The development and initial validation of the systemic lupus international collaborating clinics/American college of rheumatology damage index for systemic lupus erythematosus

Objective. To develop and perform an initial validation of a damage index for systemic lupus erythematosus (SLE). Methods. A list of items considered to reflect damage in SLE was generated through a nominal group process. A consensus as to which items to be included in an index was reached, together with rules for ascertainment. Each center submitted 2 assessments, 5 years apart, on 2 patients with active and 2 with inactive disease, of whom 1 had increased damage and the other had stable disease. Analysis of variance was used to test the factors physician, time, amount of damage, and activity status. Results. Nineteen physicians completed the damage index on 42 case scenarios. The analysis revealed that the damage index could identify changes in damage seen in patients with both active and inactive disease. Patients who had active disease at both time points had a higher increase in damage. There was good agreement among the physicians on the assessment of damage in these patients. Conclusion. This damage index for SLE records damage occurring in patients with SLE regardless of its cause. The index was demonstrated to have content, face, criterion, and discriminant validity.     

Systemic lupus erythematosus in patients with chronic renal failure

The clinical courses of 36 patients with systemic lupus erythematosus (SLE) in whom chronic renal failure developed and who required dialysis for more than three months were studied. At the time dialysis was initiated, 14 of 36 patients (38.9 percent) had clinically active SLE, but only three of 24 (12.5 percent) had activity in subsequent years while receiving dialysis therapy. In the majority of patients, however, renal disease progressed to end-stage despite clinical quiescence of SLE. During the follow-up period (mean +/- SD, 36 +/- 39.8 months), eight patients died--six from infections and two from cardiac disease. Actuarial survival rates at one, two, and five years after dialysis treatment were 91.1, 78.8, and 68.9 percent, respectively. This study suggests that the progression of renal disease to end-stage in patients with SLE may be mediated by nonimmunologic mechanisms as well as SLE-related immunologic insults. In most of these patients undergoing long-term dialysis, SLE remains clinically inactive despite persistent serologic abnormalities. Survival of the patients undergoing dialysis is comparable with that of the general dialysis population.     

Systemic lupus erythematosus after renal transplantation: patient and graft survival and disease activity. The Dutch Working Party on Systemic Lupus Erythematosus

OBJECTIVE: To determine the outcome of renal transplantation in patients with systemic lupus erythematosus and end-stage renal failure and to compare disease activity after transplantation with disease activity before transplantation. DESIGN: Retrospective case finding using data for an 8-year period from the central registry for renal replacement therapy in The Netherlands. SETTING: Tertiary care hospitals with facilities for renal transplantation in the Netherlands. PATIENTS: Twenty-eight patients who fulfilled at least four of the American Rheumatology Association's criteria for the classification of systemic lupus erythematosus and who received a renal transplant. MEASUREMENTS: Actuarial survival rates for grafts and patients after transplantation, maximal nonrenal scores on the Systemic Lupus Erythematosus Disease Activity Index, and time-adjusted disease exacerbation rates in all patients before and after transplantation. RESULTS: The actuarial graft survival rate at 1 year and 5 years was 68% (95% CI, 47% to 82%) and 54% (CI, 25% to 77%), respectively, whereas the actuarial patient survival rate was 87% (CI, 69% to 96%) at 1 and 5 years. High disease activity was not found to affect graft survival adversely before the start of renal replacement therapy or during dialysis. After transplantation, disease activity per patient and the overall incidence of disease exacerbations decreased. One case of recurrent lupus nephritis was seen. CONCLUSIONS: Patients with systemic lupus erythematosus and end-stage renal failure are excellent candidates for renal transplantation; disease activity after transplantation is sporadic and low, and the recurrence of lupus nephritis is rare.     

Systemic lupus erythematosus without clinical renal abnormalities: renal biopsy findings and clinical course.

