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Libman-Sacks endocarditis complicating systemic lupus erythematosus has rarely been reported to cause hemodynamically significant valvular lesions. This report presents a case of severe aortic stenosis combined with severe mitral regurgitation associated with systemic lupus erythematosus in a young woman who died while on the quota list for surgery.
The prevalence of autoimmune diseases, including systemic lupus erythematosus, is increased in failure of certain host defence mechanisms. Systemic lupus erythematosus, however, has not been recorded as a late complication of the Staphylococcus aureus hyperimmunoglobulinaemia E (hyper-IgE) syndrome. Such a case was investigated in a man suffering from a classic example of the syndrome. Antinuclear antibodies were analysed on a molecular basis. The emergence of immunological and clinical features of systemic lupus erythematosus in patients with defective host defence mechanisms against staphylococcal infections is unlikely to be fortuitous and may help elucidate the pathogenesis of systemic lupus erythematosus. The observations will also aid the long term management of patients with S aureus hyper-IgE syndrome.
This paper presents a case of lepromatous leprosy complicated with systemic lupus erythematosus and diagnosed as having typical systemic lupus erythematosus from clinical features and results of laboratory tests. We discuss the appearance of autoantibodies in systemic lupus erythematosus as well as in leprosy.
OBJECTIVES: The aims of this study were to better characterize valve disease in systemic lupus erythematosus and to determine its association with antiphospholipid antibodies. BACKGROUND: Estimates of the prevalence of valve disease in systemic lupus erythematosus have been higher in autopsy series than in clinical studies using transthoracic echocardiography. Antiphospholipid antibodies have been suggested to be a primary pathogenetic factor. METHODS: Transesophageal echocardiography was performed on 1) 54 patients with lupus erythematosus, 22 of them with (group I) and 32 without (group II) antiphospholipid antibody; 2) on 10 patients with antiphospholipid syndrome (group III); and 3) on 35 normal subjects (group IV). RESULTS: Patients in groups I and III had similar types and concentrations of antibodies. Leaflet thickening was found in 50% of group I, 47% of group II, 10% of group III and 9% of group IV patients (group I or II vs. group III or IV, p < 0.03). Leaflet thickening in patients with lupus erythematosus was diffuse; it usually involved the mitral and aortic valves and was associated with valve regurgitation (73%) or valve masses (50%). Valve masses were observed in 41% of group I, 25% of group II, 10% of group III and in none of group IV patients (group I or II vs. group IV, p < 0.002). Most valve masses in patients with lupus erythematosus were located near the base on the atrial side of the mitral valve or on the vessel side of the aortic valve, had variable size (0.2 to 0.85 cm2), shape and echodensity. Valve regurgitation was observed in 64% of group I, 59% of group II, 10% of group III and 20% of group IV patients (group I or II vs. group III or IV, p < 0.006). Moderate or severe regurgitant lesions were noted in 27% of group I and 25% of group II patients. CONCLUSIONS: Lupus erythematosus valve disease is frequent (74%) regardless of the presence or absence of antiphospholipid antibodies. Therefore antiphospholipid antibodies may not be a primary pathogenetic factor. The characteristic appearance of leaflet thickening and masses in patients with lupus erythematosus may be unique.
OBJECTIVES: To evaluate the European Consensus Lupus Activity Measurement (ECLAM) for responsiveness to change in disease activity when used in childhood-onset systemic lupus erythematosus (cSLE). To confirm the construct validity and to characterize the measurement properties of the ECLAM by assessing its ability to predict damage and steroid requirements. METHODS: The disease courses of 66 newly diagnosed cSLE patients were reviewed. The ECLAM and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) were scored for all clinic visits and hospitalizations. Damage was assessed at the end of the followup period using the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index. Disease activity at the time of diagnosis, 6 months after diagnosis, at the time of first flare, and 6 months after first flare was used to estimate responsiveness of the measures. Responsiveness was measured by the effect size, the effect size index, the standardized response mean, and the relative efficiency index (REI). The measurement properties of the ECLAM and SLEDAI over time were examined by comparing the ability of both measures to predict damage and oral steroid requirement. RESULTS: The ECLAM and SLEDAI are both responsive to change in disease activity irrespective of the statistic used. The ECLAM is more sensitive than the SLEDAI using the REI (all >1.9). Cumulative disease activity as measured by the SLEDAI or the ECLAM are important predictors of damage. There are no statistically important differences between the 2 measures with regard to their ability to predict steroid requirements. CONCLUSIONS: The ECLAM has construct validity in cSLE and, like the SLEDAI, is highly sensitive to clinically important change in disease activity. The ECLAM may be more responsive. The quantitative properties of the 2 measures are very similar. The SLEDAI likely remains the preferable disease activity measure for cSLE given its overall measurement properties and ease of use.
