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Renal transplantation for systemic lupus erythematosus and recurrent lupus nephritis. A single-center experience and a review of the literature.

Prior to 1975 patients with systemic lupus erythematosus were generally not considered candidates for renal transplantation because of concern that immune complex deposition would rapidly destroy the allograft. However, recent evidence suggests that good patient and graft survival rates can be achieved comparable to other renal diseases. Between September 23, 1963 and July 31, 1990, 1070 renal transplants were performed at Washington University Medical Center (WUMC). During this period, 14 patients with SLE (12 female and 2 male) received 16 renal transplants (7 living-related donor [LRD], 1 living-unrelated donor [LURD], and 8 cadaver [CAD]). The mean age at the time of the first transplant was 32.5 +/- 10.3 years. The duration of disease prior to transplant was 88.0 +/- 45.9 months and the duration of hemodialysis prior to transplant was 36.0 +/- 33.7 months. Of these patients, 7/14 (50%) had negative and 3/14 (21%) positive SLE serology pre- and post-transplant, 3/14 (21%) had negative serology pretransplant that became positive posttransplant, and 1/14 (2%) was positive pretransplant and became seronegative posttransplant. Patient survival was 92.8% (13/14), and of the 16 kidneys transplanted 62.5% (10/16) are still functioning with a mean follow-up period of 43.7 +/- 45 months. The current mean serum creatinine was 1.4 +/- 0.26 mg/dl. One noncompliant patient developed recurrent lupus nephritis bringing the total number of cases reported in the literature to seven. The present study demonstrates that patients with SLE can be transplanted with excellent patient and graft survival and function and a low rate of recurrent lupus nephritis. From a review of the literature, there appears to be an association between positive SLE serology pre- and posttransplant and recurrent lupus nephritis.

Adult↗

Reticuloendothelial system Fc receptor function in systemic lupus erythematosus.

Reticuloendothelial system Fc receptor function was measured in 10 patients with systemic lupus erythematosus (SLE) taking corticosteroids, 10 SLE patients not receiving corticosteroids and in 19 controls, 9 of whom were corticosteroid-dependent asthmatics and 10 of whom were healthy. Clearance studies were performed using autologous 51 Cr-labeled erythrocytes that had been sensitized with human IgG anti-Rh(D) [6,700 molecules/cell]. The clearance curves could be split into a fast (t1/2 fast) and a slow (t1/2 slow for any of the groups of patients. The results were correlated with serum C3, C4, DNA-binding, fluid phase 125I-Clq binding, a disease activity index, corticosteroid dose and duration of therapy with corticosteroids. The only significant correlation was an inverse correlation of C4 with the t1/2 slow in SLE patients not on corticosteroids (r=- 0.71, p less than 0.05). The t1/2 slow of the 3 SLE patients with active nephritis (86 +/- 40 min) was significantly different from the 17 SLE patients with inactive nephritis or normal renal function (37 +/- 5 min) (P less than 0.05). We conclude that there is no overall defect of Fc receptor function in our patients with SLE although there is decreased clearance in patients with active lupus nephritis.

Asthma↗

[Clinical and morphological characteristics of lupus nephritis in systemic lupus erythematosus with antiphospholipid syndrome].

AIM: To ascertain clinical and morphological features of lupus nephritis (LN) in systemic lupus erythematosus (SLE) associated with antiphospholipid syndrome (APS). MATERIAL AND METHODS: Immunological markers of SLE and APS, clinical picture, urine indices were examined in 138 patients with SLE, APS and renal dysfunction. RESULTS: LN associated with APS is characterized with marked arterial hypertension, such patients had arterial thromboses more frequently than patients with isolated LN. Patients with anticardiolipin antibodies have arteriolosclerosis, in APS - diffuse interstitial sclerosis. CONCLUSION: Renal impairment in SLE may run not only with LN but also with thrombotic microangiopathy modifying clinical symptoms and course of the disease.

Adolescent↗

[Effects of acute pharmacological blockade of the renin-angiotensin system on intrarenal hemodynamics in patients with systemic lupus erythematosus and systemic scleroderma].

