Free tissue transfer in patients with systemic lupus erythematosus.

Systemic lupus erythematosus is a non-organ specific, autoimmune disease characterised by antinuclear antibodies and vasculitis. This may manifest itself with areas of cutaneous necrosis which may require extensive excision and reconstruction. Major areas of skin and soft tissue loss may require coverage with either local or microvascular free tissue transfer. This presents potential difficulties in patients with generalised vasculitis. We present the case of a professional cellist with extensive soft tissue loss in her dominant hand, successfully reconstructed with a free latissimus dorsi muscle flap, treated with peri- and postoperative anticoagulation and corticosteroids.     

Surgical morbidity in patients with systemic lupus erythematosus

Thirty-six surgical procedures were performed on 29 patients with systemic lupus erythematosus (SLE). Nineteen cases involved active lupus at the time of surgery and 11 were performed on an emergent basis. Most patients had multiple organ involvement and were on some form of systemic therapy at the time of surgery. Thirty-seven postoperative complications were confined to 20 of these cases. Comparing this complicated group with the remaining 16 uncomplicated cases, the patients in the former group had a higher mean dose of steroid preoperatively, more organ involvement by SLE, and more frequent renal involvement; a higher percentage of the cases in this group were emergent rather than elective. The majority of factors examined failed to show predictive value in the outcome of surgery in lupus patients. We conclude that surgical complications are frequent in SLE patients and have identified four factors predictive of increased morbidity.     

Celiac disease in patients with systemic lupus erythematosus

ObjectivesCeliac disease (CD) is one of the most common chronic diseases. Celiac disease has been associated with several autoimmune disorders, but the association with systemic lupus erythematosus (SLE) as a systemic autoimmune disease is still controversial. In this study, we aimed to determine the prevalence of biopsy-proven CD in patients with SLE, and to determine the clinical symptoms and laboratory data in these patients.Material and methodsIn a cross-sectional study, SLE patients at a referral clinic were evaluated for gastrointestinal symptoms between March and December 2016. Patients were evaluated by a gastroenterologist, and upper gastrointestinal endoscopy with intestinal biopsy was performed if deemed necessary. The clinical symptoms, laboratory data, and endoscopy results were recorded and compared between groups.ResultsIn total, 130 patients were evaluated in this study. Gastrointestinal symptoms were present in 40% of the patients. Endoscopy was performed in all SLE patients with gastrointestinal symptoms. Four patients (3%) were diagnosed as having CD based on biopsy results and response to a gluten-free diet. Anti-endomysium antibody (AEA) was found to be 100% sensitive and 99.2% specific for the diagnosis of CD in SLE patients, and anti-gliadin antibody (AGA) had a 50% sensitivity and 98% specificity. Patients with comorbid CD and SLE were significantly more likely to have diarrhea, abdominal pain, nausea/vomiting, recurrent oral aphthosis, and anemia.ConclusionsThe results of this study suggest that a significant association is present between CD and SLE. We found a prevalence of 3% for biopsy-proven CD in patients with SLE, which is five times the prevalence of CD in the general population.     

Cardiac surgery in patients with systemic lupus erythematosus

Cardiac surgery was infrequently performed in patients with systemic lupus erythematosus (SLE), and its clinical outcome was reported only in small series. We sought to evaluate the clinical outcome of cardiac operation in patients with SLE. Between January 1996 and March 2005, 9 patients with SLE underwent cardiac surgery at the authors' hospital. Six patients underwent coronary artery bypass grafting (three conventional and three on-pump beating heart), two patients underwent valve replacement and 1 patient underwent simultaneous heart-kidney transplantation. All 6 patients with coronary artery bypass grafting had saphenous venous grafts and two of them had additional left internal mammary artery graft. The overall in-hospital mortality rate was 11% (1/9). Major postoperative complications occurred in 4 patients (44%) including profuse postoperative bleeding, ventricular tachycardia and early graft thrombosis. There were two late deaths including sudden cardiac death and sepsis. The median follow-up duration was 23 months (range, 1-110). In conclusion, although the postoperative complication was common, cardiac operation could be performed in patients with SLE.     

