Time of initial appearance of renal symptoms in the course of systemic lupus erythematosus as a prognostic factor for lupus nephritis

The prognosis of lupus nephritis (LN) was studied retrospectively in two LN categories, LN manifested initially at systemic lupus erythematosus (SLE) onset (I-LN) and LN of delayed manifestation after SLE onset (D-LN), based on a chart review (C) of 154 SLE (85 LN) patients with a mean observation of 20.8 ± 9.3 years and a questionnaire study (Q) of 125 LN patients outside our hospital with mean observation of 17.6 ± 9.2 years. In both study groups, half of I-LN patients were relapse-free by Kaplan–Meier analysis after initial therapy, and the relapsed I-LN patients responded to retherapy at higher 5-year relapse-free rates than those of patients receiving initial therapies for D-LN. At last observation, a higher frequency of prolonged remission was shown in I-LN compared with D-LN patients (C: 22/31, 71% versus 14/49, 29%, P < 0.01; Q: 65/89, 73% versus 11/33, 33% P < 0.01) and also a higher frequency of irreversible renal damage in D-LN compared with I-LN patients (C: 25/49, 51% versus 2/31, 6%, P < 0.001; Q: 14/33, 42% versus 6/89, 7%, P < 0.001), although class IV pathology was common in patients (C) in both LN categories. Onset time of lupus nephritis in the course of SLE may affect renal prognosis.     

Lupus nephritis among 624 cases of systemic lupus erythematosus in Riyadh, Saudi Arabia

The aim of this article is to study the prevalence, clinicolaboratory features, WHO histological types, therapy and renal outcome of lupus nephritis (LN) in Saudi Arabia. During the 27-year-period (1980–2006), 299 (47.9%) cases of LN were identified among the 624 cases of systemic lupus erythematosus (SLE) follow-up at King Khalid University Hospital, Riyadh. The female:male ratio in LN was 8.3:1, with a mean age of 32 years and a mean age of onset of 23 years. The WHO renal histological types were; Class I (1%), Class II (18.1%), Class III (10%), Class IV (37.1%), Class V (11.7%), and Class VI (2.7%). Azathioprine was given to 43.1% and pulse cyclophosphamide to 65.6% in combination with other drugs. Remission was seen in 226 (75.6%) patients, renal flares in 14 (4.7%), end stage renal disease (ESRD) in 27 (9.0%), death in 18 (6.0%), and 14 (4.7%) lost follow-up. The 5- and 10-year patient survival rates in our whole LN cohort by Kaplan–Meier analysis were 96% and 95%, respectively. The survival did not differ significantly in different LN classes nor did it differ significantly during the three periods of presentation (1980–1990, 1991–2000, and 2001–2006; P > 0.05). The risk factors for poor survival were found to be older age at onset (>50-years age; P = 0.034), ESRD (P = 0.000), and low C3 (P = 0.022). The risk factors for progression to ESRD were older age at onset (>50-years age; P = 0.037), hypertension (P = 0.009), elevated serum creatinine (P = 0.000), and proliferative LN (Classes III, IV; P = 0.013, P = 0.039). Different treatment modalities did not have significant effect on survival in the whole LN cohort (P = >0.05). However, pulse cyclophosphamide favored remission in Classes II, III, IV, and V (P = 0.023). The main causes of death were renal failure (50%) and infections (44.4%).     

狼疮肾炎危险因素分析

目的探讨狼疮肾炎(LN)病变的危险因素。方法对79例LN患者进行回顾性分析,并与同期住院的91例无肾炎病变的SLE患者作对照。所有实验室检查均采用标准方法。结果LN患者占同期住院患者的40.3%。与无肾炎病变的SLE患者作对照,其发病年龄明显小于对照组(P<0.001),两组性别和病程方面差异无显著性(P>0.05)。实验室指标对比显示,抗dsDNA抗体、抗Sm抗体、抗心磷脂抗体(aCL)和抗中性粒细胞胞质抗体(ANCA)在LN组有较高的阳性率,与对照组相比差异有显著性(P<0.05)。结论发病年龄小,存在抗dsDNA抗体、抗Sm抗体、aCL和ANCA阳性是SLE易并发肾炎的危险因素。     

Anti-dsDNA,anti-Sm antibodies,and the lupus anticoagulant: significant factors associated with lupus nephritis