AIM: To evaluate effects of an ACE inhibitor captopril on intrarenal hemodynamics during acute test in patients with lupus nephritis (LN) and chronic sclerodermic nephropathy (CSN). MATERIAL AND METHODS: Renal hemodynamics was studied using ultrasound duplex scanning of renal vessels (UDS) on Vingmed SD-100 unit in 5 patients with SLE with renal affection, 5 patients with scleroderma systematica (SS) with renal affection. The hemodynamic parameters were measured before and 1 hour after administration of 50 mg captopril. RESULTS: LN were found to have low arterial perfusion. CSN patients exhibited low intrarenal blood flow in high resistance of small vessels. Captopril improved intrarenal hemodynamics (enhancement of linear and volume blood flow) in LN patients; in CSN renal artery dilated and resistance of small vessels reduced while renal arterial perfusion increased. CONCLUSION: Improvement of intrarenal hemodynamics in LN and CSN patients in response to captopril allows to recommend administration of ACE inhibitors in SLE and SS with renal damage. Ultrasonic duplex scanning of renal vessels provides detailed information about the condition of intrarenal vessels without invasion.

Angiotensin-Converting Enzyme Inhibitors↗

A patient with systemic lupus erythematosus presenting both central nervous system lupus and steroid induced psychosis.

We describe a patient with systemic lupus erythematosus (SLE) who presented with both steroid induced psychosis and central nervous system (CNS) SLE at different times. The cerebrospinal fluid (CSF) immunoglobulin indices and electroencephalogram were found to be helpful and reliable for the correct diagnosis of both conditions. There have been no reports of patients with SLE who showed both steroid induced psychosis and CNS SLE, and our patient has provided substantial evidence that both conditions can occur in the same individual.

Adult↗

Characteristics of patients with late onset systemic lupus erythematosus in Turkey.

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder mainly affecting young women. In this study, we aimed at investigating the clinical, laboratory and management characteristics of our SLE patients with an age of onset > or =50. Twenty patients with late onset SLE (> or =50 years) were identified from the records, on the basis of their first SLE-related symptoms (Group I). A hundred consecutive SLE patients with initial symptoms before the age of 50 were also selected as controls (Group II). Clinical, laboratory and management characteristics of the patients were recorded according to pre-defined protocol and compared by chi(2), Student's t-test and Fisher's exact test. Comparison of the demographic findings between the Group I (F/M: 18/2) and the Group II (F/M: 90/10) were as follows: the mean age of disease onset was 53.9 +/- 4.5 years vs. 26.3 +/- 9.2 years, mean time of follow-up was 44.2 +/- 40.5 months vs. 50.1 +/- 47.4 months, mean damage index was 0.6 +/- 0.6 vs. 0.58 +/- 1.4. There was no significant difference between the two groups with regard to clinical, laboratory parameters, damage index and immunosuppressive treatment characteristics. SLE-related manifestations were similar in two groups except fever (10% in the Group I vs. 41% in the Group II; p = 0.01). The only two patients with pulmonary fibrosis were found in the Group I (p = 0.027). The clinical and laboratory characteristics and the disease outcome in SLE patients with an age of onset > or =50 years did not show significant differences from the control SLE patients with a younger age of onset.

Adolescent↗

Epstein-Barr virus and systemic lupus erythematosus.

PURPOSE OF REVIEW: Systemic lupus erythematosus is a complex human disease likely influenced by a compilation of necessary, but not individually sufficient, features. Although many genetic and environmental factors are associated, this review will focus on the evolving evidence for key Epstein-Barr virus specific roles. RECENT FINDINGS: Recent studies have shown additional molecular mimicry mechanisms between early events in lupus autoimmunity and specific Epstein-Barr virus responses. In addition, several recent papers have demonstrated increased Epstein-Barr viral load, increased numbers of latently infected peripheral B cells, impaired functional T cell responses, and association of the presence of Epstein-Barr virus DNA in systemic lupus erythematosus patients compared with controls. Additional work has continued to show association of various aspects of Epstein-Barr virus serology with systemic lupus erythematosus and a recent paper outlines differences in the pediatric systemic lupus erythematosus humoral immune response to Epstein-Barr virus nuclear antigen-1 compared with matched controls. SUMMARY: This review will briefly outline the recent advances that show serologic, DNA, gene expression, viral load, T cell responses, humoral fine specificity, and molecular mimicry evidence for differences between systemic lupus erythematosus patients and controls and the impact that these findings have on understanding the role of Epstein-Barr virus in systemic lupus.

