Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The association of anticardiolipin antibodies (aCL) with unexplained vascular occlusive disease (VOD) is well known. We reviewed the records of 102 consecutive patients seen over a 9 months period who had positive IgG or IgM aCL to determine the frequency and types of VOD in this unselected group of patients. Lupus anticoagulant was detectable in 17 of 67 (25%) patients tested. VOD occurred in 80 of 102 (78%) aCL-positive patients comprised of 17 (16.7%) with systemic venous VOD or pulmonary embolism; 27 (26.5%) with cerebral VOD: 11 (10.8%) with systemic arterial VOD; 3 (2.9%) with coronary thrombosis; and 5 (4.9%) with visceral venous or arterial VOD. Of the 19 obstetric patients with positive aCL, 17 (89%) had at least one unexplained fetal loss and 8 of the 17 (47%) had multiple or recurrent fetal losses. Twelve (11.7%) of the 102 patients met the ACR criteria for systemic lupus erythematosus (SLE). Additionally, 12 (11.7%) patients were identified as nonSLE or undifferentiated connective tissue disease (CTD). The remaining 78 (76%) had no known underlying disease (primary antiphospholipid syndrome). We conclude that IgG and IgM aCL with or without lupus anticoagulant are associated with diverse types of VOD but cerebral VOD appears predominant. aCL-associated unexplained VOD occurs frequently in patients without evidence of CTD-65 of 80 (81%) in our series. Testing for aCL is essential for identifying patients with unexplained VOD, and it should be performed in prospective clinical studies of such patients to better define the pathogenic role of aCL in the natural history of unexplained VOD.
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PMID:The prevalence of vascular occlusive disease associated with antiphospholipid syndromes. 152 53

To study the effect of fibromyalgia syndrome (FS) on the expression of systemic lupus erythematosus (SLE) and on the measurement of disease activity, we performed a cross-sectional study of subjects with SLE. Eighty-seven subjects were studied and 22 (25.3%), all female, had FS (Yunus' criteria). Disease activity and organ system involvement were assessed using the systemic lupus activity measure (SLAM) and using 10 cm visual analogue scales (VAS) completed by both physician and patient. No significant difference between FS and non-FS groups in the objective measurement of disease activity was present, median (range) SLAM scores being 5 (0-18) and 6 (0-24), respectively (NS). Similarly, expression of SLE, as measured by the prevalence of specific organ system involvement, was similar in the two groups. In contrast the prevalence of glucocorticoid use, antibodies to DSDNA and fulfilling four ACR criteria was higher in the non-FS group. The rating of SLE disease activity was affected by concomitant FS. Physician and patient disease activity VAS correlated significantly with SLAM scores in non-FS subjects (P < 0.001, Spearman analysis), whereas in FS subjects, neither physician nor patient VAS correlated with SLAM scores. We conclude that FS is prevalent in individuals with SLE and does not affect disease expression but may interfere with the rating of disease activity.
Lupus 1994 Jun
PMID:Fibromyalgia syndrome and disease activity in systemic lupus erythematosus. 795 4

Monthly intravenous cyclophosphamide (IVCY) has been a recommended therapy for severe lupus nephritis or neurological flare-ups in lupus patients. But the optimal treatment regimen and duration remains unknown. We report our experience in an open study of 37 patients treated with monthly IVCY. Thirty-four women and 3 men, mean age 35.5 with a mean disease duration of 59 months, with a mean 5.7 ACR criteria for SLE were analysed. 27 (group I) had lupus nephritis (OMS Class III or IV) and 10 had neurological involvement (group II). In group I, after six months of IVCY, a significant improvement was noticed in the UCH-Middlesex clinical index (2.9 pts vs 7.8), the proteinuria (3.12 g/d vs 5.4), complement and split fractions (CH50 98.4 vs 48.9%; C3 877 vs 600 mg/l; C4 177 vs 128 mg/l), the level of anti-DNA antibodies (67.5 vs 775 UI/ml) and the daily dose of steroids (22 vs 44 mg/d). Kidney biopsies showed a reduction of the activity index despite a slight increase of the chronicity index (4.1 vs 6.3 pts and 5.5 vs 3.6 pts). Those results were not maintained at medium and long term. Moreover five patients presented with worsening of renal function during IVCY treatment and two patients relapsed after the end of the treatment. In group II significant improvement was noticed at six months concerning the clinical index (1.77 pts vs 7.17) and the daily dose of steroids, 3 patients died because of cerebral vasculitis refractory to IVCY. Adverse effects are frequent: infectious (25 among 20 patients), hemorrhagic cystitis (2 events in 1 patient), gastrointestinal side effects were common (12/37 patients). Were also noticed: neutropenia (5/37), transient amenorrhea (4/28), drug induced menopausis (2/28). Overall mortality is important (7/37), uneffectiveness of IVCY was noticed in 5 patients, flares occurred in 8 patient during or after stopping treatment. IVCY seems efficacious if given at the very beginning of the flare. Its usefulness is obvious at six months among clinical and biological data in patients with severe lupus nephritis or neurological flare. It seems that long term outcome on the renal function is not modified.
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PMID:[Treatment of acute systemic lupus erythematosus with intravenous infusions of cyclophosphamide. Value and limitations]. 802 84

Systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) are distinct clinical disorders which rarely occur in the same patient. We report on a 65-year-old woman with coexistence of both conditions. Diagnosis of SLE was ascertained by the presence of seven ACR criteria (cutaneous lesions, photosensitivity, antinuclear and anti-double-stranded-DNA antibodies, pancytopenia, arthritis, oral lesions). PBC was disclosed by clinical investigation, liver histology and highly positive antimitochondrial M2 antibodies. The most important differential diagnoses of lupus hepatitis are PBC and autoimmune hepatitis. Diagnostic criteria for these conditions are discussed, and previous reports on overlap between SLE and PBC are reviewed.
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PMID:Unusual coexistence of systemic lupus erythematosus and primary biliary cirrhosis. 819 7

Systemic vasculitis are an heterogeneous group of diseases characterized by inflammatory infiltration and necrosis of blood vessel walls. Antineutrophil cytoplasmic antibodies (ANCA) with different immunofluorescent patterns (C or P) have been described as serological markers of some of these diseases and some types of glomerulonephritis. The presence of ANCA by immunofluorescence on normal fixed polymorphonuclear neutrophils was investigated in 182 patients. Results are depicted in Table 1. ANCA was present in 16/17 (94%) patients with Wegener Granulomatosis (W.G.) (ACR criteria) (p < 0.001). In 14 out of the 16 (82%), the pattern was ANCA-C (associated in 10 with ANCA-P) and only ANCA-P was observed in the remaining two. The presence of ANCA was associated with active disease: 15/16 samples of active patients and 3/9 of inactive patients were ANCA positive (p < 0.01). Among the other groups, ANCA-C was detected in only one patient with isolated subglottal stenosis. The specificity of ANCA-C for W.G. was 99%. ANCA-P was also detected in 3/49 (6%) patients with connective tissue disorders and in 3/63 (5%) patients in chronic hemodialysis with exclusive or predominant renal disease of unknown etiology. Three additional ANCA positive patients with known diagnosis (2 W.G. and 1 Systemic Lupus Erythematosus) were also in hemodialysis in the same unit. Thus, an ANCA related mechanism may be involved in the pathogenesis of approximately 10% of cases undergoing this procedure. None of 45 sera submitted for the detection of antinuclear antibodies were ANCA positive. Detection of ANCA (especially C pattern) may be of help in the diagnosis of W.G. and in monitoring clinical activity of the disease.
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PMID:[Anticytoplasm antibodies of polymorphonuclear neutrophils in Wegener's granulomatosis, other autoimmune diseases, and in patients undergoing hemodialysis]. 829 26

We investigated the Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index as a predictor of severe outcome and an indicator of morbidity in different ethnic groups, and in regard to its validity. We retrospectively studied disease course within 10 yr of diagnosis in an inception cohort of 80 patients with systemic lupus erythematosus (SLE). The mean renal damage score (DS) at 1 yr after diagnosis was a significant predictor of endstage renal failure and the mean pulmonary DS at 1 yr significantly predicted death within 10 yr of diagnosis. Compared to Caucasians, Afro-Caribbeans and Asians had significantly higher mean total DS at 5 and 10 yr, and higher mean renal DS at 10 yr. At 5 yr, the mean renal DS in Afro-Caribbeans and the mean neuropsychiatric DS in Asians were significantly higher than in Caucasians. The rate of endstage renal failure in Caucasians was significantly lower than in the other ethnic groups. Our results confirm the validity of the SLICC/ACR Damage Index.
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PMID:SLICC/ACR Damage Index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with systemic lupus erythematosus. 862 Mar

Systemic lupus erythematosus (SLE) is a multisystem organ disease, and involvement of the gastrointestinal system is relatively rare. We describe a 13-year-old girl who presented initially with abdominal pain, diarrhea, edema, and hypoalbuminemia. She was diagnosed with protein losing enteropathy (PLE) based on the significant increase of alpha 1-antitrypsin clearance in the stool. Two weeks after admission she developed clinical and serological findings that fulfilled the ACR criteria for SLE. Over 22 cases of lupus associated PLE have now been reported, but only 3 in children. Children with PLE should be evaluated for SLE. In addition, PLE should be suspected as a possible cause of unexplained edema and/or hypoalbuminemia in SLE.
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PMID:Protein losing enteropathy as the initial manifestation of childhood systemic lupus erythematosus. 882 4

