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PubMed1982

Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of lupus erythematosus.

We studied the autoimmune serologic features and histocompatibility antigen associations of 27 patients who had a widespread, nonscarring and often photosensitive form of histologically specific cutaneous lupus erythematosus. We designated this disorder as subacute cutaneous lupus erythematosus. Skin lesions from this disorder can be distinguished from scarring discoid lupus erythematosus lesions both on a morphologic and histopathologic basis. Antinuclear and anticytoplasmic antibodies (Ro or Ro and La) and circulating immune complexes were frequently present in patients with subacute cutaneous lupus erythematosus, whereas rheumatoid factor and anti-lymphocyte, anti-DNA, anti-nRNP, and anti-Sm antibodies were found less frequently. Patients having annular skin lesions represented a particularly homogeneous subgroup in which there was a striking concordance of anti-Ro antibodies and the HLA-DR3 phenotype. These studies clearly establish that the presence of these lesions can serve as a cutaneous marker for a distinct subset of patients with lupus erythematosus who share similar clinical, serologic, and genetic features.

Adult↗
PubMed2003

Lupus mucinosis: a case report and review of cutaneous lupus.

Lupus mucinosis (LM) is a rare disorder found only in patients with systemic lupus erythematosus (SLE) or discoid lupus erythematosus (DLE). We describe a patient with lupus mucinotic nodules as the initial presenting sign of SLE. We further discuss other forms of cutaneous lupus and contrast them with our findings.

Ankle↗
PubMed2004

[Significance of serum anti-Clq Ab in evaluation of lupus nephritis activity and its curative effects of pharmacotherapy on lupus nephritis].

OBJECTIVE: To investigate the significance of serum anti-C1q Ab of evaluation of lupus nephritis activity and its curative effects of cyclophophamide therapy on lupus nephritis (LN). METHODS: The level of serum anti-C1q antibody of 75 patients with LN was examined by enzyme-linked immunosorbent assay (ELISA) of the 75 patients the incipient cases had never received corticosteroid and immunosuppressant and the recurrent cases had stopped the immunosuppressant treatment for more than 3 months and were treated, if so, with prednisone with the dosage </= 10 mg/d. Thirty-one patients underwent renal biopsy. The patients underwent treatment of cyclophophamide. The relationships between serum anti-C1q Ab level and lupus nephritis activity, renal pathohistology, as well as laboratory parameters were analyzed, followed by further regular follow-up to investigate its influence to the curative effect. RESULTS: Fifty-five of the 75 patients (73.3%) were anti-C1q Ab positive with the level of (92 U/ml +/- 41 U/ml). The mean time of conversion of urinary protein into negative was 9 months in the positive C1q positive group and 6 months in the C1q negative group. One year after, 25% of those with positive C1q antibody failed to convert into urinary protein negative, and 90% of those with negative C1q antibody converted into urinary protein negative. One year after, 32% of those whose serum C1q antibody remained positive 1 month after the treatment failed to convert into urinary protein negative, and 91% of those whose serum C1q antibody remained positive 1 month after the treatment converted into urinary protein negative. The serum anti-C1q Ab level was positively correlated with the lupus nephritis clinical active index, proteinuria, and titer of anti-dsDNA, and was negatively correlated with the levels of serum C3 and C4. Renal biopsies showed a positive correlation between the serum anti-C1q Ab level and the activity index of renal pathohistology. Multivariate analysis showed that the serum anti-C1q Ab level after treatment were associated with the curative effect and prognosis of LN. CONCLUSION: Serum anti-C1q Ab is not only a good index of lupus nephritis activity, but also reflects renal involvement and curative effect. That serial measurement of serum anti-C1q Ab may provide better clinical strategies for the therapy.

Adult↗
PubMed1987

Biological and clinical heterogeneity of lupus and lupus-like anticoagulant in fifty-seven patients.

A lupus or lupus-like inhibitor was detected in 57 patients: 24 systemic lupus erythematosus, 9 autoimmune diseases, 10 lymphoproliferative disease, 11 miscellaneous diseases and 3 asymptomatic patients. No hemorrhagic diathesis was observed in spite of major surgery. Thromboembolism occurred in 19 patients. Among them, 5 patients had recurrent abortions. An extensive study of coagulation profile compared different assays to investigate lupus-like inhibitor: the most sensitive assay was the partial thromboplastin time performed without activator. When performed with kaolin, it was the only assay detecting the lupus cofactor. Prothrombin time was prolonged in only 53% of the patients. Factors VIII, IX, XI and XII were in the normal range in 40% of the patients. When decreased, apparent deficiencies were usually not detectable on further dilutions of the test samples. In 7 patients factor XII antigen and activity were both decreased, suggesting an apparent factor XII deficiency. No relationship was observed between thromboembolic events, underlying disease or biological pattern.

