New onset of lupus nephritis in two patients with SLE shortly after initiation of treatment with belimumab

PurposeBelimumab is currently approved for the treatment of patients with active SLE despite standard treatment. However, it has not been formally tested for patients with lupus nephritis because such patients had been excluded from the clinical trials. In this report, we present two patients with SLE who developed lupus nephritis de novo shortly after belimumab treatment initiation; both patients improved rapidly upon belimumab discontinuation.ResultsThe first patient (a 30-year-old female, with a 15-year disease duration, receiving prednisolone, hydroxychloroquine, and azathioprine, with no previous history of nephritis that was repeatedly anti-dsDNA negative) had exacerbation of a facial butterfly-like rash developed after 3 months of belimumab treatment initiation. Concomitantly, her urinalysis became abnormal for the first time during her long follow-up (15–20 red blood cells per hpf, and a 24-h urine protein of 1600 mg), and a renal biopsy documented the diagnosis of a Class III (WHO classification). Her anti-dsDNA titers became highly positive for the first time. Belimumab was discontinued and her proteinuria and abnormal urinalysis reverted to normal rapidly, and before MMF administration was approved by local regulatory authorities. Our second patient (a 38-year-old female with a 19-year disease duration) was being treated with prednisone and azathioprine. Two months following belimumab treatment initiation, she became edematous and had an active urine sediment (50–60 rbc per hpf, dysmorphic, and a 24-h urine protein levelabove 6000 mg) for the first time during her disease course. Her renal biopsy was compatible with a Class V membranous nephritis. Belimumab was discontinued and MMF (2 g/d) was substituted for azathioprine with her urinary protein declining to 2.7 g/d just 10 days afterwards.ConclusionsIn this report, apart from our two patients, we discuss the relevant literature consisting of a handful of studies and case reports. The studies analyze patients with renal involvement treated with belimumab and are inconclusive. There are only a few case reports in which belimumab along with other agents had a potential benefit, although not straightforward. There is only one case report with striking similarities to the two patients with SLE we report herein. It could be claimed that belimumab was unable to prevent the appearance of lupus nephritis during a potentially serious disease exacerbation. Certainly, a causative association between belimumab treatment and the de novo appearance of lupus nephritis cannot be claimed because of our report. However, a potential association between belimumab treatment and the development of such a serious manifestation cannot be entirely excluded. In support of the latter hypothesis is the quick resolution/significant reduction of proteinuria shortly after belimumab discontinuation and before other treatment measures had any reasonable effect. Studies evaluating the potential usefulness of belimumab in patients with lupus nephritis are currently ongoing; until then, one should keep in mind unanswered questions as far as renal safety is concerned.     

血清C1q抗体水平与系统性红斑狼疮活动性和狼疮肾炎的关系

目的分析探讨血清C1q抗体水平与系统性红斑狼疮（SLE）活动性以及狼疮肾炎之间的关系。方法采用ELISA方法检测92例SLE患者C1q抗体水平。并与其他SLE活动性指标进行相关分析。结果SLE患者C1q抗体阳性率为67.4%。活动性狼疮组的C1q抗体阳性率及C1q抗体水平显著高于非活动性狼疮组（P〈0.001）。活动性狼疮肾炎组C1q抗体阳性率（P〈0.05）和C1q抗体水平（P〈0.01）显著高于非活动性狼疮肾炎组。联合抗dsDNA抗体检测,没有1例活动性狼疮肾炎患者的C1q抗体和抗dsDNA抗体同时阴性。结论血清C1q抗体与狼疮活动以及活动性狼疮肾炎关系密切,C1q抗体的检测有助于活动性狼疮的诊断,联合抗dsDNA抗体的检测是活动性狼疮肾炎的特异性检测指标。     

Mizoribine therapy for patients with lupus nephritis: the association between peak mizoribine concentration and clinical efficacy

