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PubMed1990

[Chronic lupus erythematosus, subacute cutaneous lupus].

The cutaneous manifestations of lupus erythematosus (LE) are extremely varied, and they predominate among the clinical signs of chronic LE and subacute cutaneous LE. In chronic LE, typical lesions are found in regions exposed to sunlight and consist of erythema, hyperkeratosis and skin atrophy. The skin of patients with subacute cutaneous LE is also highly sensitive to light; the lesions consist of an annular or polycyclic or sometimes psoriasiform erythemato-squamous eruption observed mainly in uncovered areas. These dermatological forms are part of the wide spectrum of "lupus". Cutaneous manifestations are often associated with systemic signs of varying type and severity and with immunological abnormalities, such as antibodies to Ro in subacute cutaneous LE, which act as markers of the disease.

Adult↗
PubMed1989

Prognostic value of lupus band test in unexposed normal skin of patients with systemic lupus erythematosus.

Biopsy specimens of normal unexposed buttock skin were taken from 71 patients with systemic lupus erythematosus (SLE) for lupus band test (LBT). Correlation of positive incidence, fluorescent intensity, protein classes and numbers of LBT with SLE activity including active nephropathy were studied. Positive LBT results are associated with SLE disease activity, decreased CH50 and C3, ANA peripheral pattern and positive AdsDNA antibodies, but not with active nephropathy per se. Although LBT results of unexposed normal skin can reflect SLE activity, its prognostic value should not be overestimated.

Adolescent↗
PubMed1985

[Brain stem infarction, systemic lupus erythematosus and circulating lupus anticoagulant].

A 30 year old man admitted with a brain stem infarct presented with intellectual deterioration. A diagnosis of systemic lupus erythematosus (S.L.E.) was based on the presence of five A.R.A. criteria :photosensitivity, arthralgia, leukopenia and thrombopenia, false positive syphilitic serology, antinuclear factors on immunofluorescence. A lupus type circulating anticoagulant (L.C.A.) was no longer detected after corticotherapy. This appears to be a case of the "hematologic" form of S.L.E. in which the presence of L.C.A. predisposes towards thrombosis, not only of veins--which is typical--but also of arteries. These may be isolated and reveal the underlying disease as in the present case. The L.C.A. is an antibody possessing antiphospholipid specificity which explains the anticoagulant action in vitro and the thrombogenic effect in vivo. Some authors consider this to be sufficient justification for administration of anticoagulants and/or antiplatelet agents, but corticotherapy may be effective.

Adult↗
PubMed1979

Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset.

We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus (LE) that occurred in a characteristic distribution (subacute cutaneous lupus erythematosus [SCLE]). This clinically distinct form of cutaneous LE has not previously been analyzed as a separate entity and thus, its clinical importance has not been fully appreciated. We found that these patients frequently had a mild systemic illness marked by musculoskeletal complaints and serologic abnormalities. Forty-eight percent had systemic LE by American Rheumatism Association criteria; however, none had serious CNS or renal disease. Thus, those with SCLE are a subset of patients with LE who generally have an illness intermediate in severity between discoid LE and severe systemic LE.

Adult↗
PubMed1994

Systemic disease in subacute cutaneous lupus erythematosus: a controlled comparison with systemic lupus erythematosus.

OBJECTIVE: We sought to compare the prevalence of systemic disease in subacute cutaneous lupus erythematosus (SCLE) with systemic lupus erythematosus (SLE). METHODS: Clinical and laboratory features of 14 patients with SCLE were compared with 22 age and sex matched controls with SLE. RESULTS: Manifestations other than arthritis and skin disease were present in 11/14 (79%) SCLE cases compared with 19/22 (82%) SLE cases. Severe systemic disease occurred in one-half of patients with SCLE (5/7 men, 1/7 women), and most had the papulosquamous pattern. CONCLUSION: Systemic manifestations may be as varied in SCLE as in SLE, and severe disease is not unusual. In particular, men with papulosquamous SCLE may be at higher risk for severe extracutaneous disease.

