient underwent bronchoalveolar lavage and transbronchial lung biopsy, which established the diagnosis of lupus pneumonitis.

Adult↗
PubMed1991

Plasmapheresis does not increase the risk for infection in immunosuppressed patients with severe lupus nephritis. The Lupus Nephritis Collaborative Study Group.

OBJECTIVE: To determine whether plasmapheresis increases the risk for infection in immunosuppressed patients. DESIGN: Randomized, controlled trial. SETTING: Multicenter. PATIENTS: Eighty-six patients enrolled in a trial of plasmapheresis for severe diffuse proliferative lupus nephritis. INTERVENTIONS: Forty-six of the patients received high-dose steroid therapy plus cyclophosphamide therapy for 8 weeks. Thereafter, cyclophosphamide therapy was discontinued, and steroid therapy was tapered (standard treatment group). Forty patients received identical treatment and had 12 plasmapheresis procedures during the first 4 weeks of the treatment. MEASUREMENTS: Patients were examined for the development of infection. MAIN RESULTS: No statistical difference in age, sex, race, serum creatinine level, proteinuria, or complement levels was found between the two groups. Over a follow-up period of 5376 patient-weeks, 74% of patients in the standard treatment group had 62 infections, yielding an aggregate infection rate of 1.15 infections per 100 weeks (median individual infection rate, 1.08; 25th and 75th percentiles, 0.0 and 2.44). This rate was comparable to that seen in the plasmapheresis-treated patients who were followed for 4187 patient-weeks: 68% had 51 infections, for an aggregate infection rate of 1.22 infections per 100 weeks (median individual infection rate, 0.94; 25th and 75th percentiles, 0.0 and 2.32). The infection rate was also comparable in the initial acute phase of the study, despite the fact that patients who received plasmapheresis then had significantly lower immunoglobulin (IgG) levels (P less than 0.001). Neither the site (superficial compared with systemic) nor the nature (conventional compared with unconventional) of infection differed statistically between the two groups. Of 14 patient deaths, 7 were from infection (4 in control group and 3 in the plasmapheresis group). CONCLUSION: Plasmapheresis did not increase the risk for infection in immunosuppressed patients with severe lupus nephritis.

Adult↗
PubMed1976

[A special form of lupus disease. Deep cutaneous lupus. Apropos of 2 cases].

Deep cutaneous lupus is a clinical form of lupus disease for which a very old description has recently been brought up to date. Two new cases are reported here and a review of the literature makes it possible to analyse the clinical, biochemical, histological, and immunological features of this panniculitis. Different from the Weber-Christian syndrome, it is characterized by repeated eruptions of nodules and/or subcutaneous plaques, and histologically by vasculitis, lymphocyte infiltration, and sometimes te presence of immunoglobulins on the basal dermal membrane and around the vessels.

Adipose Tissue↗
PubMedDate not supplied

[Psychosis as the initial manifestation of systemic lupus erythematosus: the role of lupus band test and anti-ribosomal antibodies].

INTRODUCTION: Diffuse brain disease in systemic lupus erythematous (SLE) can be difficult to assess because of the sparse biological expression of the disorder, resulting in usually normal neuroimaging and laboratory findings. For this reason, it is likely that patients without a previous SLE diagnosis that presented initially with a pure psychiatric disorder, as psychosis or depression, can remain undiagnosed, and, in fact, they are exceptionally reported. As a biological marker, antiribosomal P protein antibodies have been closely related to SLE psychosis although their pathogenic role and specificity is under discussion. CLINICAL CASE: A young woman, without relevant medical history, presented with an acute psychotic catatonic picture, suspected organic in nature due to the existence of persistent mild CSF pleocytosis, while neuroimaging and laboratory studies were negative, including antinuclear and antiribosomal antibodies. Finally, a SLE diagnosis could be established because of a strong lupic band phenomenon in skin biopsy. CONCLUSIONS: It is important to maintain a high suspicion of SLE in acute psychotic patients, especially if atypical features are present. As the biological expression of the disorder in such cases may be elusive, we propose the study of lupus band in skin biopsy for these patients, regardless the absence of antiribosomal antibodies.

Adult↗
PubMed2000

The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index for Systemic Lupus Erythematosus International Comparison.

OBJECTIVE: To compare patients with systemic lupus erythematosus (SLE) from different centers with respect to demographics and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) scores, and to assess whether the SLICC/ACR DI changed over time, and whether initial DI scores were related to outcome. METHODS: Members of SLICC completed DI scores and patient demographics on patients followed in their centers. Information was provided at 2, 5-10, and > 10 years of followup. Data were entered on computer and analyzed on SPSS/PC+ and SAS using descriptive statistics and analysis of variance. RESULTS: Information for 1297 patients within 2 years of first clinic visit was submitted from 8 centers. There were 1187 women and 110 men with a mean age at diagnosis of SLE of 32 years. Seven hundred sixty-two were Caucasian, 423 were black, and the remainder were of other races. There were more blacks in the American centers than in Canadian or European centers. Five centers provided information for the 3 time periods. The DI increased over time. Ninety-nine patients had died. Higher SLICC/ACR DI scores were documented in patients who went on to die. CONCLUSION: The SLICC/ACR DI is a valid measure for damage in SLE.

American Medical Association↗
PubMed1975

Discoid lupus erythematosus as part of a larger disease spectrum. Correlation of clinical features with laboratory findings in lupus erythematosus.

This study compares the immunologic features of a homogeneous group of patients with discoid lupus erythematosus (DLE) strictly limited to the skin (group 1) with those of patients with active discoid skin lesions plus visceral involvement (group 2) and with those of lupus erythematosus (LE) patients with proliferative glomerulonephritis (group 3). Positive antinuclear antibody (ANA) was found in 4% of group 1, 93% of group 2, and 100% of group 3. Low total hemolytic complement (CH50) was found in 4% of group 1, 47% of group 2, and 100% of group 3. Antibodies to native DNA (nDNA) were not found in group 1, were rarely found in group 2, and were present in nearly all patients in group 3. No group 1 patient had subepidermal immunoglobulin deposits in normal skin, 20% of group 2 had this finding, and 100% of group 3 had this finding. The ability to develop chronic discoid skin lesions appears to be associated with a reduced incidence of immunologic parameters of disease activity. The data suggest that patients with active discoid skin lesions rarely have severe renal disease.

Antibodies, Antinuclear↗