Antibody Formation↗

[Cerebral infarct in systemic lupus erythematosus].

INTRODUCTION AND OBJECTIVE: Systemic lupus erythematosus is a syndrome of variable clinical and immunological expression, which may affect any organ. Stroke is an uncommon complication of systemic lupus erythematosus, attributed to many different etiopathogenic mechanisms such as early atherosclerosis, coagulopathy, vasculitis, cardiogenic embolism, etc. Our objectives were to analyze the incidence, clinical and immunological characteristics of this condition and to evaluate the therapeutic approach and evolution. CLINICAL CASES: We present the cases of six patients with cerebral infarcts who also had systemic lupus erythematosus. The frequency of cerebral infarct in our series of systemic lupus erythematosus was 6%. The average age was 45 years (range 13-67). All the cerebral infarcts occurred whilst the systemic lupus erythematosus was active. There were positive antiphospholipid antibodies in three of the patients and two patients had potentially embologenic cardiopathy (33%). Fifty percent of the patients required treatment with cyclophosphamide for their severe systemic disease and 66% received anticoagulants for an antiphospholipid syndrome and/or suspected thromboembolism. The clinical course was satisfactory in all cases but the one who died. CONCLUSIONS: The incidence of cerebral infarct in relation to systemic lupus erythematosus is low, and generally appears during advanced, active phases of the disease. The many etiopathogenic mechanisms involved generally act together so that it is difficult to say which is the main cause, especially in elderly patients. Amongst these mechanisms is the presence of antiphospholipid antibodies, detection of which is essential in this condition, and cardiogenic embolism. The evolution depends mainly on the multi-organ involvement and how early immunosuppressive and/or anticoagulant treatment is started.

Adolescent↗

Sudden cardiac ischaemic death associated with systemic lupus erythematosus.

Association between systemic lupus erythematosus and coronary atherosclerosis has been reported only rarely in the medical literature. Sudden ischaemic cardiac death in such cases is an uncommon terminal event, particularly in young patients. The severity of coronary atherosclerosis in these cases may be similar to those seen in the classic coronary disease. A case of a 22-year-old female systemic lupus patient is presented in whom sudden death was found at autopsy to be due to extensive coronary atherosclerosis.

Adult↗

Neurologic complications of systemic lupus erythematosus.

Nervous system dysfunction occurs in 50 to 75 percent of patients with systemic lupus erythematosus and is a common cause of morbidity and mortality. This may be a direct effect of the disease or an indirect effect of associated infection, metabolic derangement or therapy. Though the pathogenesis is poorly understood, steroids are still the mainstay of therapy. Since the family physician is often the first to encounter the patient with SLE, an awareness of the associated neurologic complications may facilitate early recognition and treatment.

Diagnosis, Differential↗

Systemic lupus erythematosus in pregnancy.

Systemic lupus erythematosus is an inflammatory autoimmune disease that can affect multiple organ systems. The most popular theory regarding the origin of its clinical manifestations is that autoantibodies and circulating immune complexes become trapped in the capillaries of visceral structures.

Antiphospholipid Syndrome↗

Adrenomedullin--a potential disease activity marker and suppressor of nephritis activity in systemic lupus erythematosus.

