Lupus nephritis in childhood: a review of 53 patients followed at a single center

We retrospectively evaluated the clinical and histopathological features, treatment modalities, and outcome of 53 children and adolescents with biopsy-proven lupus nephritis (LN), followed between September 1983 and September 2001. The mean age (±SD) at the time of diagnosis of systemic lupus erythematosus (SLE) was 12.9±2.6 years and the mean follow-up from the time of biopsy was 4.8±3.4 years. At the time of biopsy, all 53 patients had proteinuria, 21 (40%) had nephrotic syndrome, and 14 (26%) had impaired renal function. Class IV nephritis, observed in 34 (64%) patients, was the most frequent histopathology on initial renal biopsy. The patients with class IV LN had a significant tendency to develop hypertension (P=0.04) and nephrotic syndrome (P=0.027), and a lower mean glomerular filtration rate (P=0.000). Based on the renal histopathology and clinical presentation, patients were treated with corticosteroids alone or combined with azathioprine or with intravenous cyclophosphamide. Plasmapheresis or cyclosporine was used in 4 and 1 patient, respectively. Follow-up biopsies, performed in 13 patients, showed no change in 6 patients, were progressive in 4, and regressive in 3. On final clinical evaluation, renal disease was in complete or partial remission in 42 of 53 patients (80%), 4 had clinically active disease but with normal renal function, and 7 (13%), all with WHO class IV LN, were classified as having an adverse outcome, i.e., either preterminal (2) or terminal (4) renal failure or death (1). Five-year kidney and patient survival rates from the time of biopsy to the endpoints of terminal renal failure or death were 88.6% and 98.1%, respectively, in the whole group, and 82.4% and 97.1%, respectively, in the WHO class IV group. Nephrotic syndrome and class IV nephritis at initial biopsy were the only parameters significantly associated with adverse outcome in our study group. There was no association with gender, age, hypertension, impaired renal function, anemia, increased morphological index scores, and treatment modalities. We conclude that clinical and histopathological features of LN and treatment regimens in our study do not differ markedly from those in most pediatric series. However, the 5-year kidney and patient survival rates are among the best reported in recent pediatric series. The prognosis of LN is primarily dependent on the histopathological lesions.In memory of Professor Miodrag Sindji (1927–2000), nephropathologist, our teacher and friend     

Primary focal segmental glomerulosclerosis in children: prognostic factors

To study the clinical course and the predictors of outcome in children and adolescents, 39 patients with nephrotic syndrome and primary focal segmental glomerulosclerosis (FSGS) were followed for a mean of 84.6 months. Thirty-six patients were treated with prednisone, either alone or in conjunction with cyclophosphamide. The clinical course was one of sustained remission in 4 patients, frequent relapse in 13, persistent non-nephrotic proteinuria in 5, and persistent nephrotic syndrome in 17; 2 patients had stable renal failure and 8 had progressive renal failure, 5 of them evolving to end-stage renal failure (ESRF). Resistance to prednisone was recorded in 76.6% of patients. The use of cyclophosphamide plus prednisone was of benefit in 42.8% of patients; 22.2% of the prednisone-resistant patients achiev-ed remission of the nephrotic syndrome. A Kaplan-Meier analysis revealed a survival rate of 92% after 5 years, 86% after 10 years, and 76% after 15 years. Using both univariate and multivariate analysis, persistent nephrotic syndrome was associated with progression to ESRF and the remission of proteinuria with maintenance of renal function. As the outcome of the nephrotic syndrome in FSGS is significantly improved by remission of proteinuria, it is conceivable that immunosuppressive medication may be used in conjunction with prednisone in patients with steroid resistance. Received: 5 June 2000 / Revised: 16 April 2001 / Accepted: 19 April 2001     