BACKGROUND: Lupus nephritis (LN) is a common manifestation in patients with systemic lupus erythematosus (SLE). Autoantibodies and ethnicity have been associated with LN, but the results are controversial. OBJECTIVE: To study the immunological and demographic factors associated with the development of LN. PATIENTS AND METHODS: A retrospective case-control study of 127 patients with biopsy-proven LN, and 206 randomly selected patients with SLE without nephritis as controls was designed. All patients had attended our lupus unit during the past 12 years. Standard methods were used for laboratory testing. RESULTS: Patients with LN were significantly younger than the controls at the time of SLE diagnosis (mean (SD) 25.6 (8.8) years v 33.7 (12.5) years; p<0.0001). The proportion of patients of black ethnic origin was significantly higher in the group with nephritis (p=0.02). There were no differences in sex distribution or duration of follow up. A higher proportion of anti-dsDNA, anti-RNP, anti-Sm, and lupus anticoagulant (LA) was seen in the group with nephritis (p=0.002; p=0.005; p=0.0001; p=0.01, respectively). In univariate, but not in multivariate, analysis male sex and absence of anti-dsDNA were associated with earlier onset of renal disease (p=0.03; p=0.008). In multivariate analysis the only factors associated with nephritis were younger age at diagnosis of SLE, black race, presence of anti-dsDNA, anti-Sm, and LA. No demographic or immunological associations were seen with WHO histological classes. CONCLUSIONS: Young, black patients with anti-dsDNA, anti-Sm antibodies, and positive LA, appear to have a higher risk of renal involvement. These patients should be carefully monitored for the development of LN.     

Anti-C1q antibodies are associated with systemic lupus erythematosus disease activity and lupus nephritis in northeast of China

This study aimed to investigate the associations of anti-C1q antibodies with systemic lupus erythematosus (SLE) disease activity and lupus nephritis (LN) in northeast of China. Ninety patients with SLE, 37 patients with other autoimmune diseases, and 40 healthy donors in northeast of China were enrolled. Serum anti-C1q antibodies were measured by ELISA with 20 RU/ml as the threshold of positive results. The prevalence and levels of anti-C1q antibodies in SLE group (50%, 20.54 ± 34.67 RU/ml) were significantly higher than those in autoimmune disease and healthy control groups (P < 0.05), yet no significant difference between LN patients and non-LN lupus patients (57.14% vs 41.46%, P > 0.05; 25.92 ± 39.94 vs 13.07 ± 27.39 RU/ml, P > 0.05). Anti-C1q antibody levels were positively correlated with levels of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores, anti-dsDNA, and anti-cardiolipin and negatively correlated with serum C3 and C4 (P < 0.05). The prevalence of anti-Sm and anti-nucleosome increased in anti-C1q-positive lupus patients (P < 0.05). Compared with anti-C1q-negative lupus patients, patients with 20–40 RU/ml anti-C1q antibodies had comparable disease activity (P > 0.05); patients with 40–80 RU/ml anti-C1q antibodies had significantly lower levels of serum complement (P < 0.05); patients with above 80 RU/ml anti-C1q antibodies had much more severe hypocomplementemia, increased SLEDAI scores, and higher incidence of hematuria and proteinuria (P < 0.05). Furthermore, the specificity and positive predictive value of 80 RU/ml anti-C1q antibodies for LN was 97.56% and 87.50%, respectively. In conclusion, anti-C1q antibodies are associated with SLE and LN disease activity, and the contribution hinges on the titers. Moreover, high-level anti-C1q antibodies are valuable for diagnosing LN.     

Venous thromboembolism in southern Chinese patients with systemic lupus erythematosus

The objective is to study the annual incidence and standardized incidence ratio (SIR) of venous thromboembolism (VTE) in a cohort of Chinese patients with systemic lupus erythematosus (SLE). VTE events of SLE patients occurring between 1999 and 2008 were identified from our database, and the annual incidence of VTE was calculated according to the cohort size. SIRs were estimated by the ratios of the incidence of VTE in SLE to the general population. In 2008, 516 SLE patients were in our cohort. The mean age of SLE onset was 32.2 ± 14 years and the duration of SLE was 9.3 ± 8.8 years. Fifty-seven percent of the patients had disease duration of ≥5 years. Between 1999 and 2008, 18 episodes of VTE occurred in 14 patients. The incidence of VTE did not show significant fluctuation and the mean annual incidence was 4.2/1,000 patient–year. The reported VTE events were: popliteal vein thrombosis (56%), pulmonary embolism (22%), renal vein, retinal vein, subclavian vein and dural sinus thrombosis (5.6% each). The cumulative risks of VTE since SLE diagnosis were 2.8% and 3.7% at 5 and 10 years, respectively. Compared to the general population, the mean SIR of VTE in SLE patients within this period was 11.9 (7.31–19.6; p < 0.001). The SIR of VTE was highest in patients under the age of 30 years. The presence of the antiphospholipid antibody was independently associated with VTE (HR 4.36 [1.67–11.4]; p = 0.003). Although venous thrombosis is uncommon in Chinese, Chinese patients with SLE are 12 times more prone to VTE than the general population.     

