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[Comparison of severity and native anti-DNA antibody levels in systemic lupus erythematosus. Serodiscordant lupus].

The authors studied the clinicobiological correlations between the severity of systemic lupus erythematosus (SLE) and native anti-DNA antibody levels in 176 patients suffering from this disease. Certain patients were studied at different stages of their disease. This correlation was very close in one out of two cases and relative in one case out of four. In one-fourth of these patients, a significant and persistent discordance existed, and these patients were therefore studied apart. Certain SLE which have been suppressed over long periods of time continue to have elevated anti-DNA levels which are occasionally associated with lowered levels of complement. This has been previously reported in the literature. All decisions to modify corticosteroid treatment based only on biological data is therefore questionable. In contrast, in certain cases of SLE where the evolution of the disease is relatively severe, no anti-DNA or antinuclear antibodies are present. These "seronegative" SLE cases are proof that the "specific" biology of this disease may be erroneous for diagnosis as well as for prognosis. The authors review the possible or known causes for the absence of these antibodies in SLE. They believe that native anti-DNA levels are only of relative value in SLE and feel that these values must be examined with respect to clinical and other biological data in order to treat and survey patients affected with this disease.

Antibodies, Antinuclear↗

[Erythermalgia and systemic lupus erythematosus].

We report three cases of erythermalgia associated with systemic lupus erythematosus corresponding to different clinical situations in such an association. The first patient developed erythermalgia during the course of systemic lupus erythematosus. In the second, erythermalgia preceded other symptoms of systemic lupus erythematosus by four years. In the third, erythromelalgia was not related to a flare-up of systemic lupus erythematosus, but to thrombocythemia, a complication of immunosuppressive therapy. These cases permit a discussion on terminology and classification of erythromelalgia and erythermalgia. However, more than terminology or classification into three types or into adult-onset and early-onset (childhood) erythromelalgia, the important is to consider primary and secondary forms. We used a classification into two types: primary (or erythermalgia) with subdivision into sporadic and familial subtypes, and secondary with subdivision into erythermalgia related to myeloproliferative disorders and erythermalgia related to other diseases, such as systemic lupus erythematosus, or to drugs (erythermalgia-like syndrome).

Adolescent↗

Effectiveness of testing for anti-DNA and the complement components iC3b, Bb, and C4 in the assessment of activity of systemic lupus erythematosus.

Seventy-one patients with systemic lupus erythematosus (SLE), seen in an outpatient setting for follow-up evaluation during a 3-mo period, were tested (in addition to routine lupus monitoring studies) with enzyme immunoassays (EIAs) for anti-DNA, iC3b, and factor Bb to determine the relationship of these test results to disease activity. SLE activity was scored by four previously reported scoring systems, and six patients were identified as active by all four systems. We found that the EIA for anti-DNA was the best indicator of disease activity and that iC3b and Bb were not informative for this purpose in this group of patients. Mean iC3b levels were higher in a subset of seven patients with past biopsy evidence of severe (WHO class IV) glomerulonephritis than in the rest of the study population, even though none of these patients had active disease at the time of this study.

Adolescent↗

Low serum antimycobacterial glycolipid antibody titers in the sera of patients with systemic lupus erythematosus associated with central nervous system involvement.

Antibodies which bind to glycolipids derived from Mycobacterium tuberculosis were sought in the sera of patients with systemic lupus erythematosus (SLE), with and without neurological disease, patients with active tuberculosis and normal controls. A significantly lower activity against the mycobacterial glycolipids was detected in the sera of patients with SLE with neurological involvement compared to other patients with SLE or controls. We suggest that low antibody levels against mycobacterial glycolipids may be useful in the diagnosis of patients with SLE in whom involvement of the central nervous system is suspected.

Antibodies, Bacterial↗

Childhood systemic lupus erythematosus and neonatal lupus syndrome.

Systemic lupus erythematosus in children can present with a wide spectrum of disease manifestations. Significant organ system involvement appears to be more severe in children than in adults. Central nervous system disease continues to be difficult to diagnose because of the lack of sensitive and specific diagnostic tests. Renal function is the major determinant of long-term prognosis and management in children with lupus. Identification of patients who are most at risk for progression of renal disease and aggressive treatment, including corticosteroids and immunosuppressive agents, are indicated. Genetic susceptibility studies in lupus reveal multiple contributions from HLA and non-HLA genes. Current concepts regarding apoptosis and DNA-protein complexes and autoreactive T-cell help for anti-DNA antibody production suggest novel directions for therapies. New understandings of the pathogenesis of neonatal lupus syndrome and congenital heart block reveals important information about prospective monitoring and management of mothers and fetuses at risk.

Cardiovascular Diseases↗

Diagnostic Performance of Machine Learning for Systemic Lupus Erythematosus: Systematic Review and Meta-Analysis.