Lupus nephritis: a retrospective review of 78 cases from a single center

Lupus nephritis (LN) is a frequent and serious manifestation of systemic lupus erythematosus. However, the outcome has progressively improved in the last 3 decades and this was due to more efficient and early treatment of LN and comorbid situations. The aim of our study was to analyze our experience and outcome in LN, to evaluate clinicopathologic and clinicolaboratory correlations and to search for risk factors for renal and patient survival. PATIENTS AND METHODS: We conducted a retrospective study of 78 patients with biopsy-proven LN. RESULTS: Acute renal failure and subnephrotic proteinuria with microhematuria occurred each one in 39.7% of the patients, nephrotic syndrome in 16.7% and nephritic syndrome in 3.8%. The mean serum creatinine at presentation was 1.45 +/- 1.03 mg/dl and the creatinine clearance was 68.2 +/- 40.3 ml/min. Class IV LN existed in 71.8%, Class III in 20.5%, Class V in 6.4% and Class VI in 1.3%. The treatment included steroids and cytotoxic agents in 87.5% of the patients with proliferative LN. Hypertension, serum creatinine and acute renal failure at presentation, as well as significant chronicity on renal biopsy, were significantly correlated with the progression to chronic renal failure in our population. Males were more prone to develop renal flares. 3.8% of the patients died, 9% lost their renal function, 26.9% are in remission, 33.3% still have subnephrotic proteinuria and microhematuria, 7.7% have nephrotic syndrome and 19.2% have chronic renal failure. The mean global follow-up was 102 +/- 74 months and 96.2% of the patients survived. The actuarial renal survival was 96.1% in the first year; 89.9% at 5 years; and 83.7% at 10 and 20 years. CONCLUSION: We can say that hypertension, serum creatinine and acute renal failure at the onset and significant chronicity on renal biopsy, proved to be risk factors for chronic renal failure in our study population. Male gender was a risk factor for renal flares. The achieved global outcome can be considered a good result.     

Lupus nephritis in Chinese children — a territory-wide cohort study in Hong Kong

We report a multicenter study of Chinese children in Hong Kong with systemic lupus erythematosus (SLE) nephritis. Children were included if: they fulfilled the ACR criteria, had significant proteinuria or casturia, were Chinese and younger than 19 years and had been diagnosed with SLE between January 1990 and December 2003. Investigators in each center retrieved data on clinical features, biopsy reports, treatment and outcome of these patients. There were 128 patients (eight boys, 120 girls; mean age: 11.9±2.8 years). About 50% presented with multisystem illness and 40% with nephritic/nephrotic symptoms. Negative anti-dsDNA antibodies were found in 6% of the patients. Renal biopsy revealed WHO Class II, III, IV and V nephritis in 13 (10%), 22 (17%), 69 (54%) and 13 (10%) patients, respectively. The clinical severity of the nephritis did not accurately predict renal biopsy findings. The follow-up period ranged from 1 to 16.5 years (mean±SD: 5.76±3.61 years). During the study five patients died (two from lupus flare, one from cardiomyopathy, two from infections). Four patients had endstage renal failure (ESRF) (one died during a lupus flare). All deaths and end-stage renal failure occurred in the Class IV nephritis group. Chronic organ damage was infrequent in the survivors. The actuarial patient survival rates at 5, 10 and 15 years of age were 95.3, 91.8, and 91.8%, respectively. For Class IV nephritis patients, the survival rates without ESRF at 5, 10, and 15 years were 91.5, 82.3 and 76%, respectively. The survival and chronic morbidity rates of the Chinese SLE children in the present study are comparable to those of other published studies.An erratum to this article can be found at     

Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis.

BACKGROUND: Prognostic factors and outcome are incompletely known in childhood mesangiocapillary glomerulonephritis (MCGN). This study aimed to correlate renal outcome with clinical and histopathological variables. METHODS: We conducted a two-centre retrospective analysis of children with MCGN. RESULTS: Fifty-three children presented at a mean age of 8.8 years (range: 13 months-15 years). They were followed for a median of 3.5 years (range: 0-17 years). Histological classification identified 31 type 1, 14 type 2, two type 3 and six undetermined type. Mean renal survival [time to end-stage renal failure (ESRF)] was projected to be 12.2 years [confidence interval (CI): 9.7-14.6 years]. Five and 10 year renal survival was 92% (CI: 88-100%) and 83% (CI: 74-92%), respectively. Those with nephrotic syndrome at presentation had mean renal survival of 8.9 years (CI: 7.1-10.7 years) vs 13.6 years for those without (CI: 10.8-16.5 years) (P = 0.047). The mean estimated glomerular filtration rate (eGFR) at 1 year in those who progressed to ESRF was 52 vs 98 ml/min/1.73 m2 in those who did not (P < 0.001). Chronic damage scored on the first biopsy in 31 children (one centre) was positively associated with adverse renal outcome at 5 years: <20% was associated with 100% and > or =20% with 71% 5-year renal survival (P = 0.006). In 29 children treated with steroid there was a higher proportion (76%) with reduced eGFR at presentation and a significantly higher incidence of nephrotic syndrome (P = 0.002) and hypertension (P = 0.037). There were no significant differences in outcome eGFR, hypertension or proteinuria. CONCLUSIONS: Nephrotic syndrome at presentation and subnormal eGFR at 1 year were adverse features. The finding that structural disease at onset predicted poor renal outcome at 5 years has implications for the design of therapeutic trials. Treatment of MCGN was variable and not evidence-based.     