The clinical efficacy of mizoribine (MZR; 4-carbamoyl-1-b-d-ribofuranosylimidazolium) in patients with lupus nephritis was investigated. Thirteen Japanese patients with biopsy-proved lupus nephritis were enrolled in this study. A change in global assessments score, total protein (TP) of serum, serum creatinine, creatinine clearance (Ccr), proteinuria, titers of serum anti-ds DNA antibody, C3, C4, and hemolytic complement activity (CH50) were examined. Following MZR treatment, the level of urinary protein decreased (P < 0.05), whereas the level of Ccr increased (P < 0.05). Moreover, the level of TP significantly increased from 5.5 g/dl to 6.3 g/dl (P < 0.01) and the level of C3 increased significantly (P < 0.01). However, there was no change in the levels of both C4 and CH50. The titer of anti-ds DNA antibody significantly decreased (P < 0.05). The dosage of prednisolone could be tapered from 24.8 mg to 14.9 mg daily during the period. The clinical effects associated with MZR concentration in the blood revealed that there was a significant correlation between the peak MZR blood concentration of more than 0.66 μg/ml and clinical improvement (P = 0.021). Our results suggest that an optimal MZR blood concentration was important for the treatment of lupus nephritis. The first two authors contributed equally to this work.     

Mizoribine therapy for patients with lupus nephritis: the association between peak mizoribine concentration and clinical efficacy

Abstract

The clinical efficacy of mizoribine (MZR; 4-carbamoyl-1-b-d-ribofuranosylimidazolium) in patients with lupus nephritis was investigated. Thirteen Japanese patients with biopsy-proved lupus nephritis were enrolled in this study. A change in global assessments score, total protein (TP) of serum, serum creatinine, creatinine clearance (Ccr), proteinuria, titers of serum anti-ds DNA antibody, C3, C4, and hemolytic complement activity (CH50) were examined. Following MZR treatment, the level of urinary protein decreased (P < 0.05), whereas the level of Ccr increased (P < 0.05). Moreover, the level of TP significantly increased from 5.5?g/dl to 6.3?g/dl (P < 0.01) and the level of C3 increased significantly (P < 0.01). However, there was no change in the levels of both C4 and CH50. The titer of anti-ds DNA antibody significantly decreased (P < 0.05). The dosage of prednisolone could be tapered from 24.8?mg to 14.9?mg daily during the period. The clinical effects associated with MZR concentration in the blood revealed that there was a significant correlation between the peak MZR blood concentration of more than 0.66?µg/ml and clinical improvement (P = 0.021). Our results suggest that an optimal MZR blood concentration was important for the treatment of lupus nephritis.     

Evaluation of visfatin in patients with systemic lupus erythematosus: Correlation with disease activity and lupus nephritis

IntroductionRenal involvement affects about 50% of SLE patients accounting for significant morbidity and mortality in these patients. The adipokine “visfatin” acting as a growth factor for B-lymphocyte-precursors, exerts several proinflammatory functions. It was demonstrated as a marker of endothelial dysfunction (ED) in chronic kidney disease (CKD) thus could be a factor linking inflammation in SLE and kidney disease.Aim of the workTo assess serum visfatin level in SLE patients and its correlation to disease activity and lupus nephritis (LN) in these patients.Patients and methodsSerum level of visfatin using enzyme-linked immunosorbent assay (ELISA), chemical and immunological markers of SLE and LN were measured in 40 SLE patients and 40 age and sex matched healthy controls. Disease activity and renal involvement were assessed using SLE Disease Activity Index (SLEDAI) and Renal SLEDAI respectively further dividing patients into active versus inactive and LN versus non-LN respectively. Renal biopsies were taken from LN subgroup and were classified according to the modified WHO classification.ResultsA significantly higher serum visfatin level was found on comparing SLE patients (mean 109 ± 180 ng/ml, median18) with controls (mean 9.4 ± 11 ng/ml, median2.5) with statistically highly significant difference (z = 5.2, P < 0.001). Also there was a statistically significant difference as regards serum visfatin level between active SLE patients (mean 173 ± 111 ng/ml, median 14) and inactive patients (mean 139 ± 88 ng/ml, median 5) (z = 2.1, P < 0.05) as well as between patients with LN (mean 226 ± 180 ng/ml, median18) and patients with no LN (mean 101 ± 140 ng/ml, median 8(2-229)) (z = 2.1, P < 0.05). Visfatin had a highly significant positive correlation with disease duration (r = 0.48, P < 0.001), SLEDAI (r = 0.62, P < 0.001) as well as ESR, CRP and, renal score (r = 0.45, 0.35, and 0.65, respectively) while inverse correlation with estimated GFR (r = ?0.614) and C3 and C4 titre (r = ?0.26, r = ?0.35, respectively) was recorded. Visfatin showed high sensitivity in detecting active SLE and LN 83% and 85%, respectively.ConclusionSerum visfatin is strongly associated with LN in SLE patients and is a promising biomarker for prediction of renal involvement in these patients. It reflects SLE activity specially LN activity namely renal score and GFR decline. Further prospective studies are required to confirm visfatin as a destructive mediator of predictive and prognostic value in active lupus nephritis.     