The extent and significance of renal biopsy abnormalities in patients with systemic lupus erythematosus (SLE) without clinical renal abnormalities is controversial. We report 11 consecutive SLE patients who were biopsied without clinical renal abnormalities. All 11 patients had mesangial changes either by light microscopy or by immunofluorescent staining, and none had changes of focal or diffuse proliferative glomerulonephritis. Additionally none had deterioration of renal function during the mean follow-up period of 6.3 years.     

Systemic lupus erythematosus in Chinese: The singapore experience

Summary A clinical survey of idiopathic systemic lupus erythematosus (SLE) was conducted in 183 Chinese patients from 1970–1980. The major initial manifestations were skin and mucous membrane involvement (52%), fever and malaise (48%), arthritis and arthralgia (44%). Renal and neuropsychiatric involvements are described in some detail. Five-year survival was 70% and 10-year survival was 60%. The three major causes of death were renal, neurologic and infective. Factors contributing to this relatively high mortality compared with Western series are discussed.     

Systemic lupus erythematosus in Martinique: an epidemiologic study

PURPOSE: To review epidemiological and clinical aspects of systemic lupus erythematosus (SLE) in Martinique, French West Indies. METHODS: Cases of SLE were identified by attending physicians. Patients who presented with at least four of the criteria defined by the American College of Rheumatology were included. Determination of incidence and prevalence included the new cases arising during the 1990-1999 period and 1999 population census results. Probability of survival was based on the use of the Kaplan-Meier estimator. RESULTS: Two hundred and eighty-six patients were studied, including 265 females (92.7%). The average annual incidence was 4.7 cases per 100,000 inhabitants (95% confidence interval [CI]: 2.5-6.9). The prevalence for 1999 was 64.2 cases per 100,000 inhabitants (CI: 56.2-72.2). The mean age at onset was 30 years. Eleven percent of all patients had at least one parent with SLE. Renal disease was present in 139 patients (48.6%), and neurological disorders were diagnosed in 70 patients (24.5%). Patients tested positive for the following antibodies: anti-Sm (37.1%), anti-RNP (58.7%), anti-SSA (47.2%). Mean survival time was: 96.4% (CI: 94.1-98.7) at 5 years, 91.8% (CI: 87.9-95.7) at 10 years. Survival was significantly reduced in patients with end-stage renal disease (n = 40, chi 2 = 6.96, P < 0.01). CONCLUSION: The high incidence of SLE in Martinique and the immunological characteristics of patients were found to be similar to those described in other populations of African descent. The frequency of renal disease and survival rates were identical to those reported in Caucasians.     

Analysis of lupus activity in end-stage renal disease treated by hemodialysis

OBJECTIVE: The activity of systemic lupus erythematosus (SLE) has been reported to decrease in patients who have developed end-stage renal disease (ESRD). However, extrarenal symptoms attributable to the disease activity are noted, especially during the first year of dialysis. We studied the clinical course and evaluate the disease activity of SLE in patients with ESRD on hemodialysis for more than 6 months. SUBJECT AND METHODS: Fourteen patients with SLE who had been initiated on maintenance dialysis at our center between 1982 and 1999 were examined retrospectively. Their clinical details, organ system manifestations, serologic profiles and immunosuppressive treatment regimens were reviewed. Patients with and without postdialysis flaras of SLE were compared statistically. RESULTS: Five patients exhibited 6 SLE flares under treatment with corticosteroids. Two flares occurred within the first year of the initiation of dialysis, and in 1 patient, aggravation of the disease activity was noted 98 months after the initiation of dialysis. Polyarthritis was noted in 5 cases and fever in 4 cases. The serum complement levels decreased in all 6 cases with relapse of SLE activity. Compared with the other 9 patients who did not exhibit SLE relapse, no significant differences were found in 5 patients who did with respect to the demographic and serologic features at the initiation of dialysis. CONCLUSION: We conclude that the disease activity does not always burn out in patients of SLE who show progression to ESRD. SLE flares can sometimes occur even after one year of the initiation of dialysis. SLE patients on dialysis should be carefully followed up by clinical and serological monitoring, and treated by appropriate immunosuppressive therapy.     