Discoid lupus erythematosus (DLE) is an uncommon disease in childhood. We present two patients initially diagnosed as impetigo and photosensitive eczema with impetigo, respectively, who failed to respond to topical and systemic antistaphylococcal agents and in whom a diagnosis of discoid lupus erythematosus subsequently became apparent.
INTRODUCTION: Association of Crohn's disease and systemic lupus erythematosus is infrequent. We report an observation of Crohn's disease that appeared in an 18-year-old woman followed-up for systemic lupus erythematosus for four years. EXEGESIS: The patient had all the clinical, biological and histological criteria of Crohn's disease and systemic lupus erythematosus was diagnosed according to the American Rheumatism Association criteria. On the base of this observation, we discuss the digestive manifestation of systemic lupus erythematosus and extradigestive manifestations of Crohn's disease. CONCLUSION: The immunological background of both diseases was proposed to explain the mechanism of this rare association.
Four patients with systemic lupus erythematosus (SLE) are described in whom there were major psychiatric complications. Two of these patients had cerebral lupus with psychiatric manifestations of the disease together with other features of disease activity and responding to treatment with high dose steroids. The first of these had had a ten-year history of recurrent episodes of depression before other features of the disease became evident; in the second patient recurrent psychotic episodes occurred after the onset of typical multi-system disease. The third patient had had a minor cerebro-vascular accident four years before other features of SLE became manifest, and cerebral deterioration later on in her life was probably due to hypertensive cerebro-vascular disease secondary to the renal disease of SLE. The fourth patient, a young man, had had recurrent episodes of depression and aggressive behaviour for several years and committed suicide at the age of 33.
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.
We describe a pregnant woman with systemic lupus erythematosus, complicated by severe pre-eclampsia (the HELLP syndrome) and adult respiratory distress syndrome, who died in the intensive care unit. A 33-year-old multigravid woman was referred to our university hospital at 17 weeks' gestation because of an exacerbation of systemic lupus erythematosus with elevated liver enzymes and thrombocytopenia. At the time of admission, on physical examination she had revealed a butterfly rash over the cheeks, purpura, acute synovitis and oedema of the legs. Her blood pressure was 180/100 mmHg. The initial laboratory tests and immunological evaluation confirmed active systemic lupus erythematosus complicated by severe pre-eclampsia. One week after admission, she became suddenly confused and had a convulsion with Glascow Coma Score 10 (3+3+4). Therapeutic abortion was induced in the Obstetric Department. She was transferred to the intensive care unit with a diagnosis of respiratory failure, probably due to acute respiratory distress syndrome, and was intubated and ventilated. Dialysis was instituted on two consecutive days from the eighth day. In total she received over 20 units of red blood cells and large quantities of fresh frozen plasma and platelets. On the 24th day her Glascow Coma Score was 2 (1+1+E) and severe hypotension developed. She died from worsening acute respiratory distress syndrome on the 25th day. Women with systemic lupus erythematosus should be advised to become pregnant when the disease is inactive and should be observed at an appropriate centre using a multidisciplinary approach. Therapeutic abortion is an acceptable option if active nephropathy and severe pre-eclampsia are present in early pregnancy.