Adult↗
PubMed1993

Lupus anticoagulant in Thai systemic lupus erythematosus patients.

In the past decade, interest in the potential clinical significance of lupus anticoagulant (LA) has grown tremendously. Recent reviews from the Western countries have found an average frequency of 34% for LA in patients with systemic lupus erythematosus (SLE). By using various laboratory procedures, namely, standard and diluted activated partial thromboplastin time, kaolin clotting time, tissue thromboplastin inhibition test and platelet neutralization test, we found the frequency of LA in 91 consecutive Thai SLE patients to be 17.5%, compared with 0.8% in the age-matched normal control population. The presence of LA was significantly associated with disease activity (p = 0.01). A statistically significant association was also observed between the presence of LA and convulsive disorders (p = 0.04), thrombocytopenia (p = 0.001) and autoimmune hemolytic anemia (p = 0.02).

Adolescent↗
PubMed1993

What is lupus? What is not lupus?

Present understanding suggests that lupus reflects a spectrum of syndromes that share many clinical, inflammatory, and immunologic features. Those patients not fulfilling criteria for systemic lupus erythematosus (SLE) do not seem to evolve frequently to classic SLE, tend to have a good prognosis, and may be managed conservatively. This is important for clinical management. Whether this pertains to etiopathogenesis is speculative and will await information not yet available.

Antibodies, Antinuclear↗
PubMed1998

Systemic lupus erythematosus in three ethnic groups: II. Features predictive of disease activity early in its course. LUMINA Study Group. Lupus in minority populations, nature versus nurture.

OBJECTIVE: To determine the factors associated with disease activity in patients with recent-onset (< or =5 years) systemic lupus erythematosus (SLE) who were of Hispanic, African-American, or Caucasian ethnicity. METHODS: Incident and prevalent cases of SLE, as defined by the American College of Rheumatology criteria for SLE, among the 3 ethnic groups were identified in Alabama (The University of Alabama at Birmingham) and Texas (The University of Texas-Houston Health Science Center and The University of Texas Medical Branch at Galveston). Variables from the sociodemographic, clinical, immunologic, immunogenetic, behavioral, and psychological domains were obtained using validated instruments. Disease activity was ascertained with the Systemic Lupus Activity Measure (SLAM). Stepwise domain regressions with SLAM score as the dependent variable were performed. Final ethnic-specific and overall regression models were obtained by entering variables that were retained in the domain regressions. RESULTS: SLAM scores at study entry were higher in the African Americans (mean +/- SD 12.6 +/- 6.9) and Hispanics (11.0 +/- 6.2) than in the Caucasians (8.5 +/- 3.7) (P < or = 0.001). The final overall regression model (R2 = 28%) for higher SLAM score included the following variables: African-American ethnicity, lack of private health insurance, abrupt disease onset, presence of anti-Ro antibodies, absence of HLA-DRB1*0301, higher levels of helplessness, and abnormal illness-related behaviors. CONCLUSION: Socioeconomic, immunologic, immunogenetic, behavioral, and psychological variables were all predictive of disease activity early in the course of SLE, irrespective of ethnic group. However, there remain ethnic group differences in disease activity that were not explained by these factors.

Adolescent↗
PubMed1986

Induction of lupus inclusions by sera from patients with systemic lupus erythematosus.

A sensitive in vitro bioassay for the alpha-interferon induction of lupus-type inclusions (LI) has been established with the human B lymphoblastoid cell line, Daudi. Sera from 11 patients with systemic lupus erythematosus (SLE) were evaluated with this assay. LI induction by these sera increased in proportion to their antiviral activity on Madin-Darby bovine kidney (MDBK) cells. Two of these sera did not induce LI; they showed no antiviral activity on the MDBK cell assay. Clinically and serologically, their donors were in remission. Two sera induced the formation of LI that exceeded the maximum frequencies obtained with 3 alpha-interferon preparations. These sera had the greatest antiviral activities, and their donors had the greatest disease activities. Antisera to alpha-interferons prevented the induction of LI with the pure and homogeneous recombinant human leukocyte interferon, IFLrA, and SLE sera. Together, these results provide evidence that the alpha-interferon endogenous to SLE patients has a great ability to induce LI, and the SLE serum induction of LI corresponds well to the patient's disease activity.