Adolescent↗
PubMed1984

Silent lupus nephritis among patients with discoid lupus erythematosus.

A kidney biopsy was performed in 7 hypocomplementemic discoid lupus erythematosus patients despite the absence of overt renal involvement. Five patients had glomerular immune deposits and 2 patients with disseminated discoid lupus erythematosus exhibited definite proliferative glomerulonephritis. Those findings show that silent lupus nephritis may be encountered in discoid as well as in systemic lupus erythematosus, providing additional evidence supporting the unity of the disease. We suggest that hypocomplementemic patients with discoid lupus erythematosus must be carefully screened for renal disease by periodic urinalysis examinations.

Complement C3↗
PubMed1993

Blood levels of sex hormone in lupus nephritis and their relationship to lupus activity.

One hundred and fifty-nine patients (140 females, 19 males) with lupus nephritis (LN) were studied. Renal biopsy was performed for each patient with light microscopy, immunofluorescence and electronic microscopic studies. 27 normal subjects were selected as controls. Blood levels of sex hormone including follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2) and testosterone (T) were measured by radioimmunoassay (RIA), and some clinical, laboratory and histological parameters were collected for analysis of lupus activity. It was found that serum FSH, LH and plasma E2 of all patients were much higher than normal (P < 0.01) while plasma T was much lower in female patients (P < 0.01) but normal in male patients. The lupus activity was proved to be closely related to plasma E2 and T (P < 0.05). There was no definite relationship between abnormal plasma E2 or T and serum IgG, C3 C4, ANA, A-dsDNA or A-Sm. The levels of sex hormones among every histological type of LN were not significantly different. It was concluded that: 1. The increment of plasma E2 is associated with lupus activity in both male and female patients. 2. The decrement of plasma T only in female patients is also related to lupus activity. 3. No relationship could be found among FSH, LH, E2 and T.

Adolescent↗
PubMed1995

Diagnostic evaluation of the lupus band test in discoid and systemic lupus erythematosus.

BACKGROUND: The usefulness of the lupus band test (LBT) in the diagnosis of cutaneous lupus erythematosus remains controversial. The study was done to determine the sensitivity, specificity, and predictive value of the LBT in discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE). METHODS: During the study period, 32 patients with SLE and 28 patients with DLE were included. The final classification of cases was based on the American Rheumatism Association Criteria (ARA) for SLE, histopathology, and consensus of the clinical staff. Thirty controls, 15 each for DLE and SLE, were chosen from a group of patients, who had lesions clinically simulating DLE and SLE, but could be excluded by histopathology, laboratory tests, and follow-up. Histopathologic examinations and direct immunofluorescence (DIF) tests were done on lesional skin. RESULTS: In DLE, the sensitivity of the LE band test was 58% and the specificity 87%. The positive predictive value was 95% and negative predictive value was 32%. In SLE the sensitivity was 93% and specificity 87%. The positive predictive value was 64% and negative predictive value 98%. CONCLUSIONS: The high negative predictive value of LBT in SLE suggests that it is valuable in excluding diseases clinically similar to SLE.

Adolescent↗
PubMed2003

Anti-dsDNA, anti-Sm antibodies, and the lupus anticoagulant: significant factors associated with lupus nephritis.

