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At least 1,531 records · Page 85Linked to original sources
PubMed1998

[Neonatal lupus erythematosus and maternal HELLP syndrome: is there a pathogenetic link?].

A newborn boy developed annular erythematous lesions on his entire body. Histopathological examination showed typical features of lupus erythematosus. His mother was positive for anti-Ro/SSa and anti-La/SSb antibodies. Neonatal lupus erythematosus was diagnosed. During pregnancy the mother had suffered from HELLP syndrome. The reported case points out the necessity to differentiate HELLP syndrome from first manifestation of lupus erythematosus during pregnancy. A direct causal relationship between neonatal lupus erythematosus and HELLP syndrome of the mother seems to be unlikely.

Adult↗
PubMed1996

[Successful treatment of chronic discoid lupus erythematosus with argon laser].

We report on a patient with chronic discoid lupus erythematosus who was treated with argon-laser. The patient suffered from long-standing lesions and had been pretreated with various drugs; with no or slight improvement. After a few argon-laser applications, the treated skin lesions improved dramatically while the untreated lesional skin showed continuous disease activity. Histological and immunohistological investigations of biopsies from treated and untreated lesional skin suggest that endothelial mechanisms play a role in the generation and maintenance of discoid lesions in lupus erythematosus. This is the first reported case of successful treatment of chronic discoid skin lesions of a lupus erythematosus patient with argon-laser.

Adult↗
PubMed1998

Association between ulcerative colitis and systemic lupus erythematosus: report of two cases.

BACKGROUND: Common aetiopathogenic factors may explain the association of ulcerative colitis with autoimmune disorders such as systemic lupus erythematosus. PATIENTS: We report two cases of ulcerative colitis associated with idiopathic systemic lupus erythematosus: one patient who developed ulcerative colitis 11 years after having been diagnosed as a case of systemic lupus erythematosus and one case of simultaneous appearance of the two diseases. The lupus clinical manifestations were in neither case correlated with the treatment of ulcerative colitis. CONCLUSION: The association between ulcerative colitis and systemic lupus erythematosus is rare. Although a chance occurrence cannot be excluded it is possible that both conditions share some genetic or immunological defects.

Adult↗
PubMedDate not supplied

Prospective measure of serum 3-nitrotyrosine levels in systemic lupus erythematosus: correlation with disease activity.

Systemic lupus erythematosus (SLE) is a prototypical autoimmune disease. Overproduction of nitric oxide (NO) has been implicated in its pathogenesis. Several retrospective studies have indicated a correlation between serum nitrate and nitrite (NOx) and disease activity. This measure of NO production can be falsely elevated by exogenous dietary and medication sources of NOx and variably reduced by serum thiols. These variables can make NOx a less reliable tool for studying the role of NO in SLE. Peroxynitrite, a by-product of NO and superoxide, nitrates tyrosine moieties. The resulting 3-nitrotyrosine (3NT) serves as a long-term indicator of NO-mediated protein modifications that is not affected by exogenous sources of NOx or serum thiols. We hypothesized that for these reasons serum 3NT levels would correlate with lupus disease activity more significantly than serum NOx. To address this hypothesis, we prospectively evaluated lupus disease activity, serum protein 3NT levels, and NOx levels in a cohort of lupus patients at 3-month intervals. Serum 3NT correlated with disease activity among African-Americans, while NOx correlated with disease activity among Caucasians. Subjects with active lupus nephritis had higher levels of serum 3NT than those without renal disease. Immunohistochemical analysis of renal biopsies from subjects with active proliferative lupus nephritis revealed renal expression of inducible NO synthase. The results of this study suggest that overproduction of NO may play a pathogenic role in SLE and lupus nephritis. Serum 3NT may be a useful, new tool for studying the contributions of NO to the pathogenesis of SLE.

Autoantibodies↗
PubMed2006

Coexisting subacute and systemic lupus erythematosus after terbinafine administration: successful treatment with mycophenolate mofetil.