Defining response in systemic lupus erythematosus: a study by the Systemic Lupus International Collaborating Clinics group

OBJECTIVE: In a preliminary attempt to develop a drug responder index for patients with systemic lupus erythematosus (SLE), 2 validated disease activity instruments were studied for their responsiveness and compared to a physician visual analog scale (VAS) assessment of disease activity. We attempted to determine whether these validated instruments were useful components in characterizing response in the setting of a clinical trial. METHODS: Eighty paper patients were assessed using the British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Disease Activity Index (SLEDAI) and by physician's assessment of global activity. The cases were arranged in random order and divided into groups of 20 patients and each group was assessed by 20 lupus experts; change in disease activity was recorded at 3 and 6 months compared to baseline using a physician VAS. RESULTS: Four different lupus experts assessed disease activity in all 80 patients at baseline and 3 and 6 months after initiation of therapy using the BILAG and SLEDAI instruments. BILAG and SLEDAI scores correlated well over time; however, in a regression analysis where average physician VAS were chosen as the outcome variable, a significant amount of variation in the average physician VAS not related to the SLEDAI and BILAG scores was noted. CONCLUSION: The physician VAS may be too blunt to assess response in SLE, because even among experienced lupus assessors, there were considerable differences in what influenced scoring decisions.     

Systemic inflammatory response to exhaustive exercise in patients with chronic obstructive pulmonary disease

Systemic inflammation may be present in patients with chronic obstructive pulmonary disease (COPD). Exercise is known to elicit an inflammatory response. We hypothesized that the systemic inflammatory response to exercise might be exaggerated in COPD patients compared to healthy subjects. Sixteen COPD patients and 11 healthy subjects performed a maximal incremental bicycle test. Before and at maximal exercise arterial blood samples were taken to determine circulating catecholamines, (subsets of) leukocytes, acute phase proteins, creatine kinase and myoglobin. At rest, increased levels of norepinephrine and systemic inflammation were present in COPD. The response of catecholamines to exercise was lower in COPD patients (P<0.01), which in part was due to the lower maximal exercise capacity of these patients (P<0.01). Exercise-induced leukocytosis showed similar responses in both groups, but occurred at higher levels in COPD. Although patients had increased levels of CRP at rest (P<0.001), exercise did not affect acute phase proteins. No systemic signs of muscle damage were found. The present study shows that COPD patients are exposed to systemic inflammation that is intensified by exhaustive exercise. The inflammatory response in COPD is not exaggerated compared to healthy subjects but occurs at a higher level and is observed at lower external workload.     

Late recurrence of lupus nephritis in a renal transplant recipient: response to mycophenolate mofetil

Clinically significant recurrence of lupus nephritis in the renal allograft is low, with an incidence of 1 to 3%, and usually occurs within the first 6 years after transplantation. We report an unusual case of a patient with end-stage renal disease caused by lupus nephritis who received a kidney transplant from a living relative; 13 years later, the patient had a severe recurrence of diffuse proliferative lupus nephritis. The patient relapsed after receiving intravenous cyclophosphamide therapy and had a partial response to oral mycophenolate mofetil. In this report we review the risk factors for the recurrence of the systemic lupus erythematosus in the kidney graft and the anti-lupus activity of mycophenolate mofetil.     

Systemic lupus erythematosus pregnancies: the Sarawak experience and review of lupus pregnancies in Asia

We performed a cross-sessional study of all systemic lupus erythematosus (SLE) pregnancies during a 4-year period (2006–2009) to describe the clinical features, maternal and foetal outcomes in our centre. There were 48 pregnancies in 44 women with SLE. Our patients have a mean age of 30.0 years (SD 6.36) and a mean disease duration of 40.67 months (SD 48.23). Our patients have complicated pregnancies: 32.7% have SLE flares, 17.3% have preeclampsia and 48.9% needed caesarean sections. There were 20.0% foetal losses and 17.8% preterm deliveries in our patients. SLE flares contributed to 60.0% of foetal losses in our patients. Lupus pregnancies in our centre generally have a good maternal and foetal outcome comparable to developed countries in Asia. The low incidence of APS, the high usage of hydroxychloroquine and the high SLE remission rate in our patients prior to conceptions contributed to the good outcome.     