Maternal and fetal outcomes of 72 pregnancies in Argentine patients with systemic lupus erythematosus (SLE)

The purpose of the following study was to analyze maternal and fetal outcomes in pregnant patients with systemic lupus erythematosus (SLE) and the influence of SLE exacerbations on those pregnancies. Seventy-two pregnancies in 61 SLE patients treated between January 1986 and February 2004 in Hospital de Clínicas “José de San Martin” were reviewed retrospectively. Patient age was 28.1 ± 6.2 years (mean±standard deviation [SD]). Mean SLE duration was 4.5 ± 3.2 years (range 6 months–10 years). No patient acquired the disorder during gestation. Four (5.5%) patients had signs of active disease at the beginning of her pregnancy. Sixteen patients, accounting for 20 pregnancies, had a history of lupus nephritis. Nine patients met secondary antiphospholipid syndrome criteria and had 13 pregnancies. There were 14 exacerbations of the disease during pregnancy (19.4%), with most flares being mild. The most common obstetric complications were gestational hypertension in 15 pregnancies (20.8%) and preeclampsia in 8 pregnancies (11%). Forty-six percent of pregnancies ended in preterm deliveries. There were 62 live births (1 twin birth; 85%), 6 stillbirths (8%), and 5 spontaneous abortions (7%). Thirty-nine percent of newborns had low birth weight. Adequate pregnancy follow-up and delivery care by an interdisciplinary team in Argentine SLE patients with no pre-gestational preparation resulted in maternal and fetal outcomes similar to those seen in world reference centers.     

Serum interleukin-18 levels in patients with systemic lupus erythematosus: Relation with disease activity and lupus nephritis

Aim of the workTo further investigate the possible role of IL-18 in the pathogenesis of systemic lupus erythematosus (SLE) and development of lupus nephritis (LN), and to explore its relationship with pathological classes of LN, degree of acute renal activity and chronic damage.Patients and methodsForty-one SLE patients with LN, thirty-one lupus non-nephritis patients and fifteen age and sex matched healthy controls were enrolled in this study. SLE patients were subjected to disease activity assessment by SLEDAI, renal disease activity assessment by the Systemic Lupus International Collaborating Clinics (SLICC) Renal Activity Score, laboratory investigations including measurement of serum interleukin-18 using Enzyme Linked Immunosorbent Assay. Renal biopsy was obtained from LN patients and pathological classification was made according to World Health Organization (WHO) criteria. Analysis of activity and chronicity indices was done on these biopsy specimens.ResultsSerum levels of IL-18 were significantly higher in patients with LN than lupus non-nephritis patients and healthy controls (p < 0.001). There were significant correlations between IL-18 and SLEDAI (p = 0.002), proteinuria (p = 0.027), renal activity score (p = 0.003) and activity index (p = 0.039) in patients with LN. There was no significant difference in the serum levels of IL-18 between WHO classes of LN.ConclusionIL-18 appears to have a pathogenic role in the development of SLE and plays a crucial role in triggering inflammation in LN. Serum IL-18 levels could be a useful biomarker to assess the activity of renal disease in SLE.     