BACKGROUND: Early and accurate diagnosis of systemic lupus erythematosus (SLE) and its organ involvement is essential. Previous reviews of machine learning (ML) in SLE combined heterogeneous tasks and validation strategies and may have overinterpreted model performance. OBJECTIVE: This study evaluated the diagnostic performance of ML and deep learning (DL) models for 3 clinically distinct SLE-related tasks: SLE classification or diagnosis, lupus nephritis (LN) diagnosis, and neuropsychiatric systemic lupus erythematosus (NPSLE) discrimination. We also assessed methodological quality and certainty of evidence. METHODS: PubMed, Embase, Cochrane Library, Web of Science, and IEEE Xplore were searched from January 2014 to April 2026. Eligible peer-reviewed diagnostic accuracy studies developed or validated ML or DL models for 1 of the 3 prespecified tasks, used an accepted reference standard, and provided data for a 2×2 contingency table. Bivariate random-effects meta-analyses with the Hartung-Knapp-Sidik-Jonkman adjustment were used to pool sensitivity and specificity. We reported 95% prediction intervals (PIs), assessed risk of bias using the Quality Assessment of Diagnostic Accuracy Studies for Artificial Intelligence tool (QUADAS-AI; Viknesh Sounderajah [Imperial College London]), and evaluated certainty of evidence using the Grading of Recommendations Assessment, Development, and Evaluation framework for diagnostic test accuracy. RESULTS: Twenty-nine studies were included: 17 for SLE classification, 5 for LN diagnosis, and 7 for NPSLE discrimination. In the primary task-stratified analysis, pooled sensitivity was 0.91 (95% CI 0.86-0.94; 95% PI 0.56-0.99), and pooled specificity was 0.94 (95% CI 0.91-0.96; 95% PI 0.69-0.99), with low heterogeneity (I²=23.9% and 22.9%, respectively). DL models showed a sensitivity of 0.93 and specificity of 0.95, compared with 0.88 and 0.94 for traditional ML models. Certainty of evidence was high for most analyses but low for LN diagnosis because of inconsistency and imprecision. All studies were retrospective, and only 9 of 29 (31%) performed independent external validation. Overall risk of bias was high or unclear in 22 of 29 (75.9%) studies. No study reported model calibration, decision-curve analysis, or net clinical benefit. CONCLUSIONS: ML models showed promising diagnostic accuracy across 3 distinct SLE-related tasks, but wide PIs, limited external validation, and pervasive risk of bias restrict conclusions about real-world generalizability. Prospective multicenter studies with standardized tasks and reference standards, independent external validation, and formal assessment of calibration and clinical utility are required before clinical implementation.

Humans↗

Autoantibodies against all the phospholipids: a comparative systematic study with systemic lupus erythematosus and healthy sera.

The sera of systemic lupus erythematosus patients were tested by ELISA for the presence of autoantibodies against all the phospholipids: cardiolipin, phosphatidic acid, phosphatidylcholine, phosphatidylethanolamine, phosphatidylglycerol, phosphatidylinositol and phosphatidylserine. The quantity of phospholipid coated (in comparison with that initially deposited) on the microtitration plates was precisely evaluated in order to determine if the results (absorbance values) obtained for each phospholipid could be compared directly. The systemic lupus erythematosus sera tested gave positive results for all the phospholipids. The highest level of autoantibodies was observed with phosphatidic acid followed by phosphatidylserine, phosphatidylinositol, cardiolipin, phosphatidylglycerol, phosphatidylcholine and phosphatidylethanolamine. The sera seemed to contain antibodies directed either against all the 8 phospholipids tested or more specifically against one or two phospholipids. The results were compared with those obtained with 17 healthy sera. Much lower values were obtained for the sera of healthy subjects, the majority of which showed a weak binding, similar for all the phospholipids. These results suggest that the anti-phospholipid autoantibodies present in systemic lupus erythematosus sera are significantly higher than those of healthy subjects. It is concluded that in the investigation of anti-phospholipid antibodies, tests should be carried out for all the phospholipids.

Antibody Specificity↗

[Prognosis of filial generation from mothers with systemic lupus erythematosus].

Seventy three pregnancies in 44 women with systemic lupus erythematosus in our hospital from June 1970 to June 1993 were studied. It included 15 spontaneous abortions (20.55%), 8 induced abortions (10.96%), 9 perinatal fetal deaths (12.33%) and 41 live births (56.16%). Therapy with steroids during pregnancy is harmless and effective. It may reduce pregnant loss rate. Of 41 live births, 35 children were followed up (85.36%). Their physical growth, routine blood test and urinalysis were normal. There were no definite evidences of mental developmental abnormality in children. Two girls had been diagnosed of systemic lupus erythematosus at about nine years old. It is possible that systemic lupus erythematosus may be hereditable to female filial generation. So we suggest that the following-up on children born to mothers with systemic lupus erythematosus have to be done till puberty.