Systemic lupus erythematosus in identical twins: a case report

In this report we describe the case of identical twin sisters that developed systemic lupus erythematosus (SLE). These patients have in common major histocompatibility complex class I and class II alleles and identical red blood cell antigens, which is a clear indication of monozygotic twins. Both twins showed high titers of anti-dsDNA antibody. However, only one of them manifested signs of lupus psychosis and was positive for the LE test, rheumatoid factor, anti-Scl 70, anti-SSA, and antiribosomal P antibodies. Both sisters lived together; therefore, the environmental factors were considered to be the same. Interestingly, these patients expressed different types of autoantibodies and the manifestation of disease was also quite different. When one of the twins was diagnosed with SLE, we began to closely follow up signs of the disease in the other twin periodically. This enabled us to promptly diagnose the second twin with SLE and she was successfully treated without progression of the disease. It is important to mention that following up the subsequent history of an identical twin diagnosed with SLE allowed early detection of the disease in the other twin.     

Systemic lupus erythematosus in children: current and emerging therapies

Treatment of children with systemic lupus erythematosus (SLE) is challenging. The therapeutic issues and risks and balances faced by adult patients are further complicated by an unpredictable disease course and long requirement for therapy in children with SLE. Further, non-compliance is a major obstacle to satisfactory outcome which must be recognized and dealt with in every adolescent in our efforts to attain optimal outcome. Treatment with combinations of cytotoxic agents and biologics which result in significant B-cell depletion often provide improved disease control. As our knowledge of the pathogenesis of SLE delineates more specific targets for immunotherapy the incidence of long-term remission rises. Our current emphasis is on therapeutic regimens which will induce remission followed by maintenance therapy in the oncologic model. SLE like neoplastic disease is no longer simply 'treatable'. With appropriate therapy many children with SLE attain sustained remissions. In the foreseeable future childhood SLE may be curable.     

Poor agreement of objectively measured and self-reported physical activity in juvenile dermatomyositis and juvenile systemic lupus erythematosus

To examine the agreement and association between objectively measured and indirectly assessed physical activity levels in patients with juvenile dermatomyositis (JDM) and juvenile systemic lupus erythematosus (JSLE) patients. The sample consisted of 19 JDM patients (age 8 to 22 years) and 20 JSLE patients (age 9 to 18 years). Physical activity level was objectively measured using Actigraph® accelerometers and indirectly assessed by the short-form International Physical Activity Questionnaire (IPAQ). Spearman’s correlation coefficients were calculated to test possible associations between physical activity levels across the two instruments. The Bland–Altman technique was used to calculate bias and limits of agreement. Correlations between objectively measured and indirectly assessed physical activity levels in JDM and JSLE were weak, varying from R?=?0.03 to R?=?0.33 (all p?>?0.05). Total physical activity was correlated between accelerometer and IPAQ in JSLE (R?=?0.51, p?=?0.021). Bland–Altman analyses suggested that IPAQ tended to highly underestimate sedentary time and light physical activity in JDM (mean bias 105.7 and 199.8 min, respectively) and JSLE (mean bias 36.4 and 127.8 min, respectively). Mean biases of moderate-to-vigorous physical activity were also highly variable, ranging from ?42.9 to 54.9 min and ?59.4 to 89.8 min for JDM and JSLE, respectively. IPAQ was shown to not be valid to assess physical activity levels in patients with JDM and JSLE when compared against accelerometry. While the validation of reliable self-reported instruments that measure physical activity in pediatric rheumatic patients remains necessary, the use of validated tools that objectively measure physical activity is recommended in both clinical and research settings.