OBJECTIVE: The purpose of this study was to verify whether stress worsens the clinical symptomatology perceived by patients with lupus erythematosus. Toward this end, we considered two types of stressors-daily stress and high-intensity stressful life events. METHODS: In 46 patients with systemic lupus erythematosus and 12 patients with chronic lupus discoid, we studied the stress they experienced daily for 6 months and their disease symptoms. During this period, we also analyzed the levels of C3 and C4 complements and anti-DNAn antibodies. The systemic lupus erythematosus activity (assessed by the Systemic Lupus Activity Measures) and cumulative organ damage (assessed by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index) were also analyzed. RESULTS: We did not find that high-intensity stressful life events produced a worsening of the symptomatology of the disease. However, using a time-series analysis (Box-Jenkins), we found that a high percentage of lupus patients (74.1%) perceived a worsening in their clinical symptomatology due to the effects of daily stress. Of this 74.1%, 53.4% worsened the same day they suffered the perceived daily stress, and the remaining 20.7% experienced an increase in symptoms both the same day and the following day. Subsequent Mann-Whitney analyses showed that the patients who worsened for 2 days because of the effects of stress had greater lupus activity, as evaluated by their levels of C3, C4, and anti-DNAn. CONCLUSION: Daily stress, and not stressful life events, worsened the clinical symptomatology perceived by lupus erythematosus patients. This increase extended at times to 2 days, and was associated with greater lupic activity.
Both systemic lupus erythematosus and Sjogren's syndrome are autoimmune diseases. Almost all organs can be involved but the gall bladder is an unusual site. We report a 39-year-old woman with systemic lupus erythematosus and Sjogren's syndrome presenting with acute acalculous cholecystitis. It is a very rare complication and, in the literature review, surgical interventions are mostly applied for treatment. In our case, high dose corticosteroid was tried and clinical manifestations and radiologic findings were improved.
Systemic lupus erythematosus (SLE) and infection with human immunodeficiency virus are rarely seen in the same patient. We describe a case of a 32-year-old lady, who was diagnosed with systemic lupus erythematosus (anti-dsDNA antibody positive) and was initially serologically negative for human immunodeficiency virus (HIV) infection. Following three cycles of IV cyclophosphamide, she was subsequently found to be infected with HIV, with marked depletion of peripheral CD4-positive T lymphocytes. While her SLE remained completely inactive, the course of HIV was rapidly progressive, suggesting that retroviral replication may have been enhanced by the underlying immunomodulation caused by cyclophosphamide and also by SLE per se.
PURPOSE: To determine whether cerebral atrophy in systemic lupus erythematosus is associated with decreased levels of the neuronal marker N-acetyl-aspartic acid. METHODS: Two groups of patients with systemic lupus erythematosus were studied, those with significant atrophy (n = 11) and those without significant atrophy (n = 10), using proton MR spectroscopy on a 1.5-T imaging unit. The solvent-suppressed, short-echo, volume-localized proton spectroscopy technique showed typical brain metabolites, including N-acetylaspartate, creatine/phosphocreatine, and choline-containing compounds. RESULTS: The N-acetylaspartate-to-creatine/phosphocreatine ratio was smaller in those patients with significant cerebral atrophy (1.68 +/- 0.27) than in those patients with minimal or no atrophy (2.17 +/- .30). The degree of atrophy was negatively correlated with the N-acetylaspartate-to-creatine/phosphocreatine ratio. The choline-to-creatine/phosphocreatine ratio was not significantly altered in systemic lupus erythematosus patients with atrophy. CONCLUSION: These data suggest that cerebral atrophy in systemic lupus erythematosus is associated with neuronal dropout (or damage), which results in decreased N-acetylaspartate ratios. A change in choline ratios is not implicated in the biochemical changes associated with cerebral atrophy. Proton MR spectroscopy may be useful in correlating brain metabolites with cerebral structural changes in patients with autoimmune diseases.
Anti-Ro autoantibodies are frequently found in the sera of patients with Sjögren's syndrome, systemic lupus erythematosus, and subacute cutaneous lupus erythematosus as well as in the sera of mothers of infants with the neonatal lupus syndrome. Close associations have been found between anti-Ro and a number of clinical manifestations, particularly including hematologic cytopenias, heart block, and photosensitive skin rashes. Serologic and genetic associations have been found between anti-Ro and anti-La, rheumatoid factor, hypergammaglobulinemia, the histocompatibility alleles DQ1 and DQ2, and alleles of the T-cell receptor beta chain gene. The origin of anti-Ro and other autoantigens is thought to relate to the etiology of Sjögren's syndrome and systemic lupus erythematosus and remains the most fundamental unanswered question preventing a comprehensive understanding of these diseases.