A 39-year-old female patient with systemic lupus erythematosus was treated with terbinafine for onychomycosis. After only 7 days of treatment with 250 mg terbinafine, a widespread severe erythematous eruption developed. The results of clinical, histological and immunofluorescent examinations confirmed the diagnosis of coexisting subacute and systemic lupus erythematosus. The patient was treated with drug withdrawal and administration of cyclosporine and methylprednisolone. One year later, mycophenolate mofetil was successfully used. Exacerbation or induction of lupus erythematosus is an extremely rare cutaneous side-effect of terbinafine. Patients with lupus erythematosus should be advised about the risk of some drugs that might exacerbate their disease.

Acute Disease↗
PubMed1991

Quantitative nailfold capillary microscopy in cutaneous and systemic lupus erythematosus and localized and systemic scleroderma.

Quantitative television microscopy of nailfold capillaries of the fingers was performed in 12 patients with cutaneous lupus erythematosus (six with discoid type and six with disseminated type), in six patients with localized scleroderma (two with circumscribed type, two with linear types, and two with atrophic type), in 10 patients with systemic lupus erythematosus, and in eight patients with systemic scleroderma. The following features were analyzed and compared with a control group (n = 15) of similar age: venous plexus visibility; density of capillaries; avascular fields; hemorrhages; giant capillaries; diameters of the transitional segment, the arterial, and the venous limbs; loop width; and flow stop caused by local cooling test. The patient groups with cutaneous lesions only showed no essential differences as compared with the controls. Patients with systemic scleroderma differed in almost every finding from the controls and from patients with localized scleroderma. Patients with systemic lupus erythematosus exhibited significant differences in several findings as compared with the controls and the cutaneous lupus erythematosus group, but there was overlap.

Adolescent↗
PubMed1996

An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus.

BACKGROUND: Valvular heart disease is the most important cardiac manifestation of systemic lupus erythematosus. We performed a study to determine the relation of valvular disease to other clinical features of lupus, whether or not the valve disease progresses, and the associated morbidity and mortality. METHODS: We performed transesophageal echocardiography and rheumatologic evaluations in 69 patients with systemic lupus erythematosus. The echocardiographic findings were compared with those in 56 healthy volunteers. Fifty-eight patients (84 percent) had second evaluations a mean (+/-SD) period of 29 +/- 13 months later. The patients and controls were followed for 57 months. RESULTS: Valvular abnormalities were common on the initial and the follow-up echocardiograms (in 61 and 53 percent of the patients, respectively). Valvular thickening was the predominant finding initially and on follow-up (in 51 and 52 percent of the patients, respectively), followed by vegetations (in 43 and 34 percent), valvular regurgitation (in 25 and 28 percent), and stenosis (in 4 and 3 percent). Valvular abnormalities frequently resolved, appeared for the first time, or persisted but changed in appearance or size between the two studies. Mild or moderate valvular regurgitation did not progress to become severe, and new stenoses did not develop. Neither the presence of valvular disease nor changes in the echocardiographic findings were temporally related to the duration, activity, or severity of lupus or to its treatment. The combined incidence of stroke, peripheral embolism, heart failure, infective endocarditis, and the need for valve replacement was 22 percent in the patients with valvular disease, but only 8 percent in those without it. A total of seven patients died during follow-up, in most cases as a result of valvular disease. Valvular abnormalities and complications were uncommon in the controls (occurring in 9 and 2 percent, respectively). CONCLUSIONS: Valvular heart disease is common in patients with systemic lupus erythematosus, frequently changes over time, appears to be temporally unrelated to other clinical features of lupus, and is associated with substantial morbidity and mortality.

Adolescent↗
PubMedDate not supplied

Comparison of tests for the lupus anticoagulant and antiphospholipid antibodies in systemic lupus erythematosus.

A variety of laboratory assays are used to screen for the presence of the lupus anticoagulant. Six commonly used coagulation tests, and the ELISA assay for antiphospholipid antibody using three different substrate phospholipids, have been evaluated in 110 patients with systemic lupus erythematosus or lupus-like disease. One or more coagulation assays was abnormal in 41% (45/110) of the patients. No individual test detected more than 78% of these abnormalities, indicating that a single phospholipid based coagulation test cannot be used to screen for a possible lupus anticoagulant. A combination of Actin FSL, DTTA and DRVVT detected all the abnormalities. The most sensitive two-test combination was Actin FSL and DRVVT. Approximately half (56%) of the patients with a positive clotting test had an abnormal antiphospholipid antibody assay. A similar proportion (58%) of the aPL positive patients had a prolonged coagulation test. The marked discordance between the coagulation assays and a positive antiphospholipid antibody test further complicates the laboratory definition of this abnormality, at least in patients with systemic lupus erythematosus.