Animals↗
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↗
PubMed2004

Systemic lupus erythematosus in a multiethnic lupus cohort (LUMINA). XVII. Predictors of self-reported health-related quality of life early in the disease course.

OBJECTIVE: To determine the baseline factors predictive of self-reported health-related quality of life (HRQOL) early in the course of systemic lupus erythematosus patients (SLE) from a multiethnic LUMINA (Lupus in Minorities: Nature versus nurture) cohort. METHODS: LUMINA patients with > or =2 visits were studied. Self-reported HRQOL was examined with the 8 subscales and 2 summary measures (the Physical Component Summary [PCS], and the Mental Component Summary [MCS]) of the Short Form 36 (SF-36). Bivariable and multivariable analyses were done with the PCS, MCS and 8 subscales as the dependent variables. The analyses were performed including and excluding the corresponding SF-36 measure from the independent variables. Age, sex, and ethnicity were included in all models. Time was modeled in all regressions. RESULTS: A total of 1,351 visits (346 patients [80 Hispanics-Texas, 34 Hispanics-Puerto Rico, 126 African Americans, and 106 Caucasians]) were included in these analyses. Mean +/- SD PCS and MCS scores were 36.7 +/- 12.0 and 46.6 +/- 11.5, respectively. The scores for the eight subscales of the SF-36 were also lower than those for the general population. Baseline SF-36 measures were highly predictive of subsequent HRQOL. In the same set of regressions, older age was found to consistently predict poor self-reported HRQOL whereas fibromyalgia, helplessness, fatigue, and abnormal illness-related behaviors were also predictive, but less consistently. Estimated adjusted variances in these regressions ranged from 23% (Role-Emotional [RE]) to 43% (Physical Functioning [PF]). CONCLUSION: In patients with SLE, poor baseline HRQOL was highly predictive of subsequent poor HRQOL. Other predictive variables of poor functioning were primarily psychological/behavioral and socioeconomic-demographic.

Adult↗
PubMed2006

A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus.

OBJECTIVE: To devise a modified version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) for use in children and adolescents with systemic lupus erythematosus (SLE), based on the frequency and distribution of damage in patients with juvenile-onset SLE and the sources of damage that are most suitable for inclusion in a pediatric damage index. METHODS: In this cross-sectional study, damage was assessed through the SDI. Clinical assessments included evaluation of growth failure and delayed puberty, which were believed to be important sources of damage that are not incorporated in the SDI but should be included in a pediatric version of the instrument. RESULTS: A total of 1,015 patients with juvenile-onset SLE in 39 countries were enrolled in the study. Of these, 405 patients (39.9%) had an SDI score of > or =1 (mean +/- SD score 0.8 +/- 1.4). Renal damage (13%), neuropsychiatric damage (10.7%), and musculoskeletal damage (10.7%) were observed most frequently, followed by ocular damage (8.2%) and skin damage (7.6%). Growth failure and delayed puberty were recorded in 15.3% and 11.3% of patients, respectively. A pediatric version of the SDI was devised, with inclusion of growth failure and delayed puberty as new domains. CONCLUSION: We propose a modified version of the SDI for use in patients with juvenile-onset SLE. This new instrument warrants prospective validation in other populations of patients seen in different clinical or research settings.

Adolescent↗
PubMed2004

Differences between CD8+ T cells in lupus-prone (NZB x NZW) F1 mice and healthy (BALB/c x NZW) F1 mice may influence autoimmunity in the lupus model.