Anti-nucleosome antibodies: A potential surrogate marker for renal affection in lupus patients with insignificant proteinuria

BackgroundLupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus (SLE). Clinical renal involvement is present in about two-thirds of lupus patients and more patients would have morphologic evidence of renal disease without clinical manifestations.Aim of the workTo investigate serum anti-nucleosome antibodies role as a biomarker for renal affection in lupus patients with insignificant proteinuria.Patients and methodsTwenty-four lupus patients with proteinuria <500 mg/d (group-A), 30 patients with established lupus nephritis (group-B) and 15 controls were included. Systemic lupus erythematosis disease activity index (SLEDAI), anti-nucleosome, anti-dsDNA antibodies and renal biopsy were assessed in all patients.ResultsSerum anti-nucleosome antibodies were significantly higher in all lupus patients than control (P < 0.001) and showed significant positive correlation with SLEDAI score. SLE patients with positive anti-dsDNA antibody had more active disease by SLEDAI and higher levels of anti-nucleosome antibodies than those with negative anti-dsDNA antibodies. In both studied groups, serum anti-nucleosome antibodies were significantly higher in patients with class II LN than the control and in class III LN than in class II LN (P < 0.001). Yet, in both groups, anti-nucleosome was not useful in differentiating active from chronic renal affection.ConclusionSerum levels of anti-nucleosome antibodies are associated with active lupus disease and correlate with the degree of renal affection. In patients with insignificant proteinuria, serum levels of anti-nucleosome antibodies were elevated and were related to the degree of renal affection. Anti-nucleosome antibodies may be used as a surrogate marker for early renal affection in lupus patients with insignificant proteinuria.     

Renal involvement in childhood-onset systemic lupus erythematosus in Egypt

Lupus nephritis has been described as the most serious complication of systemic lupus erythematosus (SLE) and the strongest predictor of poor outcome. While the incidence of childhood SLE is relatively low, renal involvement appears to be more common and more severe in childhood SLE. This study aims to characterize the features and outcome of renal involvement in childhood-onset SLE based on a study of 100 Egyptian patients (mean age at diagnosis 10.1 years, range 2–17 years). Initial data regarding disease manifestations and biopsy findings were reviewed. Disease activity was assessed using SLEDAI scores. Follow-up data (mean duration 6 years) were noted regarding specific treatment, response, complications and renal survival. Initial renal involvement was present in 78 patients, including 66 with hypertension and 23 with renal impairment. Pathologically, class IV nephropathy was found in 18 patients, class V in 9 and low-grade lesions (class II–III) in 49. Twenty patients required follow-up biopsy, and all transformations were observed. SLEDAI scores significantly decreased from initial (mean ± SD) of 21.4 ± 7.3 to 13.4 ± 7.8, in association with response to therapy (P < 0.0001). Poor response was associated with initial hypertension and renal impairment but not with initial SLEDAI score or pathological class. The projected renal survival was 82.4 and 64.7% 5 and 10 years from diagnosis. Early renal involvement in childhood SLE is common, serious and requires proper evaluation and management.     