Adolescent↗
PubMed2004

T lymphocytes in systemic lupus erythematosus: an update.

PURPOSE OF REVIEW: T cells from patients with systemic lupus erythematosus have been shown to be activated in vivo and provide cognate and noncognate help to autoreactive B cells. In particular, T cells exhibit aberrant responses to stimuli with increased calcium influx and decreased production of interferon-gamma and interleukin-2. An imbalance in the proapoptotic/antiapoptotic mechanisms also seems to contribute to the persistence of autoreactive clones and the lack of productive immune responses. The purpose of this review is to discuss recent studies that shed light into the pathogenetic mechanisms underlying T-cell dysfunction in systemic lupus erythematosus. RECENT FINDINGS: Significant progress has been made in understanding the causes of the abnormal T-cell receptor and other surface molecule-mediated signaling. Furthermore, investigators have characterized better the intracellular and nuclear signaling pathways that lead to abnormal cytokine production in lupus. Finally, efforts to correct these abnormalities in vitro have yielded promising results. SUMMARY: New findings in the pathophysiology of T cells in lupus and especially the application of novel techniques to correct immune cell aberrations on the transcriptional and translational levels give hope for the development of rational treatments in systemic lupus erythematosus.

Apoptosis↗
PubMed2003

Cognitive dysfunction and antiphospholipid antibodies in systemic lupus erythematosus.

Nervous system involvement in systemic lupus erythematosus (SLE) is typically diagnosed on the basis of clinical psychiatric and/or neurologic syndromes (NPSLE). Neuropsychological tests can be used to assess nervous system integrity even in the absence of major NP syndromes. Their application has uncovered significant cognitive dysfunction, ranging from mild to severe, in a sizeable proportion of SLE patients irrespective of clinical NP status. Cognitive dysfunction has now been accepted as a bona fide manifestation of NPSLE. The heterogeneity of clinical NPSLE manifestations is paralleled by the diversity of cognitive deficits reported in different studies and within different patients. The success of attempts to explain these deficits on the basis of potential pathogenetic mechanisms, such as antibrain antibodies and proinflammatory cytokines, has been uneven. To date, the most robust findings have emerged in relation to antiphospholipid antibodies, which carry with them important therapeutic implications.

Adult↗
PubMedDate not supplied

Cardiac abnormalities in children with systemic lupus erythematosus.

Children and adolescents with systemic lupus erythematosus (SLE, n = 14) with no cardiac symptoms were examined for cardiac involvement by physical examination, electrocardiography (ECG) and echocardiography. The indexes of left ventricular (LV) systolic and diastolic function were compared with the findings of 20 healthy, age-matched control subjects. Echocardiographic examination revealed mild tricuspid valve regurgitation in three, and moderate tricuspid and mitral valve regurgitation in two patients. Pericardial thickening was found in one patient. Indexes of LV systolic and diastolic function of SLE patients differed significantly from control subjects, with marked reduced ejection fraction (EF) and fractional shortening (FS) as well as reduced peak early diastolic filling velocity (E) and ratio of early-to-late diastolic filling velocity (E/A). Deceleration time (DT) was longer in the patients than in the control group. Late filling velocity (A) and isovolumic relaxation time (IRT) did not differ between the two groups. Valvular and pericardial involvement was found to be lower than previous reports. We conclude that asymptomatic diastolic and systolic dysfunction is common in children with SLE, most likely representing myocardial involvement. Routine cardiac evaluation by echocardiography can be recommended in the follow-up of children with SLE in order to detect silent cardiac abnormalities.

Adolescent↗
PubMed1994

Autoantibodies of neonatal lupus erythematosus.