BACKGROUND: Lupus nephritis (LN) is a common manifestation in patients with systemic lupus erythematosus (SLE). Autoantibodies and ethnicity have been associated with LN, but the results are controversial. OBJECTIVE: To study the immunological and demographic factors associated with the development of LN. PATIENTS AND METHODS: A retrospective case-control study of 127 patients with biopsy-proven LN, and 206 randomly selected patients with SLE without nephritis as controls was designed. All patients had attended our lupus unit during the past 12 years. Standard methods were used for laboratory testing. RESULTS: Patients with LN were significantly younger than the controls at the time of SLE diagnosis (mean (SD) 25.6 (8.8) years v 33.7 (12.5) years; p<0.0001). The proportion of patients of black ethnic origin was significantly higher in the group with nephritis (p=0.02). There were no differences in sex distribution or duration of follow up. A higher proportion of anti-dsDNA, anti-RNP, anti-Sm, and lupus anticoagulant (LA) was seen in the group with nephritis (p=0.002; p=0.005; p=0.0001; p=0.01, respectively). In univariate, but not in multivariate, analysis male sex and absence of anti-dsDNA were associated with earlier onset of renal disease (p=0.03; p=0.008). In multivariate analysis the only factors associated with nephritis were younger age at diagnosis of SLE, black race, presence of anti-dsDNA, anti-Sm, and LA. No demographic or immunological associations were seen with WHO histological classes. CONCLUSIONS: Young, black patients with anti-dsDNA, anti-Sm antibodies, and positive LA, appear to have a higher risk of renal involvement. These patients should be carefully monitored for the development of LN.

Adult↗
PubMed2002

Hair dye treatment use and clinical course in patients with systemic lupus erythematosus and cutaneous lupus.

The etiological role of hair dye treatment (HDT), some of them such as permanent hair dyes containing aromatic amines, in the development of SLE has been previously ruled out. However, the possible influence of HDT use on the course and prognosis of lupus patients has been assessed only in one short-term study. Since HDT is very extensive among the population, the knowledge of this possible negative effect may be very important. Thus, the aim of this study was to assess the long-term influence of several HDTs on the course and clinical severity of patients with both systemic lupus erythematosus (SLE) and cutaneous lupus (CL). In this longitudinal case series study, 91 SLE patients and 22 CL patients were prospectively studied from October 1988 to May 2000. They were divided into three groups: (a) non-HDT users--patients who have never used HDT (n = 65); (b) P-HDT users--HDT permanent type users, alone or in combination with other types of HDT (n = 28); (c) non P-HDT--users of other treatments different from permanent tinting (bleach, lowlights, etc; n = 20). In each patient we determined: (1) number of flares/year in SLE patients and worsening of cutaneous lesions for CL; (2) Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) damage index; (3) predominant damaged organs/systems according to the HDT use and type of HDT; and (4) subjective impression about the disease evolution in relation to HDT use. No significant differences were found with respect to flares/year and SLICC/ACR damage index between the study groups. Non-HDT group presented more renal involvement and serositis than both HDT-user groups. No patient related the HDT use to the worsening of his disease. Therefore, in this study no evidence of an association between the long-term use of several types of HDT and the clinical activity and course of SLE and CL was found.

Adult↗
PubMed2000

The lupus erythematosus cell phenomenon: comparative analysis of antichromatin antibody specificity in lupus erythematosus cell-positive and -negative sera.

OBJECTIVE: To compare and investigate antihistone and antichromatin antibody responses as well as clinical variables in patients with systemic lupus erythematosus (SLE) who were either positive (LEC+) or negative (LEC-) for the lupus erythematosus (LE) cell phenomenon. METHODS: The binding properties of LEC+ and LEC- SLE sera to chromatin-associated nuclear antigens (histones H1, H2A, H2B, H3, H4; complexes of H2A-H2B, [H2A-H2B]-DNA, H1-DNA; total and H1-stripped chromatin; native and denatured DNA) were investigated. In addition, sera from patients with drug-induced lupus (by procainamide, hydralazine, or quinidine), as well as from patients with rheumatoid arthritis and osteoarthritis, were assessed. Enzyme-linked immunosorbent assay was used to detect specific antibody binding. RESULTS: Mirroring the important role of histone H1 in the formation of LE cells, anti-histone H1 reactivity was 8-fold higher in LEC+ sera than in LEC- sera. In addition, reactivities to most of the other antigens tested, i.e., other histones and histone-DNA complexes as well as chromatin and DNA, were significantly higher in LEC+ sera than in LEC- sera. All but 1 serum sample from the patients with drug-induced lupus were negative for LE cell formation as well as for anti-histone H1 reactivity, but displayed high antibody reactivities to histone-DNA complexes, including chromatin. Sera from patients with rheumatoid arthritis and osteoarthritis did not show significant binding to these antigens. When comparing the clinical features of LEC+ and LEC- SLE patients, severe organ involvement, including nephritis and central nervous system involvement, was common in the LEC+ group, but rare in the LEC- group. CONCLUSION: A positive LE cell phenomenon not only correlated with the presence of high anti-histone H1 antibody levels in SLE, but also indicated serologically and clinically active disease with major organ involvement.