Association of serum uric acid with lupus nephritis in systemic lupus erythematosus

The aim of the present study is to assess the association of elevated serum uric acid (UA) with lupus nephritis (LN) in systemic lupus erythematosus (SLE) patients. A total of 130 SLE patients were recruited, of whom 73 patients developed LN. Blood samples were obtained for determination of uric acid, complement 3 (C3), C-reactive protein (CRP) and some autoantibodies including anti-double-stranded DNA, -Smith, -SSA, -SSB, -U1RNP, SCL-70, and -Jo-1 antibodies. Correlations of UA with LN were assessed. UA was an independent risk factor for LN [odds ratio (95% CI): 1.01 (1.005–1.014); P = 0.0000]. The best cut-off value for UA using the ROC curve was 330 μmol/L (sensitivity 78.1% and specificity 75.4%) and the area under the ROC curve was 0.803 ± 0.039 (95% CI: 0.727–0.878, P = 0.000). Spearman’s correlation coefficient analysis showed negative association of UA with C3 in SLE patients with LN (r = −0.356, P = 0.002), but no association in those without LN. No correlations were found between UA and age, SLEDAI, CRP, IgG, IgM or IgA. Furthermore, analysis of covariance demonstrated that anti-Sm (β = −0.218, P = 0.004) and -U1RNP (β = 0.177, P = 0.008) autoantibodies were independent determinants of serum UA. The UA level is independently associated with the development of LN in SLE patients.     

Membranous (Class V) Renal Disease in Systemic Lupus Erythematosus May Be More Common Than Previously Reported: Results of a 6-Year Retrospective Analysis

BackgroundThe actual incidence and prevalence of the various histological classes (based on World Health Organization classification) of lupus nephritis (LN) are not known but seem to vary with sex, age, and ethnicity. We have analyzed renal biopsies in patients with systemic lupus erythematosus (SLE) at our center, and hereby report our experience.MethodsAll renal biopsies performed at the University of Mississippi between January 1999 and December 2004 in patients with SLE were retrospectively analyzed. Results were validated by a detailed review of renal biopsy reports and additional records were reviewed for data specific to LN disease activity.ResultsThere were 92 renal biopsies performed in patients with SLE during a 6-year period. These included 84 African Americans (72 women and 12 men), 5 whites (4 women and 1 man), and 3 unknown race (1 F, 2 M) subjects. The prevalence of LN classes in our cohort was as follows: class I (0%), class II (9.8%), class III (8.7%), class IV (36.9%), class V (40.2%), and class VI (4.3%). Prevalence of class V LN among males was high at 40%.ConclusionsIn contrast to previous literature, isolated membranous lupus nephritis (MLN) was much more prevalent in this series—40% versus 14%. Also, no sex difference in the prevalence of MLN was seen. This biopsy cohort suggests that MLN/ class V disease may be more common than previously reported especially in African American population.     

Male gender results in more severe lupus nephritis

Gender may produce different characteristics in the manifestation of systemic lupus erythematosus (SLE). The present study investigated the influence of gender on clinical, laboratory, autoantibodies and histopathological classes of lupus nephritis (LN). As much as 81 patients diagnosed with SLE (ACR criteria) and active nephritis, who underwent renal biopsy between 1999 and 2004, and who had frozen serum samples and clinical data available from the time of biopsy, were selected for this study. The presence of anti-P and antichromatin antibodies was measured using ELISA, and anti-dsDNA was measured using indirect immunofluorescence. All of the renal biopsies were reviewed in a blinded manner by the same expert renal pathologist. The charts were extensively reviewed for demographic and renal features obtained at the time of the biopsy. Of the 81 patients (13.6%), 11 were male SLE patients. Both male and female lupus patients were of similar age and race, and had similar durations of lupus and renal disease. The female patients had more cutaneous (95.7 vs. 45.5%, P = 0.0001) and haematological (52.9 vs. 18.2%, P = 0.04) involvements than the male SLE patients. In addition, the articular data, central nervous system analyses, serositis findings and SLEDAI scores were similar in both experimental groups. Positivity for anti-dsDNA, anti-ribosomal P and antichromatin did not differ between the two groups, and both groups showed similarly low C3 or C4 serum levels. Our analysis indicated that no histopathological class of LN was predominant in both males and females. Interestingly, the serum creatinine levels were higher in the male SLE patients compared to the female SLE group (3.16 ± 2.49 vs. 1.99 ± 1.54 mg/dL, P = 0.03), with an increased frequency of high creatinine (81.8 vs. 47.1%, P = 0.04) as well as renal activity index (7.6 ± 3.5 vs. 4.8 ± 3.5, P = 0.02). In addition, whilst the mean levels of proteinuria, cylindruria and serum albumin were markedly altered, they were comparable between both lupus men and women. Moreover, the frequencies of dialysis, renal transplantation and death were similar between the two groups. These data suggest that male patients had a more severe LN compared to women diagnosed with this renal abnormality.     