Lupus nephritis in Chinese children – a territory-wide cohort study in Hong Kong

We report a multicenter study of Chinese children in Hong Kong with systemic lupus erythematosus (SLE) nephritis. Children were included if: they fulfilled the ACR criteria, had significant proteinuria or casturia, were Chinese and younger than 19 years and had been diagnosed with SLE between January 1990 and December 2003. Investigators in each center retrieved data on clinical features, biopsy reports, treatment and outcome of these patients. There were 128 patients (eight boys, 120 girls; mean age: 11.9±2.8 years). About 50% presented with multisystem illness and 40% with nephritic/nephrotic symptoms. Negative anti-dsDNA antibodies were found in 6% of the patients. Renal biopsy revealed WHO Class II, III, IV and V nephritis in 13 (10%), 22 (17%), 69 (54%) and 13 (10%) patients, respectively. The clinical severity of the nephritis did not accurately predict renal biopsy findings. The follow-up period ranged from 1 to 16.5 years (mean±SD: 5.76±3.61 years). During the study five patients died (two from lupus flare, one from cardiomyopathy, two from infections). Four patients had endstage renal failure (ESRF) (one died during a lupus flare). All deaths and end-stage renal failure occurred in the Class IV nephritis group. Chronic organ damage was infrequent in the survivors. The actuarial patient survival rates at 5, 10 and 15 years of age were 95.3, 91.8, and 91.8%, respectively. For Class IV nephritis patients, the survival rates without ESRF at 5, 10, and 15 years were 91.5, 82.3 and 76%, respectively. The survival and chronic morbidity rates of the Chinese SLE children in the present study are comparable to those of other published studies.An erratum to this article can be found at     

Crescentic lupus glomerulonephritis

Crescentic lupus glomerulonephritis (greater than or equal to 50% crescents) occurred in 16% of systemic lupus erythematosus (SLE) patients biopsied over a 32-month period. All had underlying WHO class IV lupus nephritis. These patients more frequently manifested with acute renal failure usually of the non-oliguric type, had heavier proteinuria and lower serum albumin, but were otherwise indistinguishable from non-crescentic WHO class IV lupus nephritis in their other renal and extrarenal manifestations or in their serological findings. Crescentic lupus glomerulonephritis may occur in patients at first presentation with SLE, or develop in patients after prolonged follow-up initially for lupus nephritis of WHO class IV or other classes. Combined methylprednisolone pulse therapy, immunosuppressives, antiplatelet agents with or without anticoagulant produced good initial responses. Prognosis was unfavorable for inadequately treated patients or for patients with persistent nephrotic syndrome and crescents.     

Clinical course of lupus membranous glomerulonephritis in children

Background. Lupus nephritis (LN) is not a common renal disorder of childhood. Few pediatric nephrologists, except for those in a large center, see enough patients with LN to build up a substantial amount of experience. Membranous glomerulonephritis (MGN) associated with systemic lupus erythematosus (SLE), so-called lupus membranous glomerulonephritis (LMGN), is also rare in patients with LN. Methods. In 36 children with SLE, we investigated the clinical course of 4 children (1 boy and 3 girls) who showed MGN. Their mean age at presentation was 7.8 years and the mean follow-up period was 7.1 years. Results. Presenting symptoms were chance hematuria and proteinuria (H and P) in 2 children, edema in 1, and petechiae and thrombocytopenia in 1. The initial biopsy showed MGN in 3 children and diffuse proliferative glomerulonephritis in 1 child, in whom transition to MGN was observed. Two patients, who presented with chance H and P and showed MGN on the initial biopsy, later developed clinical signs of SLE, 3 and 5 years after presentation, respectively. During the course, all patients developed nephrotic syndrome, but at the last follow-up, H and P was detected in 1 child and proteinuria in 1 child. Urinalysis results were normal in 2 children. No patient had nephrotic syndrome or developed renal failure. Conclusions. The clinical course of LMGN in the 4 children seemed to be favorable. Two patients with MGN developed clinical signs of SLE several years after the renal manifestation. Received: May 10, 2000 / Accepted: March 10, 2001     