Hip arthroplasty in patients with systemic lupus erythematosus

Forty-three prosthetic hip replacements (twenty-nine conventional total hip replacements and fourteen bipolar endoprosthetic replacements) were implanted between January 1971 and June 1982 in thirty-one patients who had systemic lupus erythematosus. All but four patients had stage-III or IV osteonecrosis of the femoral head. The median age at operation was forty-three years, and the median length of follow-up was fifty-seven months. Ratings were good or excellent for all but three total hip arthroplasties at a mean of sixty-six months of follow-up. Complications included delayed wound-healing (in approximately 15 per cent) and superficial wound infection (in approximately 10 per cent). The occurrence of these complications could not be correlated with the use of corticosteroids at the time of the operation. Twenty-five per cent of the patients, who were a mean of forty-three years old at operation, died less than five years postoperatively from complications related to systemic lupus erythematosus. Conclusions regarding the systemic effects of hip arthroplasty in patients with systemic lupus erythematosus could not be drawn on the basis of this study. Total hip arthroplasty uniformly provided a good or excellent result in patients of all ages who had systemic lupus erythematosus at a mean length of follow-up of sixty-six months. An increased incidence of local wound complications, which were unrelated to the use of corticosteroids, should be expected in patients with systemic lupus erythematosus who undergo prosthetic arthroplasty of the hip.     

Pneumococcal infection in patients with systemic lupus erythematosus

Objective

Our study aimed to analyze the risk factors associated with the occurrence and severity of pneumococcal infection (PI) in systemic lupus erythematosus (SLE) patients.

Glomerular podocytopathy in patients with systemic lupus erythematosus

A series of patients with systemic lupus erythematosus (SLE) and proteinuria were studied to determine whether nephrotic-range proteinuria was associated with diffuse epithelial cell foot process effacement in the absence of peripheral glomerular immune aggregate deposition. Biopsies from patients with known or suspected SLE and a histologic diagnosis of (1) normal by light microscopy, (2) mesangial proliferative glomerulonephritis, or (3) focal segmental glomerulosclerosis were studied. Biopsies were excluded when they demonstrated endocapillary proliferation or necrosis by light microscopy or electron-dense glomerular basement membrane deposits by electron microscopy. Patients were required to fulfill four of 11 American Rheumatologic Association criteria for the diagnosis of SLE, and proteinuria could not be associated with nonsteroidal anti-inflammatory drug use. Eighteen biopsies were studied, eight from patients with nephrotic-range proteinuria (>/=3 g/d) and 10 from patients with non-nephrotic proteinuria. The time from diagnosis of SLE to biopsy was shorter for nephrotic patients that for nonnephrotic patients. Seven of eight biopsies from nephrotic patients demonstrated at least 80% foot process effacement, whereas no biopsy from a nonnephrotic patient exhibited >20% effacement. There were no other significant pathologic differences between the nephrotic and nonnephrotic patients. The single common morphologic feature associated with nephrotic proteinuria was diffuse visceral epithelial cell foot process effacement. It is concluded that the development of nephrotic-range proteinuria in patients with SLE without peripheral immune aggregate deposition or endocapillary proliferation on renal biopsy is more likely a manifestation of SLE than the coexistence of idiopathic minimal-change glomerulopathy and SLE.     