OBJECTIVES: To investigate whether plasma adrenomedullin (AM) level is elevated in lupus nephritis and to examine if plasma AM level is correlated with systemic lupus erythematosus (SLE) disease activity and severity of lupus nephritis after multivariate adjustment. METHODS: Consecutive SLE patients and healthy volunteers of age >/=16 were recruited from the rheumatology clinics of two hospitals in Hong Kong. SLE patients with nephritis fulfilled the American College of Rheumatology criteria for renal involvement and had percutaneous renal biopsy performed. Subjects were divided into three groups: (i) SLE patients with nephritis, (ii) SLE patients without nephritis and (iii) normal controls. The demographic and clinical variables were compared between these groups of patients and plasma AM level was determined by radioimmunoassay. Factors associated with plasma AM level were explored by regression analysis with adjustment of confounding factors. RESULTS: Sixty SLE patients (39 with nephritis and 21 without) and 23 normal subjects were studied. The plasma AM level of SLE patients was significantly higher than that of normal controls. SLE patients with nephritis had significantly higher plasma AM level than those without nephritis and normal controls (P<0.001). In regression analysis, proteinuria was negatively associated with plasma AM level (P=0.006) whereas SLE disease activity index was positively associated with plasma AM level after multivariate adjustment (P=0.002). CONCLUSIONS: Plasma AM is elevated in lupus nephritis, which correlates with lupus disease activity. It is negatively associated with urine protein excretion although it is unrelated to the type of renal pathology per se. Plasma AM may play a role to suppress the activity of lupus nephritis.

Adrenomedullin↗

Polyarticular Salmonella bacterial arthritis in a patient with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) are at increased risk for infections. Insufficiency of the reticuloendothelial system caused either by immunosuppressive therapy or inadequate opsonisation are mechanisms by which Salmonella infections in particular appear in these patients. Salmonella infections can provoke a polyarticular reactive arthritis while Salmonella bacterial arthritis usually is monarticular. We report on the seriousness of a Salmonella infection (enteritis complicated by polyarticular bacterial arthritis) in a patient with SLE.

Adult↗

Clinical aspects of systemic lupus erythematosus in childhood.

Systemic lupus erythematosus (SLE) is an uncommon childhood illness that is characterized by the formation of autoantibodies and immune complexes, which mediate inflammatory responses in multiple organ systems. Children who develop SLE are frequently very ill at the time of presentation and need careful evaluation to determine which organ systems are involved and how severely, meticulous control of medications to suppress active disease, and close monitoring to avoid complications from both the disease and its treatment.

Bone Diseases↗

Autoantibodies to the heat-shock protein hsp90 in systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) develop multiple autoantibodies to self-antigens. Analysis of autoantibody systems in this and related autoimmune disorders can provide information of etiologic and pathogenetic significance. We report here a previously unrecognized autoantibody to the 90,000-D heat-shock protein, hsp90, a molecule thought to have important functions in the cellular response to stress, virus-induced transformation, steroid hormone receptor action, and cellular activation. Autoantibodies to hsp90 were exclusively of the IgG class, and were detected in approximately 50% of unselected patients with SLE and 2/6 patients with idiopathic polymyositis. Anti-hsp90 antibodies were not detected in sera from 10 normal subjects, 10 patients with rheumatoid arthritis, or 7 patients with scleroderma. The identity of this major intracytoplasmic antigen was established by its specific removal from nonionic detergent cell lysates following immunoabsorption with monospecific rabbit anti-hsp90, and by demonstration of increased synthesis following a 10-min 45 degrees C heat shock. These data define the frequent occurrence of a novel autoantibody to a major heat-shock protein in patients with SLE.

Autoantibodies↗

The nephritis of systemic lupus erythematosus--a review.

Systemic lupus erythematosus (SLE) is a disease of unknown aetiology. Its hallmark is anti-nucleic acid antibodies and it is accompanied by variable clinical, serologic, and histologic signs. Renal involvement in SLE has been documented for over 50 years; it represents one of the most serious complications of this systemic disorder. Although our understanding of the pathogenesis and natural history of lupus nephropathy is still inadequate, it is nevertheless more comprehensive than in most other forms of glomerulonephritis. This paper will review current concepts in the pathogenesis, histologic classification, clinical course, clinico-pathological correlation and therapeutic strategy of lupus nephropathy.

Antimalarials↗

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.

Actuarial Analysis↗