Clinicopathology of childhood-onset renal systemic lupus erythematosus

AIMS: To determine the clinicolaboratory renal manifestations; glomerular, extra-glomerular histopathologic lesions; renal tubular dysfunction (RTD) frequency and outcome of a short-term renal follow up in Nigerian children with systemic lupus erythematosus (SLE). METHODS: A non-randomized prospective study of consecutive cases of childhood-onset SLE with nephropathy was conducted. Baseline/follow-up clinicolaboratory data were collected. Each patient was followed up for 12 months. RESULTS: Seven of the 11 children studied were girls. The median age at diagnosis was 11.0 years. Median diagnosis time interval (1.9 years) and median time of renal disease onset (1.0 year) were similar. Hypertension, nephrotic syndrome and acute renal failure (ARF) occurred in 45.5%, 54.5% and 63.7% of the patients, respectively. The glomerular lesions were non-proliferative lupus nephritis (LN) in 9.0% (class II LN); focal (class III LN) and diffuse (class IV LN) proliferative LN (PLN) in 27.0% and 64.0%, respectively. Tubulointerstitial nephritis (TIN, 91.0%) and RTD (64.0%) were common. ARF (P = 0.033) and RTD (P = 0.015) were significantly associated with severe TIN. Complete renal remission rate at end-point was 71.4%. Relapse and renal survival rates were 14.3% and 86.0%, respectively. RTD was persistent in 43.0%. CONCLUSION: Renal function disorders, diffuse PLN and extra-glomerular lesions were frequent. Significant association of ARF and RTD with severe TIN in this series suggests the need for early renal tubular function (RTF) assessment in our SLE patients. Deranged RTF may be marker of severe TIN in SLE warranting early confirmatory renal biopsy and aggressive interventional treatment.     

儿童毛细血管内增生性肾小球肾炎332例临床与疗效

目的：了解毛细血管内增生性肾小球肾炎（EnPGN）的临床表现与疗效。方法：将1988年6月～2007年11月住院经肾穿刺确诊的EnPGN332例,结合临床表现、疗效进行分析。结果：急性肾炎型290例（87.4%）、单纯性肾病12例（3.6%）、肾炎性肾病27例（8.1%）,无症状性蛋白尿、迁延性肾炎、直立性蛋白尿各1例（0.9%）。临床治愈314例（94.6%）,好转15例（4.5%）,无效3例（0.9%）;其中急性肾炎型治愈275例（94.8%）,好转13例（4.5%）,无效2例（0.7%）;单纯性肾病12例均治愈;肾炎性肾病治愈24例（88.9%）,好转2例（7.4%）,无效1例（3.7%）;无症状性蛋白尿、迁延性肾炎、直立性蛋白尿各1例（0.9%）均治愈。结论：EnPGN以急性肾炎型多见（87.3%）,肾病综合征11.8%,其他0.9%,疗效以急性肾炎型、单纯性肾病型较好,肾炎性肾病型较差。     

Clinicopathologic correlations in lupus nephritis in Lima, Peru

BACKGROUND: We assessed whether immunohistologic markers for glomerular or tubulointerstitial injury might provide better correlations with ongoing renal function and disease activity as compared with the WHO classification or the NIH activity and chronicity indices in lupus nephritis. METHODS: Thirty-three patients with clinically defined systemic lupus underwent renal biopsy over a 1-year period at Hospital Loayza in Lima, Peru. Biopsy specimens were evaluated for macrophages, proliferating cells, alpha-actin expression, and type IV collagen deposition in both glomeruli and the tubulointerstitium and the results compared with the current WHO and NIH classifications in relation to the clinical presentation. RESULTS: Patients with WHO class IV lupus nephritis were more likely to have lower serum complements, greater proteinuria and hematuria, and worse renal function. An elevated NIH activity index correlated with microhematuria, proteinuria, and impaired renal function, whereas an elevated chronicity index correlated with renal function, hypertension, and microhematuria, but not with proteinuria. The presence of glomerular macrophages correlated with both glomerular alpha-actin expression and type IV collagen deposition, but did not correlate with renal function or proteinuria. In contrast, interstitial macrophages correlated not only with interstitial collagen deposition and myofibroblast accumulation, but also correlated with both renal function and the presence of nephrotic syndrome. CONCLUSIONS: Both the WHO classification and the NIH activity/chronicity indices correlate with clinical manifestations of lupus nephritis. While glomerular macrophage accumulation correlates with mesangial cell activation (alpha-actin expression) and collagen deposition, and interstitial macrophage accumulation correlates with interstitial fibroblast activation and collagen deposition, only interstitial macrophages correlate with renal function. Of particular interest will be future studies to determine whether these markers correlate with the prognosis. Copyright Copyright 1999 S. Karger AG, Basel     