Fragmented QRS in patients with systemic lupus erythematosus

Introduction. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with a variety of clinical features. Cardiac involvement is present in more than half of the patients with SLE. Fragmentation of QRS (fQRS) is presumed marker of cardiovascular risk and has not been previously evaluated in SLE. Methods. A total of 56 women previously diagnosed with SLE were recruited. In addition, a control group consisting of 51 healthy people was formed. QRS complexes were also evaluated in terms of fragmentations. All patients with SLE and control subjects underwent transthoracic echocardiographic examination. Erythrocyte sedimentation rate and C-reactive protein levels were also obtained. Results. Frequency of fQRS was higher in patients with SLE (41% vs. 21%, p = 0.03). Left ventricular posterior wall thickness and mass index were higher in the patients with SLE. CRP levels and age were significantly higher, and disease duration was significantly longer in the fQRS(+) group (p = 0.02, 0.01, and 0.006, respectively). Conclusion. A careful cardiovascular evaluation and follow-up is essential to continuously improve survival in SLE. For this purpose, fQRS may be used for the early detection in patients with SLE.     

Coronary artery bypass grafting in patients with systemic lupus erythematosus.

OBJECTIVE: Few reports exist on the results of coronary artery bypass grafting (CABG) in patients with systemic lupus erythematosus (SLE). METHODS: We retrospectively reviewed eight CABG in seven SLE patients. In early and late postoperative angiography, all grafts were evaluated for occlusion, development of string sign, or presence of significant stenosis. The early and late results were compared. The pathological studies were performed on the segments of the internal thoracic artery (ITA) and saphenous vein collected from each patient. Atherosclerosis of the ITA was analyzed using the subjective evaluation proposed by Kay et al. (Kay HR, Korns ME, Flemma RJ, Tector AJ, Lepley D. Atherosclerosis of the internal mammary artery. Ann Thorac Surg 21;1976:504-507) scale 0-4 (0 = normal, 1 = minimal disease, 2 = less than 25% luminal narrowing, 3 = 25-50% narrowing, and 4 = greater than 50% narrowing). RESULTS: The patients consisted of three men and four women with a mean age of 59.8 years. Co-morbid diseases were frequent and there were three patients (37.5%) with renal failure (two dialysis patients, one with renal dysfunction) and two patients with severe atherosclerosis of the aorta. The ITA was used in four patients. Saphenous vein graft was used in seven patients. Concomitant procedures included aortic valve replacement and mitral annuloplasty, mitral valvuloplasty and tricuspid annuloplasty, mitral valve replacement and tricuspid annuloplasty (TAP). There was one hospital death (12.5%). Early patency rates were 87.5% (21/24). No other atherosclerotic changes or stenosis suggesting vasculitis were noted. In pathological studies, there was no significant atherosclerosis in the six ITA specimens from four patients, although three patients had degree two atherosclerosis. No vasculitis was found in ITA or saphenous vein grafts. During the mean follow-up period of 35.3 months (range, 5-91 months), there was one non-cardiac late death. Late restudy (in three patients, 12, 57 and 64 months later respectively) revealed no deterioration in either ITA or vein grafts. Overall prognosis after the operation in SLE patients appears to be good. No other cardiac events were observed, and patients demonstrated marked clinical improvement. CONCLUSIONS: CABG in SLE patients can be performed with acceptable morbidity and mortality. Our data so far reveals no evidence to preclude the use of ITA and vein grafts in SLE patients.     

Ruptured cerebral aneurysms in patients with systemic lupus erythematosus

Two cases of ruptured aneurysms in the cerebral arteries in patients with established systemic lupus erythematosus are presented. A 32-year-old woman with a 3-year history of systemic lupus erythematosus was found to have a ruptured cerebral aneurysm at the top of the basilar artery. Another 38-year-old woman with a 4-year history of lupus erythematosus had a ruptured aneurysm in the anterior communicating artery. Both were treated surgically. Cerebral aneurysms associated with systemic lupus erythematosus are reviewed in the literature and the pathogenesis of these aneurysms is discussed.     