Immunization with portions of a murine antibody to DNA induced Ig peptide-reactive peripheral CD8+ inhibitory T (Ti) cells in non-autoimmune (BALB/c x NZW) F1 (CWF1) mice. Those Ti suppressed in vitro production of IgG anti-DNA by lymphocytes from MHC-matched, lupus-prone (NZB x NZW) F1 (BWF1) mice, primarily via secretion of transforming growth factor-beta (TGF-beta). However, splenic CD8+ cells from immunized BWF1 mice failed to suppress anti-DNA. Therefore, BWF1 mice were studied for defects in peripheral CD8+ T cells. The potential to suppress autoimmunity mediated by activated CD4+ helper T and B cells in BWF1 mice was assessed. As BWF1 mice aged, peripheral CD8+ T cells expanded little; fewer than 10% displayed surface markers of activation and memory. In contrast, quantities of splenic CD4+ T and B cells increased; high proportions displayed activation/memory markers. In old compared to young BWF1 mice, splenic cell secretion of two cytokines required for generation of CD8+ T effectors, IL-2 and TGF-beta, was decreased. Immunizing BWF1 mice activated peptide-reactive CD8+ T cells, but their number was decreased compared to young BWF1 or old normal mice. While peptide-reactive splenic CD8+ T cells from immunized BWF1 mice did not survive in short-term cultures, similar CD8+ T cell lines from immunized CWF1 mice expanded and on transfer into BWF1 mice delayed autoimmunity and prolonged survival. Therefore, CD8+ T cells in old BWF1 mice are impaired in expansion, acquisition of memory, secretion of cytokine, and suppression of autoimmunity. Understanding these defects might identify targets for therapy in systemic lupus erythematosus.

Animals↗
PubMed1991

Plasmapheresis and subsequent pulse cyclophosphamide versus pulse cyclophosphamide alone in severe lupus: design of the LPSG trial. Lupus Plasmapheresis Study Group (LPSG).

A group of clinics are collaborating in the Lupus Plasmapheresis Study Group (LPSG) to investigate whether repeated plasmapheresis prior to pulse cyclophosphamide improves the therapeutical results in severe systemic lupus erythematosus (SLE). The underlying rationale is the hypothesis that plasmapheresis 1) eliminates pathogenic autoantibodies and immune complexes and 2) induces compensatory lymphocyte activation via feedback mechanisms between circulating antibodies and their respective clones ("antibody rebound"). It should be possible to utilize this enhanced activity for increased clonal deletion if pulse cyclophosphamide is applied shortly after plasmapheresis. Accordingly, in a randomized study, the LPSG will be comparing the repeated application of pulse cyclophosphamide alone with a treatment involving repeated plasmapheresis prior to the cyclophosphamide pulses in severe SLE. A third arm of the study will be gathering experience with a more intensified procedure. This overview summarizes the most important details of the planned study.

Combined Modality Therapy↗
PubMed1993

Anti-DNA antibodies secreted by peripheral B cells of lupus patients have both normal and lupus-specific features.

Autoantibodies present in sera of patients with systemic lupus erythematosus (SLE) are found in low titer in sera of healthy individuals. Hence, it is possible that B cell populations in SLE patients and in normal individuals are homologous and in the absence of regulatory forces would secrete similar antibodies. We therefore studied antibody secretion of Epstein-Barr virus-transformed B cells of 20 SLE patients and 20 healthy subjects. Normal and lupus antibody repertoires did not differ significantly with respect to antigenic specificities, isotype, avidity, and titer. However, anti-DNA antibodies bearing the anti-DNA-associated idiotype 8.12 were found only in SLE. These findings suggest that the B cell repertoire of SLE and normal individuals is more similar than the serum antibody profile. However, part of the anti-DNA response in SLE probably reflects affinity (antigen driven) maturation as it differs in idiotype profile from anti-DNA response of normal individuals.