Adolescent↗

Combined oral contraceptives in women with systemic lupus erythematosus.

BACKGROUND: Oral contraceptives are rarely prescribed for women with systemic lupus erythematosus, because of concern about potential negative side effects. In this double-blind, randomized, noninferiority trial, we prospectively evaluated the effect of oral contraceptives on lupus activity in premenopausal women with systemic lupus erythematosus. METHODS: A total of 183 women with inactive (76 percent) or stable active (24 percent) systemic lupus erythematosus at 15 U.S. sites were randomly assigned to receive either oral contraceptives (triphasic ethinyl estradiol at a dose of 35 microg plus norethindrone at a dose of 0.5 to 1 mg for 12 cycles of 28 days each; 91 women) or placebo (92 women) and were evaluated at months 1, 2, 3, 6, 9, and 12. Subjects were excluded if they had moderate or high levels of anticardiolipin antibodies, lupus anticoagulant, or a history of thrombosis. RESULTS: The primary end point, a severe lupus flare, occurred in 7 of 91 subjects receiving oral contraceptives (7.7 percent) as compared with 7 of 92 subjects receiving placebo (7.6 percent). The 12-month rates of severe flare were similar: 0.084 for the group receiving oral contraceptives and 0.087 for the placebo group (P=0.95; upper limit of the one-sided 95 percent confidence interval for this difference, 0.069, which is within the prespecified 9 percent margin for noninferiority). Rates of mild or moderate flares were 1.40 flares per person-year for subjects receiving oral contraceptives and 1.44 flares per person-year for subjects receiving placebo (relative risk, 0.98; P=0.86). In the group that was randomized to receive oral contraceptives, there was one deep venous thrombosis and one clotted graft; in the placebo group, there was one deep venous thrombosis, one ocular thrombosis, one superficial thrombophlebitis, and one death (after cessation of the trial). CONCLUSIONS: Our study indicates that oral contraceptives do not increase the risk of flare among women with systemic lupus erythematosus whose disease is stable.

Adolescent↗

Detection of polyanion-restricted anti-histone antibodies in patients with systemic lupus erythematosus.

The nature of the antibodies responsible for lupus erythematosus (LE) cells in systemic lupus erythematosus (SLE) remains obscure. We examined whether polyanion-restricted anti-histone antibodies were present in serum of patients with SLE using Western blotting analysis. Dextran sulfate or alginate was used as a polyanion compound in place of DNA. Antibodies which recognized dextran sulfate-histone complexes were present in serum of patients with SLE (17/34, 50%). These antibodies were detected in most SLE patients positive for LE cells (17/18, 94%) but not in those negative for LE cells or in patients with other collagen diseases. Similar results were obtained using alginate-histone complexes as antigens for Western blotting analysis. The antibodies to dextran sulfate-histone or alginate-histone complexes in serum of SLE patients were completely absorbed by treatment of serum with DNA-histone complexes, while they were unaffected by treatment with DNA only. The presence of antibodies to free histones and dextran sulfate-histone complexes did not seem to be related to the titer of anti-single stranded DNA antibody and anti-double stranded DNA antibody. We demonstrated the presence of polyanion-restricted antibodies in SLE, which may be responsible for the LE factor.

Adolescent↗

Thrombotic disease in systemic lupus erythematosus is associated with a maintained systemic platelet activation.

Patients with systemic lupus erythematosus (SLE) have an increased risk of thrombosis. Platelet-induced extracellular phosphorylation of plasma proteins suggests that this is due to persistent activation of the platelets. We examined 30 SLE patients (15 with thrombotic disease), 18 non-SLE patients with deep vein thrombosis (DVT) and 50 healthy controls by analysing beta-thromboglobulin, activated factor XI-antithrombin complexes and fibrinogen-bound phosphate. All parameters were elevated in SLE patients, particularly those with thrombosis, but normal in DVT cases and healthy controls. We conclude that thrombotic disease in SLE patients is associated with a persistent systemic platelet activation that may lower the threshold for induction of thrombosis.

Adult↗

Can determination of lactic acid and pyruvic acid in cerebrospinal fluid help in diagnosing central nervous system involvement in systemic lupus erythematosus?

The central nervous system (CNS) is frequently affected by systemic lupus erythematosus (SLE). Other causes, especially infection, may also originate CNS disease in SLE patients. Our findings indicate that measurement of lactic acid and pyruvic acid in cerebrospinal fluid (CSF) may be of help in separating inactive CNS-SLE from active CNS-SLE. Also it may be of help in differentiating sterile meningitis caused by SLE from bacterial meningitis. Because of the special risk on tuberculous and fungal infections in SLE patients, also some findings concerning these specific forms of meningitis are given.