Antibody Specificity↗
PubMed2006

Prevalence of conventional and lupus-specific risk factors for cardiovascular disease in patients with systemic lupus erythematosus: A case-control study.

OBJECTIVE: Patients with systemic lupus erythematosus (SLE) are significantly more likely to experience a myocardial infarction or a stroke than age-matched controls. We compared the prevalence of conventional and lupus-specific risk factors in patients with SLE just before a cardiovascular event and in matched controls with SLE but no cardiovascular disease (CVD). METHODS: Twenty-nine patients with SLE and CVD were enrolled. For each patient, 2 ethnically- and sex-matched controls were obtained, 1 matched for age and 1 for SLE duration. Data regarding risk factors were collected for the time immediately preceding the relevant cardiovascular event, or at an equivalent time for controls. RESULTS: Patients' median age at event was 49 years (interquartile range 43-54 years) and mean disease duration was 12.0 +/- 7.1 years. Patients with SLE and CVD were more likely than both age and duration controls to be treated for hypertension (P = 0.01 and P = 0.001, respectively) and to have elevated triglyceride levels (P = 0.05 and P = 0.01, respectively). Compared with duration controls, CVD patients were more likely to have lupus anticoagulant (P = 0.03), but less likely to be receiving treatment with hydroxychloroquine (P = 0.003). Compared with age controls, patients were more likely to be current smokers (P = 0.03), to have taken a mean dosage >7.5 mg/day of prednisolone (P = 0.04), and to have been treated with pulsed methylprednisolone (P = 0.03). In multivariable analysis, only hypertension treatment was an independent risk factor for CVD. CONCLUSION: We identified significantly increased prevalence of some conventional and lupus-specific risk factors in patients with SLE immediately before a CVD event compared with controls matched for age or disease duration.

Adult↗
PubMed1994

Contemporary concepts for the clinical and laboratory evaluation of systemic lupus erythematosus and "lupus-like" syndromes.

Systemic lupus erythematosus (SLE) is a nonorgan-specific autoimmune disease which affects multiple organ systems and is multifactorial in etiology. SLE is the prototypic systemic rheumatic disease with immune dysregulation characterized by (1) polyclonal activation of B-cells and (2) production of a large spectrum of autoantibodies with a marked preference for nuclear and intracellular antigens. The clinical and laboratory manifestations and criteria for classification and diagnosis of systemic lupus erythematosus, lupus-like syndromes, and various subsets of systemic lupus erythematosus, are reviewed. The differential diagnosis of SLE and related diseases is described with correlation of specific intracellular autoantibodies.

Antibodies, Antinuclear↗
PubMed1989

Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome.

PURPOSE AND PATIENTS AND METHODS: Antiphospholipid antibodies, lupus anticoagulant antibodies to cardiolipin, and a false-positive result on testing for syphilis have been linked to thrombotic vascular occlusions, particularly in patients with systemic lupus erythematosus (SLE) or lupus-like disease, i.e., patients not fulfilling four American Rheumatism Association criteria for the classification of SLE. The clinical and serologic features of 35 patients with cerebrovascular disease (strokes/transient ischemic attacks) who demonstrated antibodies to phospholipids are presented. Complete histories were obtained from all 35 patients, and all underwent routine physical examinations, radiography, electrocardiography, computed tomographic brain scanning, and immunologic studies. Psychometric tests were performed in nine patients. RESULTS: The strokes were often multiple and were followed by multi-infarct dementia in nine patients. Of particular interest were 10 patients in whom the presence of antiphospholipid antibodies was the major and often the sole immunologic disturbance present. Several of these patients were antinuclear antibody-negative, and the antinuclear antibodies, when present, were usually of a low titer (1:40 to 1:160). These patients conform to a group classified as having a primary antiphospholipid syndrome. CONCLUSION: Antiphospholipid antibodies are strongly associated with cerebrovascular occlusions in patients with SLE as well as in those with lupus-like disease and the primary antiphospholipid syndrome. All patients with any of these conditions who present with vascular events should be screened for these antibodies, as their occurrence may have a bearing on future therapy.

Adult↗
PubMed1992

Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features.