The most common manifestations of neonatal lupus erythematosus (NLE) are cutaneous lupus and congenital heart block. Autoantibodies to Ro/SSA occur in almost all cases of NLE. The autoantibody response to Ro/SSA is complex, and antibodies may be detected to 60-kD Ro/SSA, 52-kD Ro/SSA, La/SSB, and U1 ribonuclear protein in anti-Ro/SSA-positive sera. Which of these anti-Ro/SSA-related autoantibody specificities are important in the clinical expression of NLE is not conclusively established. We examined the autoantibody specificities in 20 maternal NLE sera to determine whether autoantibody specificities correlate with the clinical findings and to evaluate the relative importance of autoantibodies to the different Ro/SSA-associated proteins. Autoantibodies were examined using immunodiffusion, immunoblotting, and enzyme-linked immunosorbent assay. Eleven babies had NLE skin disease, 11 had heart block, and two had both skin disease and heart block. All 20 maternal sera had antibodies to 60-kD Ro/SSA. Eighteen of the 20 had antibodies to 52-kD Ro/SSA, nine had antibodies to La/SSB, and one had antibodies to U1 ribonuclear protein. The prevalence of anti-La/SSB was the same in the skin-disease and heart-block subsets of NLE. Titers of anti-60-kD Ro/SSA were significantly (p < 0.02) lower in NLE skin disease maternal sera than in the NLE heart-block maternal sera. These results point out the importance of 60-kD Ro/SSA as a potential target in NLE. We speculate that the lower titers of anti-60-kD Ro/SSA in the sera from mothers of babies with skin disease may be due to substantial deposition of antibodies in the mothers' and babies' skin, leading to lower circulating titers, or may reflect a lower threshold for development of skin disease than for heart block.

Antibodies, Antinuclear↗
PubMedDate not supplied

Pregnancy course and complications in patients with systemic lupus erythematosus.

Among 165 patients with systemic lupus erythematosus (SLE), we observed 21 pregnancies in 19 patients since 1987. The mean duration of disease at the time of pregnancy was 4.5 +/- 3 years. All but three patients required immunosuppressive treatment before and during pregnancy. The effect of pregnancy on the course of SLE was studied. Severe disease exacerbations were rare and largely confined to patients with renal involvement. Most patients showed elevated titers of dsDNA antibodies during pregnancy but clinical activity of disease was usually mild. Complement C3 decrease appeared to be the most sensitive marker for pregnancy-related complications. The detection of antibodies to phospholipids was frequent during pregnancy in contrast to a low prevalence before and after pregnancy. Their presence could be associated with intrauterine growth retardation. Preterm delivery before the 37th week of pregnancy had to be performed in the majority of patients. None of the patients experienced abortion although three patients had to delivered in the 29th week of pregnancy because of increasing symptoms of pre-eclampsia. Two of these children died and the third child suffered from intracranial hemorrhage in the early postpartum period. Our data demonstrate that successful pregnancy outcome was related to a gestational age of more than 32 weeks, making careful monitoring and appropriate therapeutic management necessary.

Adult↗
PubMed1997

[Doppler echocardiographic study of left ventricular function in patients with systemic lupus erythematosus].

This study was aimed at investigating abnormalities in left ventricular size and function in patients with systemic lupus erythematosus without overt cardiovascular manifestations, in order to detect a very early impairment in myocardial function. Seventeen females and 1 male with systemic lupus erythematosus of 4 to 20 year duration and without clinical evidence of heart disease were studied. Twelve healthy volunteers, matched for age, sex and quatelet index, were utilized as controls. Each patient had a two-dimensional M-mode echocardiographic and Doppler examination. In patients with systemic lupus erythematosus there was an increase in left ventricular ejection fraction (p < 0.001), a slight reduction of end-diastolic volume index and a significant decrease of end-systolic volume index (p < 0.001). In the same patients we also found prolongation of the isovolumic relaxation time (p < 0.02), a clear impairment of diastolic filling parameters. Peak E velocity was lower (p < 0.01), peak A velocity was higher (p < 0.01), with a clear lowering, of the corresponding E/A ratio (p < 0.001) in patients with systemic lupus erythematosus.

Adult↗
PubMed1983

Transition from systemic lupus erythematosus to common variable hypogammaglobulinemia.