Late onset systemic lupus erythematosus in southern Chinese

OBJECTIVE—Systemic lupus erythematosus (SLE) is a multisystem disorder that predominately affects women of the reproductive age. Onset of the disease beyond the age of 50 years is unusual. This study was undertaken to compare retrospectively the clinical and laboratory features between early and late onset (onset of disease beyond the age of 50 years) SLE patients in a Chinese population.
METHODS—Case records of all SLE patients who attended our rheumatology clinics between 1971 and 1997 were reviewed. Patients with a disease onset beyond the age of 50 years were identified. One hundred consecutive SLE patients who had their disease onset before the age of 50 were recruited as controls. The presenting clinical features, autoantibody profile, number of major organs involved, number of major relapses, and the use of cytotoxic agents in the two groups of patients were obtained and compared.
RESULTS—25 patients with late onset SLE were identified. All the female patients in the late onset group were postmenopausal. The female to male ratio was 3.2 to 1, compared with 13.3 to 1 in the control group (p<0.02). Both groups had a comparable duration of disease. There were no significant differences in the presenting features between the two groups except for a lower prevalence of malar rash (24% v 86%, p<0.0001) and a higher prevalence of rheumatoid factor (32% v 1%, p<0.0001) in the late onset patients. On subsequent visits, the late onset group had a lower prevalence of lupus nephritis (4% v 51%, p<0.001), fewer major organs involved (mean number of major organs involved; 0.3 v 0.9, p<0.02), fewer major relapses (mean number of major relapses/patient; 0.08 v 0.47, p<0.002, number of major relapses/patient year; 0.009 v 0.12, p<0.001), and required fewer cytotoxic agents for disease control (percentage of patients on cytotoxic agents; 32% v 79%, p<0.002).
CONCLUSION—Late onset SLE in Chinese tends to run a more benign course with fewer major organ involvement and fewer major relapses. The significantly higher incidence of male sex in late onset SLE and the milder disease course in the postmenopausal female patients suggest that oestrogen status may influence disease activity.

Keywords: systemic lupus erythematosus; southern Chinese; Asians     

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.     

Plasma ficolin levels and risk of nephritis in Danish patients with systemic lupus erythematosus

Given the scavenging properties of ficolins, we hypothesized that variation in the plasma concentrations of the three ficolins may be associated with development of lupus nephritis (LN), type of LN, end-stage renal disease (ESRD), and/or mortality among patients with systemic lupus erythematosus (SLE). SLE patients attending a Danish tertiary rheumatology referral center were included. Plasma concentrations of ficolin-1, ficolin-2, and ficolin-3 were determined and dichotomized by the median into high and low. LN was defined by clinical criteria; type of LN by renal biopsy; ESRD follow-up time was defined as time from onset of LN to the development of ESRD or censoring at the end of follow-up. The study included 112 SLE patients with median disease duration of 8 years of which 53 (47%) had LN at the time of inclusion. During a median follow-up of 10 years, five patients developed ESRD. Sixteen patients died. Odds ratios (ORs) of LN were 1.2 (95% CI: 0.6–2.7), 4.1 (95% CI: 1.7–9.7), and 0.9 (95% CI: 0.4–2.0) for patients with low ficolin-1, ficolin-2, and ficolin-3 plasma levels, respectively. The distribution of histological classes differed between patients with high and low plasma levels of ficolin-1 (p = 0.009). Patients with high ficolin-1 plasma levels had an increased risk of ESRD. There was no association between the levels of the analyzed plasma ficolins and mortality. Low plasma ficolin-2 levels were associated with an increased risk of having LN. High plasma levels of ficolin-1 were associated with the histological subtype of LN and development of ESRD.     

Neutrophil to lymphocyte and platelet to lymphocyte ratios in systemic lupus erythematosus: Relation with disease activity and lupus nephritis

ObjectivesTo investigate the role of neutrophil to lymphocyte ratio (NLR) and platelet to lymphocyte ratio (PLR) as activity markers in systemic lupus erythematosus (SLE) without nephritis and lupus nephritis (LN) patients.Patients and methodsThis study included 60 SLE patients with LN, 60 SLE patients without renal involvement and 30 healthy controls. We analyzed correlations between NLR and PLR and both disease activity and renal affection.ResultsThe NLR of SLE patients was much higher than those of the controls. Both ratios showed significantly increased values in SLE patients with active disease. NLR and PLR were positively correlated with SLEDAI, ESR, and CRP and negatively correlated with C4. SLE patients with LN had higher levels of NLR than those without nephritis. NLR showed positive correlations with BUN, serum urea, serum creatinine and 24 h urinary protein. We found NLR to be related to anti-ds-DNA level and renal biopsy classes. While PLR was related only to anti ds-DNA. The best NLR to predict SLE active disease was 2.2 and the best PLR cut-off value was 132.9.ConclusionNLR and PLR are useful inflammatory markers to evaluate disease activity in SLE patients. Also, NLR could reflect renal involvement in SLE patients and is associated with the different classes of its histological staging.     