Central Nervous System Diseases↗

[A case report of systemic lupus erythematosus patient with severe lupus retinopathy and antiphospholipid antibody].

An eleven-year-old boy with systemic lupus erythematosus (SLE) developed severe bilateral lupus retinopathy when he was in active stage of SLE. The patient, who had suffered from SLE for 3 years, was admitted to our hospital because of high grade fever, systemic lymphadenopathy, leukopenia, elevation of erythrocyte sedimentation rate and hypocomplementemia. The dose of prednisolone was increased considering he was exacerbated of SLE, however, the convulsion as CNS lupus occurred to him. After the event he noted loss of vision in his bilateral eyes. The ophthalmologic examination revealed the lesions of cotton-wool spots, retinal vessel dilatations and diffuse occlusions of the retinal arterioles and venules which were compatible with lupus retinopathy. Although the coagulation time was normal, antiphospholipid antibodies were positive at the time of ocular involvement. Careful attention should be paid to the occurrence of lupus retinopathy when the patients with SLE developed in the active stage or CNS lupus, especially they have antiphospholipid antibodies.

Antibodies, Antiphospholipid↗

Chronic infection with cutaneous herpes simplex in a patient with systemic lupus erythematosus.

A 68-year-old black woman with systemic lupus erythematosus being managed by systemic corticosteroids developed large ulcerated lesions in a dermatomal distribution on the flank and abdomen. Subsequently, large ulcers developed progressively in the perianal region, the buttocks, the perivaginal region, and the thighs. The clinical diagnosis was vasculitis of systemic lupus erythematosus. Biopsy from the margin of an ulcer showed changes in the epidermis that are specific for infections by herpesvirus. Electron microscopy revealed viral particles of herpesvirus. Cultures from the perianal lesions grew Herpesvirus hominis. This is the first report to our knowledge of "chronic cutaneous herpes infection" in a patient with systemic lupus erythematosus.

Aged↗

Serologic abnormalities in spontaneous and drug-induced systemic lupus erythematosus.

Extensive serologic changes occur in systemic lupus erythematosus (SLE) which are probably secondary to unknow primary cause(s). The New Zealand hybrid mouse model most likely has a viral-induced disease and does not show many of the clinical features of the human disease. The best example of human SLE which provides a clue to etiology is the drug-induced type, particularly that due to procainamide. In these patients it is possible to study the development of serologic changes prior to the onset of clinical manifestations, and then observe regression of the clinical and serological changes on withdrawal of the medication. Although there is a rough correlation between the many serologic abnormalities and the clinical picture, enough exceptions exist, so that single tests such as serum complement, anti-DNA antibodies, per cent DNA binding, and others, cannot be used as a sine qua non for management. Care of the patient still remains a clinical problem guided by various laboratory procedures but not dependent on any one. Alkyating agents have a limited role in the treatment of lupus nephropathy and cutaneous vasculitis but azathioprine is probably of no value in SLE.

Animals↗

Humoral intestinal immunity in systemic lupus erythematosus.

Forty-five patients with systemic lupus erythematosus (SLE) underwent a cross-sectional study to evaluate intestinal secretory immunity. Peroral jejunal biopsy with histologic and immunohistochemical assessment of the mucosa were carried out in the patients and in 12 healthy volunteers. It was observed that an altered pattern of immunoglobulin-bearing plasma cells distributed in the lamina propria and complementary components were invariably present, mainly in the patients with active disease. The basement membrane of the intestinal crypt epithelium exhibited immunoglobulin and complementary deposits, similar to the lupus band test. None of the immunologic findings correlated with the medical treatment and with the peripheral blood analysis. The local changes in humoral immunity in patients with SLE did not correlate with gastrointestinal symptoms and may reflect the systemic effects of the disease.

Adolescent↗

Autoantibodies against neutrophil cytoplasm components in systemic lupus erythematosus and in hydralazine-induced lupus.

Anti-neutrophil cytoplasm antibody (ANCA) has been shown to be no marker of systemic lupus erythematosus (SLE) including lupus nephritis or of progressive systemic sclerosis (PSS). Antibodies against myeloperoxidase (anti-MPO) and elastase, two granulocyte lysosomal enzymes, were found in patients with SLE but not in those with PSS, except for one patient who had anti-MPO. Anti-MPO was present in 21% of patients with SLE, and at low concentrations in about 80% of these cases. Anti-elastase was found in four patients with SLE. In another group of six patients with a SLE-like syndrome induced by anti-hypertensive treatment with the anti-hypertensive hydralazine, anti-MPO antibodies occurred in all six, and anti-elastase antibodies in five. Monitored during a 2-year follow-up period, anti-MPO antibodies were found to persist, whereas anti-elastase antibodies were rapidly eliminated, after withdrawal of the drug.

Autoantibodies↗