Vitamin D deficiency in patients with active systemic lupus erythematosus

Summary  We investigated the effects of disease activity on bone metabolism in 36 patients with systemic lupus erythematosus (SLE). Changes in bone remodeling were not explained by corticosteroid use. A high prevalence of 25OHD deficiency in SLE patients indicates the need for vitamin D replacement, mainly during high disease activity periods. Introduction  We investigated the effects of SLE disease activity on bone metabolism, their relation to inflammatory cytokines and vitamin D levels. Methods  We performed a cross-sectional analysis of 36 SLE patients classified according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) in high activity (group I: 12 patients, mean age 29.6 years) or in minimal activity (group II: 24 patients, mean age 30.0 years), and compared them to normal controls (group III: 26 women, 32.8 years). Serum calcium, phosphorus, parathyroid and sex hormones, bone remodeling markers, interleukin (IL)-6, soluble IL-6 receptor (sIL-6R), IL-1, tumor necrosis factor-α (TNF), 25-hydroxivitamin D (25OHD), and 1,25-dihydroxyvitamin D3 were measured, plus bone mineral density. Results  All cytokines were significantly higher in SLE groups; IL-6 could differentiate SLE patients from controls. In group I, 25OHD levels were lower (P < 0.05), which was related to the SLEDAI (R = -0.65, P < 0.001). In multiple regression analysis, the 25OHD level was associated with SLEDAI, osteocalcin and bone-specific alkaline phosphatase. The SLEDAI score was positively correlated with all measured cytokines and especially TNF (R = 0.75, P < 0.001). Conclusions  SLE patients demonstrated changes in bone remodeling strongly related to disease activity. A high prevalence of 25OHD deficiency was observed in SLE patients, indicating the need for vitamin D replacement.     

Abnormal antidiuretic hormone secretion in patients with systemic lupus erythematosus

There have been anecdotal reports describing patients with systemic lupus erythematosus (SLE) and inappropriately elevated secretion of antidiuretic hormone (ADH), but no systematic studies of ADH and its metabolism in SLE have been performed. We measured plasma ADH levels in 36 stable SLE patients with normal renal function and examined the relationship of the circulating ADH concentration to clinical disease activity and effective extracellular fluid volume as reflected by peripheral plasma renin activity (PRA) and plasma aldosterone concentration. The mean ADH level was elevated, 11.4 +/- 1.0 microU/ml (normal 0.4-1.4 microU/ml), while mean PRA and aldosterone were 5.4 +/- 0.6 ng/ml/h and 10.6 +/- 1.6 ng/100 ml, respectively. When patients were divided into two groups according to disease duration, those with SLE for 2 years or more had significantly higher plasma ADH levels (13.9 +/- 1.4 vs. 7.7 +/- 0.9 microU/ml; p less than 0.001 and urinary osmolality (697 +/- 63 vs. 445 +/- 49 mosm/kg; p less than 0.02) compared to those with SLE of less than 2 years duration. No differences in serum Na+, K+, PRA, plasma aldosterone concentration, C3, or 24-hour urinary protein excretion were noted between these two groups. Six patients with SLE for less than 2 years underwent a standard water load (20 ml/kg); in 3/6 there was a paradoxical increase in the plasma ADH concentration. These findings indicate that SLE is associated with elevated plasma ADH levels that increase with prolonged disease duration. This abnormality is unrelated to the usual serologic indices of SLE activity, effective extracellular fluid volume status, or any apparent renal unresponsiveness to ADH.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary function in children with systemic lupus erythematosus.

BACKGROUND: Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. METHODS: After a mean of 4.5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). RESULTS: In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage of patients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. CONCLUSIONS: In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease.     

Coronary artery bypass grafting in patients with systemic lupus erythematosus

Abstract    Coronary artery disease remains a major cause of mortality and morbidity with systemic lupus erythematosus (SLE). We report two cases of coronary artery bypass grafting (CABG) associated with SLE. The first patient (a 45-year-old woman) underwent CABG operation for left main and two-vessel coronary disease using cardiopulmonary bypass. Successful CABG was done using off-pump technique in the second patient (a 39-year-old woman) under hemodialysis therapy. Both patients showed good postoperative outcome without complications.     