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.     

Treatment response and progression to end stage renal disease in adolescents and young adults with lupus nephritis: A follow up study in an Egyptian cohort

BackgroundLupus nephritis (LN) badly affects the outcome in adolescents and young adults with systemic lupus erythematosus (SLE). Many have renal disease at onset and the significance of remission and relapse in adolescents and young adults is poorly evaluated.Aim of workTo outline the clinical and laboratory characteristics of treatment resistance, renal relapse and progression to end-stage renal disease (ESRD) in adolescents and young adults with LN.Patients and methodsEighty-five biopsy-proven LN patients were examined; SLE disease activity and renal damage were evaluated at baseline and followed up at 6 and 12 months. Laboratory and immunology profiles were assessed. Patients were evaluated for predictors of treatment response, renal flares, and renal survival.ResultsThe patients mean age was 15.12 ± 4.53 years. Female/male ratio was 10.5:1. 12.9% had treatment resistance, 87.1% achieved remission: complete (CR 31.8%) and partial (PR 55.2%) within 1st year. 27 (31.8%) developed a relapse within the 1st year (9 after CR and 18 after PR). Nephrotic range proteinuria persisted in 24 (28.2%) patients (13 PR and the 11 non-responders). Baseline hypertension (p = 0.034), persistent nephrotic range proteinuria (<0.001) and PR (p < 0.001) were predictive for renal flares. Treatment resistance (p = 0.021), disease relapse (p < 0.001), persistent nephrotic range proteinuria (p < 0.001) were predictors of ESRD, especially in males (p = 0.035). Autoimmune profile and histopathology class showed insignificant differences among groups.ConclusionPrevention and aggressive management of hypertension, proteinuria and renal flares is expected to prevent progression to ESRD in lupus nephritis in adolescent and young adult SLE patients.     

Outcome of lupus renal disease in the past two decades: a report from China

Objective: To evaluate the differences in the renal survival of lupus nephritis (LN) diagnosed either during 1985–1994 or 1995–2004 and to analyse the possible causes. Methods: Fifty‐two patients with biopsy‐confirmed LN were followed up between 1985 and 1994 and 130 patients were followed up between 1995 and 2004. Renal survival was studied with Kaplan‐Meier analysis and the log rank test. Status at diagnosis and treatment schedules were also analysed. Results: Renal survival was significantly better in the patients who were diagnosed between 1995 and 2004 (P = 0.0233). The mean time from initiation until first diagnosis of SLE, from the initiation of SLE until referral to our centre, and from first detection of proteinuria until kidney biopsy was significantly shorter in the later decade (P < 0.05). In the decade from 1995 to 2004 there was significantly lower rates of early renal dysfunction and fewer histological signs of chronicity at the time of diagnosis of LN than in the decade from 1985 to 1994. In treatment schedules, more patients received intravenous cyclophosphamide (CTX) in a bolus in the later decade group (P = 0.014). Besides CTX, some new immunosuppressive agents such as cyclosporin A or mycophenolate mofetil were received by more patients in the decade from 1995 to 2004. Conclusions: The outcomes for patients with LN was significantly better between 1995 and 2004 than between 1985 and 1994. The result is maybe attributable to earlier diagnosis, earlier treatment, intravenous bolus CTX and more immunosuppressive agents from which to select.     

Systemic lupus erythematosus in Egyptian children

The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 ± 2.7 years (range 2–16). The mean disease duration was 6.47 ± 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was ≤5 years (nine patients) had significantly more common hematological affection (P value = 0.0005). The characteristics of SLE in Egyptian patients show some similarities to other series of Middle Eastern countries, but with a lower female to male ratio. Disease onset below 5 years is extremely rare (4.35%), commonly presenting with hematological manifestations. The kidney was the commonest major internal organ involved, and also an important cause of death.