Prospective study of low-dose cyclosporine A in patients with refractory lupus nephritis

We evaluated the efficacy and safety of low-dose cyclosporine A (CsA) in patients with refractory lupus nephritis. Nine patients with systemic lupus erythematosus who had lupus nephritis resistant to previous treatment with glucocorticoids and immunosuppressants other than CsA were enrolled in a prospective, open-label study. All patients initially received 2.5 mg/kg per day of CsA; the dosage was adjusted to reach a blood trough level of 80–150 ng/ml. The urinary protein concentration decreased significantly 2 weeks after the initiation of treatment. After 30 weeks of CsA treatment, the mean urinary protein concentration was more than 50% lower than the baseline value, and urinary casts had decreased significantly. There were no significant changes in the levels of serum creatinine, serum anti-double-stranded DNA antibodies, or CH50 during any part of the study. The dose of glucocorticoids was significantly tapered by approximately 50%, without any disease flare. Hypertension developed in one patient, but was controlled with antihypertensive agents. Our results suggest that low-dose CsA therapy is an effective and less toxic alternative to conventional cyclophosphamide therapy for the management of refractory lupus nephritis.     

Methodological considerations in comparing access to Pre-emptive renal transplantation between SLE and other ESRD causes in the USRDS

Background

We compared pre-emptive transplant rates between SLE and non-SLE end-stage renal disease (ESRD) from the U.S. Renal Data System (USRDS) and investigated the potential influence of frequency matching and primary ESRD causes in the non-SLE group.

Neutrophil/lymphocyte and platelet/lymphocyte ratios are useful predictors comparable to serum IL6 for disease activity and damage in naive and relapsing patients with lupus nephritis

Neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) have become recently useful as predictive and prognostic tools in patients with various medical conditions.Aim of the workTo evaluate NLR and PLR in systemic lupus erythematosus (SLE) and their relation to disease clinical characteristics, nephritis, disease activity and damage.Patients and methodThe study involved 110 Egyptian SLE patients; 80 with lupus nephritis (naive and relapsing) and 30 without as well as 50 matched control. Patients were subjected to full clinical examination, SLE disease activity index (SLEDAI) scoring, and damage using Systemic Lupus International Collaborating Clinics Damage Index (SLICC-DI). Laboratory and immunology profiles included the complete blood count (CBC) with differential white blood cell counts and estimation of both NLR and PLR, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), protein/creatinine ratio, anti-nuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-dsDNA), serum complements and interleukin-6 (IL-6) levels.ResultBoth NLR and PLR were significantly higher in SLE patients (4.8 ± 1.4 and 281.7 ± 66.7 respectively) compared to controls (3.8 ± 2 and 138.2 ± 50.4). Both ratios were significantly elevated in patients with active lupus nephritis (at presentation or as a flare) and were comparable between naive and relapsing lupus nephritis patients. In lupus nephritis patients, NLR and PLR significantly correlated with proteinuria, ESR, hypocomplementemia, IL6, SLEDAI and SLICC-DI. The best NLR cut-off value to predict nephritis activity was 5.65, whereas the best PLR cut-off value was 316.5.ConclusionNLR and PLR appear to be potentially useful cheap parameters of activity, relapse and severity in SLE patients with nephritis.     

Serum level of galectin-9 in systemic lupus erythematosus patients with lupus nephritis: Relation to clinical characteristics and disease activity