Mesangial cell-binding anti-DNA antibodies in patients with systemic lupus erythematosus

The mechanisms by which anti-DNA antibodies contribute to the pathogenesis of lupus nephritis (LN) remain to be elucidated. This study investigates the binding of polyclonal anti-DNA immunoglobulins from patients with systemic lupus erythematosus (SLE) to human mesangial cells (HMC) in vitro. Testing of cross-sectional serum samples from 280 LN patients (108 during active disease; 172 during remission), 35 SLE patients without renal involvement, 72 patients with non-lupus primary glomerular diseases, and 37 healthy subjects with a cellular enzyme-linked immunosorbent assay showed significant IgG mesangial cell-binding activity in patients with SLE, particularly those with active LN (P < 0.0001). Significant HMC-binding activity was demonstrated in 83.9%, 42.8%, and 47.1% of patients with active LN, inactive LN, and non-renal SLE, respectively. This was predominantly attributed to binding by anti-DNA antibodies, and immune complex binding accounted for 4.6%, 3.5%, and 2.8% of seropositive samples in the respective groups. Longitudinal studies in 27 LN patients demonstrated correlation between serial levels of anti-DNA antibodies, serum HMC-binding activity, and disease activity in 18 patients (66.7%). Affinity-purified polyclonal IgG anti-DNA antibodies from sera with HMC-binding activity showed significant binding to cultured HMC, and to a lesser extent glomerular and proximal tubular epithelial cells and human umbilical vein endothelial cells, but not tumor cell lines, peritoneal mesothelial cells, bronchial epithelial cells, or fibroblasts. The binding of anti-DNA antibodies to HMC was increased 1.47-fold (P = 0.0059) after the removal of Ig-associated DNA by DNase treatment, but it was unaffected by DNase treatment of HMC membrane. Controlled trypsinization of membrane proteins in HMC resulted in a 1.26-fold (P = 0.0025) increase in their binding by anti-DNA antibodies. In conclusion, subsets of anti-DNA antibodies from patients with SLE are capable of binding to HMC. The association of such binding with renal involvement and disease activity and its modulation by DNA concentration suggest that Ig binding to HMC can be a potential marker for disease activity in selected patients and that the binding of anti-DNA antibodies to HMC may be a pathogenetic mechanism in LN.     

Outcome of renal transplantation in patients with systemic lupus erythematosus

Renal transplantation is considered to be a good treatment option for patients with systemic lupus erythematosus (SLE) and end-stage renal disease. However, in patients with glomerular diseases, the outcome of renal transplantation can be adversely affected by recurrence of the original disease. Furthermore, the post-transplant course might be complicated by pre-transplant morbidity and treatment history. We studied the outcome of renal transplantation in patients with SLE who underwent transplantations in our center between 1968 and 2001. Patient and graft survival were compared with a matched control group. We specifically looked for any evidence of recurrent disease. There were 23 patients (two male, 21 female) with a mean +/-SD age of 34+/-12 years at transplantation. One patient developed renal failure with serological evidence of SLE activity at 61 months after transplantation. In the absence of urine abnormalities we favored the diagnosis of rejection, although recurrence of lupus nephritis could not formally be excluded. This was the only case of a possible recurrence of lupus nephritis. Two other patients developed extra-renal manifestations of SLE at 6 and 17 months after transplantation. Patient and graft survival rates at 5 years after transplantation were 86% and 68%, respectively. Survival rates were not significantly different from those of a matched control group, 95% and 78%, respectively. Recurrence of SLE after transplantation is rare. The results of renal transplantation in patients with SLE do not differ significantly from a matched control group. Renal transplantation is a good alternative for renal replacement therapy in patients with lupus nephritis.     

Thymoma and systemic lupus erythematosus.

The simultaneous occurrence of a thymoma and systemic lupus erythematosus (SLE) is reported. Disease state associated with thymoma are reviewed and the possible immunological basis for this spectrum of diseases is discussed. The role of angiography in the diagnosis of thymic tumours is described.