Sixty-seven patients with cutaneous lupus erythematosus (CLE) were followed up as part of a series of 570 lupus erythematosus patients seen in a private practice between 1980 and 1989. Clinical and laboratory features, treatment, and natural course were observed. Findings of interest included (1) a ratio of at least one CLE case for every seven cases of systemic lupus erythematosus (SLE); (2) occurrence of CLE in fewer women and apparently associated with an older age at diagnosis than SLE; (3) similar frequency of cutaneous lupus subsets in CLE and SLE; (4) strong family history for SLE but not CLE in CLE patients; (5) other cutaneous and musculoskeletal features in a majority of CLE patients and constitutional symptoms in 10%; (6) positive ANA titers, high sedimentation rates, and leukopenia common in CLE; (7) anticardiolipin antibody in 31% of CLE patients but not associated with systemic complications; (8) antimalarials required by 75% of patients and systemic steroids by 33%; and (9) an excellent prognosis associated with CLE, organ-threatening disease being rare.

Adult↗
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↗
PubMed1998

Dermatologic lupus: Hopkins Lupus Cohort.

Dermatologic manifestations are among the most common signs of systemic lupus erythematosus (SLE). The Hopkins Lupus Cohort study is a prospective study in which patients with SLE are seen on a quarterly basis for measurement of disease activity, laboratory tests, and assessment of morbidity and quality of life. This cohort has allowed unique insights into the epidemiologic factors of SLE, the presentation of dermatologic lupus, and morbidity, all of which are presented in this report. In addition, the dermatologic signs of antiphospholipid antibody syndrome (APS) are reviewed. Approaches to treatment of dermatologic lupus and APS are discussed.

Adult↗
PubMed1994

Evaluation of laboratory tests for lupus anticoagulant in a group of Chinese lupus patients.

The sera of 69 Chinese patients with systemic lupus erythematosus were tested for the presence of lupus anticoagulant (LA) by a panel of laboratory tests: Kaolin clotting time (KCT), dilute Russell viper venom time (DRVVT) and platelet neutralization procedure (PNP). The prevalence of LA varied among the 3 tests (10-19%), and was 10% when LA was considered present if either KCT or DRVVT and the PNP were positive. Concordance was fair between KCT and PNP, but was poor for DRVVT with either of the other 2 tests. Only 2 of our lupus patients had a history of thrombo-embolic disease, and neither were serologically positive for LA. The incidence of thrombo-embolic diseases and that of LA were both too low in this group of Chinese lupus patients for their association to be evaluated.

Adolescent↗
PubMed1991

Renal transplantation for systemic lupus erythematosus and recurrent lupus nephritis. A single-center experience and a review of the literature.

Prior to 1975 patients with systemic lupus erythematosus were generally not considered candidates for renal transplantation because of concern that immune complex deposition would rapidly destroy the allograft. However, recent evidence suggests that good patient and graft survival rates can be achieved comparable to other renal diseases. Between September 23, 1963 and July 31, 1990, 1070 renal transplants were performed at Washington University Medical Center (WUMC). During this period, 14 patients with SLE (12 female and 2 male) received 16 renal transplants (7 living-related donor [LRD], 1 living-unrelated donor [LURD], and 8 cadaver [CAD]). The mean age at the time of the first transplant was 32.5 +/- 10.3 years. The duration of disease prior to transplant was 88.0 +/- 45.9 months and the duration of hemodialysis prior to transplant was 36.0 +/- 33.7 months. Of these patients, 7/14 (50%) had negative and 3/14 (21%) positive SLE serology pre- and post-transplant, 3/14 (21%) had negative serology pretransplant that became positive posttransplant, and 1/14 (2%) was positive pretransplant and became seronegative posttransplant. Patient survival was 92.8% (13/14), and of the 16 kidneys transplanted 62.5% (10/16) are still functioning with a mean follow-up period of 43.7 +/- 45 months. The current mean serum creatinine was 1.4 +/- 0.26 mg/dl. One noncompliant patient developed recurrent lupus nephritis bringing the total number of cases reported in the literature to seven. The present study demonstrates that patients with SLE can be transplanted with excellent patient and graft survival and function and a low rate of recurrent lupus nephritis. From a review of the literature, there appears to be an association between positive SLE serology pre- and posttransplant and recurrent lupus nephritis.

Adult↗