Antibodies, Antinuclear↗
PubMed1994

Neonatal lupus erythematosus with cardiac involvement in offspring of mothers with experimental systemic lupus erythematosus.

Neonatal lupus erythematosus (NLE) syndrome is characterized by a transient dermatitis, a variety of systemic and hematological abnormalities, and isolated cases of congenital complete heart block. The latter has been reported to be due to the presence of autoantibodies specific to La (SS-B) and/or Ro (SS-A). As female mice with experimental systemic lupus erythematosus (SLE) induced by immunization with the human monoclonal anti-DNA antibody bearing the 16/6 Id produce variety of autoantibodies including anti-Ro and anti-La antibodies, we looked for NLE related symptoms in the murine model. Offspring of BALB/c mice with SLE possessed high levels of autoantibodies that declined gradually till reduced to normal levels at day 60 after delivery. Electrocardiograms recorded in groups of offspring from mothers with experimental SLE indicated that a high percentage of the offspring had defects in their conductive system including first-, second-, and third-degree heart block, significant bradycardia, and a wide QRS complex. In contrast, a normal pattern was observed in offspring of healthy mothers.

Animals↗
PubMed2005

[Lupus in Germany: analysis within the German lupus self-help organization (LULA)].

During the last few decades, the prognosis for patients with systemic lupus erythematosus (LE) has changed from high early mortality to a more chronic longterm course. Although the prevalence of LE has been estimated at 20-50/100,000, data concerning the situation of LE patients in Germany are sparse. Since 2001, a documentation within the German Lupus Self-Help Organisation scheduled for a period of 10 years (LULA) has been recording at the patient level the actual status and the long-term course of a large group of LE patients. A questionnaire adapted from the German rheumatological database is updated once a year and sent to all members.In 2001, 1033 members participated in the documentation. Of these, 92.2% were women (mean age 45.8 years) with a mean disease duration of 9.9 years. 37.6% were employed, and 24.5% were on early retirement. 50.2% rated their overall health status as "not so good" or "poor". Most were receiving treatment with [hydroxy-]chloroquine (35.2%) or azathioprine (21.9%), while 67.9% were receiving corticosteroids. The most frequent comorbidities reported were hypertension (33%), scarring skin disease (24.4%), osteoarthritis (25.2%), osteoporosis (24%), psychiatric disorders/depression (22.9%) and chronic renal disease (22%). Thromboembolic events were reported in 18.5%, myocardial infarction in 2.3% and stroke in 4.8% of cases. Concerning their main contact person for health care, 63.6% specified the rheumatologist. In comparison with other cohort studies and in particular with the German rheumatological database, the data provided exclusively by patients are feasible. Concerning the severity of their disease, their treatment and their global assessment of health status, LULA participants are comparable with other LE patients and can be seen as representative of LE patients in Germany. Further assessment especially of long-term data are needed to obtain additional insights into the burden of the disease and the need for special medical care.

Adult↗
PubMed1984

Lupus pregnancy. Case-control prospective study demonstrating absence of lupus exacerbation during or after pregnancy.

To assess whether pregnancy is associated with exacerbation of systemic lupus erythematosus (SLE), a variety of clinical markers of disease activity in 28 pregnant patients with SLE (33 pregnancies) were compared with the same markers in age-, race-, organ system-, and disease severity-matched nonpregnant women with SLE. Both groups were followed up for periods of up to one year after delivery. Eight patients elected abortion for nonmedical reasons. In all patient groups, there were no differences between pregnant and nonpregnant patient groups in frequency of any disease activity marker studied including therapy. However, new proteinuria occurred in four pregnant patients compared with one nonpregnant patient, and thrombocytopenia attributable to SLE occurred in five pregnant patients and one nonpregnant patient. Renal disease, when it occurred, more closely resembled pregnancy-induced hypertension than lupus nephritis. It is concluded that pregnancy complications are frequent, but the assertion that pregnancy causes exacerbation of SLE remains unproved.

Abortion, Spontaneous↗