Aim of the workTo assess galectin-9 (Gal-9) level in the serum of systemic lupus erythematosus (SLE) patients with and without renal involvement and clarify its relation with disease activity.Patients and methods50 SLE patients; 25 with lupus nephritis (LN) and 25 without as well as 25 controls were studied. Systemic Lupus International Collaborating Clinics (SLICC) renal activity score and SLE disease activity index 2000 (SLEDAI-2 K) were determined. Serum Gal-9 was measured in all participants.ResultsGal-9 level was significantly elevated in SLE patients with (16.7; 11.6–33.7 ng/ml) and without (15.9; 11.8–25 ng/ml) compared to controls (3.9; 2.8–5.4 ng/ml) (p < 0.001) but was comparable between the patients groups (p = 0.83). In LN patients, serum Gal-9 and SLICC renal activity score significantly correlated (r = 0.48, p = 0.016). Serum Gal-9 significantly correlated with SLEDAI-2 K in patients with (r = 0.71, p < 0.001) and without (r = 0.95, p < 0.001) LN, with anti-double stranded deoxyribonucleic acid (anti-ds-DNA) titers (with r = 0.57, p < 0.001 and without r = 0.79, p < 0.001) and inversely with C3 (with r = -0.44, p = 0.027 and without r = -0.63, p < 0.001) and C4 (with r = -0.47, p = 0.018 and without r = -0.43, p = 0.03). Gal-9 had an area under the curve (AUC) of 0.96 to distinguish SLE cases from control. However, AUC between LN group and non-nephritic SLE was 0.48. On regression, SLEDAI-2 K was the only significant factor associated with serum Gal-9 (p < 0.001).ConclusionIn SLE patients, significantly raised Gal-9 levels and relation with disease activity were detected indicating its clinical relevance as biomarker of disease activity and its potential value in the disease diagnosis. Its value in discriminating LN from non-nephritic SLE is limited.     

Anti-C1q antibody in patients with lupus nephritic flare: 18-month follow-up and a nested case-control study

Objectives: A prospective cohort was conducted to investigate the association of anti-C1q antibody and lupus/lupus nephritis (LN) flare.

Methods: Sixty-nine consecutive patients with systemic lupus erythematosus were enrolled and followed up for 18 months. Anti-C1q was recorded at the first visit and at the time of flare. For patients with flare, age and sex matched SLE patients were considered as the control group (nested case-control study). The predictability of anti-C1q and other laboratory indices for LN flare during the 18-month follow-up was calculated.

Results: Fourteen out of sixty-nine (20%) had lupus flare. Fourteen patients were chosen as controls. Nine cases and three controls had positive anti-C1q at the first visit (p?=?0.0001). Twenty-six (38%) and 43 (62%) patients had positive and negative anti-C1q antibody at the first visit, respectively, of whom 9 (34.5%) and 3 (7%) patients developed LN flare in the next 18 months (p?=?0.003). Anti-C1q and 24-hour urine protein were found as the main predictors of LN flare. The positive and negative predictive values of anti-C1q for LN flare were 35% and 93%, respectively. Predictive values of positive anti-C1q/low C3 together were 60% and 96%, respectively.

Conclusion: The combination of positive anti-C1q/low C3 had the highest reasonable predictive values for LN flare.     

Renal arterial resistive index as a noninvasive biomarker of disease activity in lupus nephritis patients

Aim of the workTo evaluate the renal resistive index (RI) in lupus nephritis (LN) patients and to study its association with clinical features, laboratory investigations and LN pathological classes in systemic lupus erythematosus (SLE) patients.Patients and methodsThe study included 45 SLE patients and 25 matched controls. SLE disease activity index (SLEDAI) was assessed and patients subdivided into LN (renal SLEDAI ≥ 4) and no-renal activity (NRA) (renal SLEDAI = 0). Ultrasound Doppler renal examination was done to measure RI. Renal biopsies were performed in 30 LN patients.ResultsThe mean age of patients was 29.8 ± 10.1 years and disease duration 4.3 ± 3.9 years. They were 40 females and 5 males (F:M 8:1). Their SLEDAI was 10.9 ± 8.2 and renal SLEDAI was 5.2 ± 5.1. They were 30 with LN and 15 NRA SLE patients. Renal RI was significantly higher in LN patients compared to NRA SLE patients and controls (0.61 ± 0.04 vs. 0.55 ± 0.01 vs. 0.55 ± 0.02; p < 0.0001). RI significantly correlated with anti-double stranded deoxyribonucleic acid (anti-dsDNA) positivity (r = 0.33, p = 0.03), 24-hour proteins in urine (r = 0.38, p = 0.01) and negatively with creatinine clearance (r = -0.33, p = 0.03). Renal RI significantly correlated with pathological classes of renal biopsy (r = 0.65, p < 0.0001). At renal RI cut-off value 0.57 renal RI can detect renal activity with sensitivity of 83.3%, specificity of 82.5%, p < 0.0001. Renal RI ≥ 0.57 had higher activity index score compared to those with normal RI (5.7 ± 0.6 vs. 9 ± 3.3, p = 0.04). Conclusion: Renal RI was significantly increased in LN compared to NRA patients and was associated with laboratory parameters and pathological classes.     