Keratinocyte cytotoxicity is an important component of the immunopathology of photosensitive lupus erythematosus, and antibody-dependent cell-mediated cytotoxicity (ADCC) has been shown to be an important mechanism by which autoantibodies, especially those specific for SS-A/Ro, can induce keratinocyte damage in models of photosensitive lupus. We provide further evidence that keratinocytes from patients with photosensitive lupus show significantly greater ultraviolet radiation (UVR)-induced cytotoxicity, and that ADCC of these targets is especially enhanced by autologous patient's serum or by anti-SS-A/Ro+ sera. Keratinocytes from normal uninvolved skin of 29 patients with cutaneous lupus erythematosus (LE) were grown in cell culture and tested as targets in cytotoxicity experiments in vitro. Cultured keratinocytes from patients with systemic lupus erythematosus (SLE) and subacute cutaneous lupus erythematosus (SCLE) showed significantly greater cytotoxicity following UVR treatment than did keratinocytes from normal adult controls or from neonatal foreskins (P < 0.01). The same cultures also showed greater UVR-induced binding of IgG from fractionated anti-SS-A/Ro+ preparations. ADCC experiments were also performed using keratinocytes cultured from patients with SLE, SCLE, discoid lupus erythematosus (DLE), and normal controls. When keratinocytes were incubated in autologous serum plus a standard mononuclear cell effector population, the percentage of ADCC observed was significantly greater in cultures containing keratinocytes and sera from the SLE and SCLE patients (P < 0.001). When cultured keratinocytes were added to different IgG antibody probes, plus standard mononuclear effector populations, greater ADCC was seen using the anti-SS-A/Ro probe and keratinocytes from patients with SLE or SCLE. With normal human neonatal keratinocyte targets, the anti-SS-A/Ro probe induced greater ADCC than that seen with anti-ssDNA or normal human serum. We have shown that keratinocytes from patients with some forms of lupus erythematosus (SLE and SCLE) show greater cytotoxicity in vitro when irradiated with UVR, and greater susceptibility to ADCC whether the antibody source is their own serum or an anti-SS-A/Ro probe.
Twenty-three cases of lupus erythematosus, including 15 cases of SLE and 8 cases of DLE treated with Tripterygium wilfordii (TW, three tablets thrice a day, each tablet contains 5 gm of crude TW) were reported. As controls, 19 cases of SLE were treated with prednisone alone at the same time. 9/15 cases in the former group and 6/19 in the latter got improvement after treatment. There was no significant difference between the two groups (P greater than 0.10). However, TW had beneficial effects to remission of arthralgia (7/10 in the former group and 7/15 in the latter group) and erythematosus rash (6/8 in the former group and 5/12 in the latter). Clinically, four out of eight cases of DLE improved after treatment with TW. TW had some toxic effects, such as decrease of peripheral blood leukocyte and kidney damage. Since the toxic dose and effective dose could be quite close, special attention should be paid when the drug was being used, especially on those patients with nephropathy. So the authors suggested that the indication of TW could include the patients of DLE and SLE without nephropathy.
The prevalence of three different types of antiphospholipid antibody in 88 consecutive patients with systemic lupus were 27.2% for lupus anticoagulant (LAC), 31.8% for anticardiolipin antibody (aCL), and 13.6% for falsely positive serologic tests for syphilis (FPSTS). The three tests were correlated, thus confirming the overlapping specificities of this family of antibodies. Although FPSTS was not associated with any particular manifestation of systemic lupus, aCL correlated with thrombosis (p = 0.0001), thrombopenia (p = 0.009), neuropsychiatric features (p = 0.02) and membranous nephropathy (p = 0.001), while LAC correlated with thrombosis (p = 0.001) and hemolytic anemia (p = 0.04). The previously unreported association between membranous nephropathy and aCL might explain some features of the former, particularly the higher incidence of thromboembolic complications and the poorly known relation with renal vein thrombosis.