A 34-year-old man had polar extremes of B cell dysfunction: systemic lupus erythematosus evolving into common variable hypogammaglobulinemia. He presented in 1974 with seizures and six other criteria for systemic lupus erythematosus; his antinuclear antibody titer was 1:1024 and IgG level, 2870 mg/dL. After 5 months of immunosuppressive treatment, a 79% decrease in serum IgG and 95% decrease in IgA levels occurred and manifestations of systemic lupus erythematosus disappeared. Six years later, he developed panhypogammaglobulinemia, had recurrent sinopulmonary infections, and showed nodular lymphoid hyperplasia on rectal biopsy. For comparison, serum immunoglobulin concentrations were measured serially in 13 other patients with systemic lupus erythematosus. Three developed severe depressions of these levels, two with IgG levels less than 300 mg/dL and one with an IgA level of 8 mg/dL. These decreases were transient, related to treatment, and not associated with infections. Daily high-dose prednisone therapy (60 mg/d) rather than treatment with cytotoxic drugs correlated with decreased immunoglobulin concentrations.

Adolescent↗
PubMedDate not supplied

Immunoglobulin deficiency in patients with systemic lupus erythematosus.

Hypergammaglobulinemia is a common laboratory finding in patients with active systemic lupus erythematosus; in contrast, immunoglobulin deficiency, except for immunoglobulin A, is unusual. We report 18 patients who developed low immunoglobulin G levels 4 months to 22 years (median = 4 years) following the diagnosis of systemic lupus erythematosus. This phenomenon was transient in 10 patients (median duration 10.5 months). Eight patients had received cytotoxic drugs prior to the development of hypogammaglobulinemia, while all had received prednisone. The nadir levels of serum IgG were 132-550 mg/dl (median = 363 mg/dl). The presence and degree of immunoglobulin G deficiency did not correlate, in general, with the type or dose of medication. None of the patients had renal failure. Only 4 patients developed recurrent infections. Urinary loss of protein was not a cause of this disorder. Study of the in vitro cellular immune responses of peripheral blood lymphocytes in 5 patients showed that excessive 'suppressor' T cell activity and decreased numbers of B cells may be responsible for the development of immunoglobulin deficiency. Serum immunoglobulin levels should not be employed as an indication of disease activity in systemic lupus erythematosus, as all 18 patients continued to have significant clinical disease. Deficiencies of immunoglobulins are often transient and may not require treatment.

Adolescent↗
PubMed2000

The characteristics of systemic lupus erythematosus. A study in a general hospital.

OBJECTIVE: There is a wide variation in the natural history of Systemic Lupus Erythematosus among different ethnic and geographical groups. Studies in Arabs are few. This study aims to demonstrate the clinical characteristics of Systemic Lupus Erythematosus patients in Jordanians. METHODS: A retrospective review of the records of the cases diagnosed as Systemic Lupus Erythematosus in a tertiary referral centre (King Hussein Medical Centre) over the years 1991-1997. The records were analyzed for age, sex, presentation, diagnostic criteria, investigations, complications and treatment. RESULTS: Seventy-six records were analyzed. The patients were from all parts of Jordan, with a mean age of 20 years. The female: male ratio was 24:1. The presentation was arthralgia-arthritis in 68 (89%) patients; skin manifestations in the form of malar rash in 32 (42%), photosensitivity in 19 (25%). Central nervous system manifestations were also noted in 21 (27%) of the patients. Anti-nuclear antibodies were positive in 71 (93%) patients, anti double stranded DNA (DsDNA) positive in 80%. Anemia and leukopenia or both were noted in 52 patients(69%). Erythrocyte Sedimentation Rate was more than 30mm in the first hour in 49 (88%) patients. Lupus anticoagulants were negative in 75% of patients, renal impairment was documented in 46% of the patients (35 patients) with positive correlation to DsDNA. All the patients received steroids, 95% (73) in the form of prednisolone and 5% in the form of methylprednisolone; cytotoxics either cyclophosphamide or azathioprine mainly for renal disease were prescribed to 25 patients, Complications were hypertension (18 patients), renal failure (7 patients), cerebral vascular disease (3 patients). Death was recorded in 3 subjects within 1-4 years of diagnosis CONCLUSION: This study demonstrates the presentations of Systemic Lupus Erythematosus patients with a high incidence of complications, which may be due to late presentation or late diagnosis. Further studies are needed on the natural history of this disease in Jordanians.

Adolescent↗