Use of belimumab throughout pregnancy to treat active systemic lupus erythematosus—A case report

Background

Pregnancy can lead to flares in systemic lupus erythematosus (SLE), and the presence of SLE in pregnancy could lead to a poor outcome for the mother and the fetus.

Objective

To describe a patient whose active SLE (including lupus nephritis) was managed with the use of belimumab throughout pregnancy.

Methods

A case report and review of relevant literature is presented.

Results

A 38-year-old Caucasian woman with SLE was seen for advice regarding planning a pregnancy and management of her active lupus (cutaneous lupus, angioedema, lupus nephritis, leukopenia, and anti-phospholipid antibody syndrome) that could only be controlled by mycophenolate, a drug contraindicated in pregnancy. Azathioprine, hydroxychloroquine, rituximab, and moderate doses of prednisone were either unable to control her disease or led to unacceptable toxicity. After detailed discussions, she was treated with belimumab, which controlled her SLE and allowed withdrawal of mycophenolate. Belimumab was continued throughout the pregnancy, leading to well-controlled SLE and uneventful course, albeit with the presence of mild Ebstein?s anomaly in the baby.

Conclusion

To our knowledge, this is the first case report of belimumab use throughout pregnancy for controlling active SLE. Data from the belimumab pregnancy registry would be useful to confirm our findings and to further assess safety of this agent for use in pregnancy.     

Efficacy and safety of tacrolimus for induction therapy in patients with active lupus nephritis

Abstract

After the completion of a double-blind placebo-controlled trial, tacrolimus (TAC) was approved for the treatment of lupus nephritis (LN) in Japan. However, the approved maximal dose, 3 mg/day, is almost half the dose used for induction therapy outside Japan. In this study, we retrospectively evaluated the efficacy and safety of low-dose TAC (≤3 mg/day) for induction therapy in 13 adult patients (2 men and 11 women) with active LN. Eight patients were treated for LN flares. Twelve patients underwent renal biopsies: 8 with class IV, 2 with class III + V, 1 with class IV + V, and 1 with class V renal histology, according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification. The mean initial doses of prednisone and TAC were 34.6 ± 14.5 and 2.7 ± 0.6 mg/day, respectively. All the patients achieved a complete remission (CR) at 7.7 ± 6.7 months (mean ± SD) after the last administration of TAC, except for 2 patients who discontinued TAC treatment; 1 because of worsening systemic lupus erythematosus and 1 because of hypertension. Two patients experienced a flare-up after achieving CR. The mean blood TAC concentration 12 h after the last administration (C12) was significantly lower among the patients with flare-ups than among those with a sustained CR (1.5 ± 1.5 vs. 5.1 ± 1.9 ng/mL, P = 0.034). These data showed that low-dose TAC was effective for induction therapy in patients with active LN, although a lower TAC concentration may be associated with a poor outcome.     

The effectiveness and safety of mycophenolate mofetil in lupus nephritis

The purpose of this study is to evaluate the effectiveness and safety of mycophenolate mofetil (MMF) for inducing and/or maintaining remission of lupus nephritis (LN). This is a retrospective study of 25 LN patients consecutively treated with MMF. The primary outcome was complete renal remission (CR) defined by urine protein/creatinine ratio ≤0.5 g/g and inactive urine sediment and serum creatinine within <15% above baseline. For induction, 21 episodes of active, moderate to severe LN were treated with MMF. Twelve cases (57%) achieved CR over a median of 8.5 months. Of 13 patients who had LN for <12 months and took ≥2 g/day of MMF, 11 achieved CR, compared to one out of the eight patients who did not meet both criteria (p = 0.0022). For maintenance therapy, 15 patients received MMF for a median of 20 months (range 5–55 months). Two patients (13%) experienced renal flares while taking MMF. Most adverse events were transient and did not require change in therapy. This study suggests that MMF is an effective treatment for both induction and maintenance of remission of moderate to severe LN with a relatively favorable safety profile. Early treatment and a dose ≥2 g/day are essential for optimal outcome. CR may take >6 months.     