Fibrinolysis triggered by t-PA bound to fibrin is one of the main antithrombotic mechanisms. Defects in the fibrinolytic system-decreased tissue-type plasminogen activator (t-PA) activity and elevated levels of plasminogen activator inhibitor (PAI-1), in patients with SLE have been associated with an increased tendency to thrombosis. In the present study, 43 patients with SLE fulfilling the ACR criteria for the disease, were studied for the presence of autoantibodies to fibrin-bound t-PA, i.e. the physiological active form of this plasminogen activator. A solution of 200 IU/ml of t-PA was incubated with solid-phase fibrin prepared as previously described (Anal Biochem 1986; 153; 201-210). Sera diluted 1:50 were incubated with fibrin-bound t-PA, the plates were then washed, and bound immunoglobulins were detected using a polyvalent peroxidase-labeled goat anti-human Ig. Plates coated with fibrin alone were used as controls. Sera were considered positive when A490/630 obtained with normal human sera in two independent test was greater than the mean plus 2 SD. Eleven of 43 (26%) SLE sera demonstrated antibody reactivity against fibrin-bound t-PA. Within the anti-t-PA positive group there was a higher proportion of SLE patients with severe Raynaud's phenomenon and thrombotic events when compared to the anti-t-PA negative group: 36% vs 6% and 18% vs 6% respectively. These results suggest that autoantibodies to fibrin-bound t-PA could play a role in the pathogenesis of vascular disease in some SLE patients.
Lupus 1996 Aug
PMID:Antibodies to fibrin-bound tissue-type plasminogen activator in systemic lupus erythematosus are associated with Raynaud's phenomenon and thrombosis. 886 98

The relationship between disease activity, cumulative damage and self-reported quality of life was examined in 96 patients with Systematic Lupus Erythematosus (SLE). Disease activity was measured by the SLE Disease Activity Index (SLEDAI) and cumulative damage by the Systematic Lupus International Cooperating Clinics/ACR damage index (DI). Quality of life was assessed by the Medical Outcomes Survey Short Form 20 (SF-20) self-report questionnaire which consists of six subscales. The study population was predominantly Caucasian (91%) and female (90%). The mean (+/- s.d.) age was 42.0 +/- 11.0 years and disease duration was 7.5 +/- 5.5 years. SLEDAI scores varied from 0-28, with a mean (+/- s.d.) of 4.8 +/- 5.2. The mean (+/- s.d.) DI score was 0.74 +/- 1.06, with a range of 0-5. Subscales of the SF-20 varied from 0-100 and the range of mean (+/- s.d.) scores varied from 35.0 +/- 42.2 to 70.5 +/- 28.7. There was no correlation between SLEDAI and DI scores or between SLEDAI scores and any of the six subscales of the SF-20. Likewise there was no correlation between DI scores and SF-20 subscales with the exception of Health perception (r = 0.34, P = 0.02). These results indicate that there are at least three independent dimensions of health status in SLE, namely disease activity, cumulative damage and quality of life. Furthermore, the extent of inflammatory disease activity and irreversible target organ damage are not the sole determinants for quality of life in SLE patients.
Lupus 1997
PMID:Disease activity, cumulative damage and quality of life in systematic lupus erythematosus: results of a cross-sectional study. 910 30

To test if lupus activity and damage predict physical function and general health in lupus was the objective of this study. Ninety-six patients with lupus were seen at baseline and monthly for 4 months. Sociodemographic characteristics and lupus damage (SLICC/ACR DI), were collected at baseline while lupus activity (SLAM-R, SLEDAI), health status measures (HAQ, SF-36) and immunological tests were collected at each visit. Associations of lupus activity and damage with general health and physical function were evaluated. Baseline health status measures were greatly impaired and comparable to those of severe medical illnesses. In cross-sectional analyses, baseline activity score measured by SLAM-R, but not by SLEDAI, correlated with most subscales of SF-36. Baseline damage score SLICC/ACR DI correlated only with the HAQ and the physical function subscale of SF-36. Differences in both activity measures (SLAM-R and SLEDAI) over time correlated with change in health status measures while baseline cumulative damage (SLICC/ACR DI) correlated with the average level of physical function only. Lupus activity measures, SLAM-R and SLEDAI, although differing cross-sectionally, both reflected patients' health status performance over time and lupus damage measure, SLICC/ACR DI, performed well in assessing physical function. Lupus patients scores for health are poor and comparable to those found in severe medical illnesses.
Lupus 1998
PMID:Impact of disease activity and cumulative damage on the health of lupus patients. 954 Oct 94


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