Sequential evaluation of clinical and laboratory changes amongst children suffering from lupus nephritis during intermittent intravenous cyclophosphamide therapy

We prospectively evaluated the efficacy and safety of a 24-month course of intermittent intravenous cyclophosphamide (IC) therapy for children suffering from lupus nephritis soon after the diagnosis of systemic lupus erythematosus (SLE) was made. Eight children with severe lupus nephritis were treated with IC monthly for 6 months and then every 3 months, totaling 24 months. The repeated measurements of sequential serological parameters of lupus nephritis, monitored over the course of the study, were analyzed statistically. The urine creatinine clearance rate (Ccr), the 24-h urine protein excretion, and the serum creatinine level significantly improved (p<0.05) after 6, 9 and 12 months of treatment, respectively. The serum C3, C4, albumin, and triglyceride level, the hemoglobin level, and the erythrocyte sedimentation rate significantly improved (p<0.05) 1 month after treatment. The IC appeared to elicit a significant effect (p<0.05) upon the mean leukocyte and neutrophil counts but had no effect (p>0.05) on the platelet count. The lymphocyte count decreased (p<0.05) during the first six monthly IC, whereas the lymphocyte count returned to the baseline level during the quarterly IC events. From a total of 96 IC doses given to those SLE patients, severe myelotoxicity occurred in one patient when lymphocyte count declined to 98 mm^-3; however, no sign of clinical infection was observed. The daily steroid dosage can be tapered rapidly, and the SLE-associated hyperlipidemia resolved parallel to the resolution of the acute lupus nephritis. We concluded that the efficacy of a 24-month IC course for a child suffering from lupus nephritis is significant.     

Serum beta2-microglobulin level in systemic lupus erythematosus patients: Relation to disease activity

Aim of the workTo assess the level of β2-microglubulin (β2M) in systemic lupus erythematosus (SLE) patients and its association with disease activity and other disease parameters.Patients and methods40 SLE patients and 22 matched controls were studied. Serum β2M was assessed using enzyme-linked immunosorbent assay (ELISA). SLE Disease Activity Index (SLEDAI) and the damage index were assessed.ResultsThe patients were 36 females and 4 males (F:M 9:1) with a mean age of 28.5 ± 7.9 years and disease duration of 6.7 ± 3.3 years. The SLEDAI was 9.3 ± 5.2 and the damage index 1.83 ± 1.84. The mean level of serum β2M was significantly higher in SLE patients (6.42 ± 2.46 mg/L) than control (2.47 ± 0.4 mg/L) (p < 0.01).The serum level of β2M was significantly higher in patients with nephritis (n = 22) (7.45 ± 2.47 mg/L) compared to those without (n = 18) (5.17 ± 1.82 mg/L)(p = 0.002), And it was similar in those with and without arthritis (7.24 ± 2.3 mg/L vs 5.88 ± 2.4 mg/L (p0.07).The β2M significantly correlated with disease activity (r = 0.86, p 0.001), serum creatinine (r = 0.52, p > 0.001), urea (r = 0.63, p < 0.001), 24 h urinary protein (r = 0.56, p < 0.001), hematuria (r = 0.4, p < 0.01) and pyuria (r = 0.41; p < 0.01), ESR (r = 0.48; p < 0.01) and inversely with hemoglobin level (r = ?0.34; p = 0.03). No significant correlation was found with C-reactive protein or with disease damage. Serum (β2M) significantly predicted nephritis and disease activity (sensitivity 63.6 %, specificity 77.8 %; p < 0.001 and 95 %CI: 0.25–0.41; p < 0.001 respectively).ConclusionSerum β2M is significantly associated with disease activity and lupus nephritis, suggesting that serum β2M may serve as a potential biomarker to monitor the disease activity and predicting lupus nephritis. However its association to disease severity needs further longitudinal studies.