狼疮肾炎的病理与临床分析

目的探讨狼疮性肾炎（LN）的病理类型与临床及实验室检查的关系。方法对34例狼疮性肾炎患者进行肾活检及病理分型,分析各病理类型的临床特点、实验室检查特点、临床活动性及肾脏病理活动性。结果病理类型Ⅳ型最多见（占55．9％）,Ⅳ型LN肾功能不全及血尿发生率高,肾脏病理活动性明显;Ⅳ型、Ⅴ型、Ⅴ＋Ⅳ、Ⅴ＋ⅢLN肾病综合征、大量蛋白尿发生率高。肾脏病理活动指数与系统性红斑狼疮的临床活动性及抗dsDNA抗体阳性率无相关性。结论狼疮性肾炎的病理类型与临床表现、实验室检查有一定联系;但二者之间关系不确定。     

Intravenous cyclosporine therapy in recurrent nephrotic syndrome after renal transplantation in children

BACKGROUND: Early recurrence of massive proteinuria after renal transplantation occurs in 20% to 30% of patients with steroid-resistant idiopathic nephrotic syndrome and is responsible for graft failure in approximately half of cases. We report our experience with the use of intravenous (IV) cyclosporine (CsA) in children with recurrent proteinuria after renal transplantation. METHODS: Between March 1991 and August 2001, 36 renal transplantations were performed in 35 patients with steroid-resistant idiopathic nephrotic syndrome in our institution. Recurrence, defined by proteinuria higher than 50 mg/kg per day in the absence of acute rejection or urinary tract infection, was observed in 17 grafts performed in 16 patients. In patients with recurrence, CsA was administered IV, at an initial dose of 3 mg/kg per day, which was afterward adapted to maintain whole-blood levels between 250 and 350 ng/mL. RESULTS: In 14 of 17 cases (82%) with recurrence, proteinuria completely disappeared after 20.8+/-8.4 (range 12-40) days. The treatment was ineffective in the remaining three patients with persistent proteinuria at the end of the second month posttransplantation. Plasma exchanges were performed in four patients during the first 2 months, and proteinuria regressed in three cases and persisted in one. Persistent remission was observed in 11 patients with a follow-up of 3.7+/-3 (range 0.3-9) years. Actuarial graft survival was 92% and 70% at 1 and 5 years. CONCLUSION: IV CsA is a safe and effective treatment in children with recurrent nephrotic syndrome after renal transplantation.     

Long-term prognosis of Henoch-Schonlein nephritis in adults and children

Background: The aim of this multicentre collaborative study was to compare the progression of renal disease in children and adults with Henoch-Schonlein purpura (HPS) nephritis selected on the basis of IgA-dominant renal deposits and biopsy material available for review. Methods: The analysis was performed in 152 patients (95 adults and 57 children <16 years old at diagnosis) with a follow-up (⩽1 year up to 20 years (4.9±3.4 years in adults and 4.8±3.9 years in children). Results: Renal histology and clinical presentation were similar in both age groups: crescents were found in 36% of adults and 34.6% of children (in only 2.7% of adults and 1.9% of children involving >50% of glomeruli), nephrotic-range proteinuria in 29.5% of adults and 28/.1% of children and functional impairment in 24.1% of adults and 36.9% of children. The outcome was similar for both age groups (remission, 32.5% of adults and 31.6% of children; renal function impairment, 31.6% of adults and 24.5% of children). End-stage renal disease was observed in 15.8% of adults and in 7% of children. Renal function survival at 5 years was not significantly different in the two groups (85% in adults and 95% in children) and at 10 years it was approximately 75% in both groups. None of the children died and adult survival was 97% at 5 years. In adults at presentation, renal function impairment (P <0.02) as well as proteinuria higher than 1.5 g/day (P <0.02) and hypertension (P <0.001) were negative prognostic factors. Multivariate analysis stressed the main statistical relevance of proteinuria (relative risk 2.37, P <0.02). Conversely, in children no definite level of proteinuria, hypertension or other data were found to be associated with poor prognosis. Conclusion: Among patients with a clinical presentation which warrants renal biopsy, HSP nephritis has a similar prognosis in children and adults. The evolution is more predictable in adults than in children.     

Henoch-Schoenlein nephritis and IgA nephropathy in children: a comparison of clinical course

The clinical presentation, initial laboratory and renal biopsy findings, and subsequent clinical course were studied and compared in 128 children with Henoch-Schoenlein (HS) nephritis and in 206 children with IgA nephropathy. The clinical and pathological findings of the two conditions were similar. After a mean follow-up period of 5 years, 72 patients (56%) with HS nephritis and 67 (32%) with IgA nephropathy showed no demonstrable abnormality, 29 (23%) with HS nephritis and 103 (50%) with IgA nephropathy had minor urinary abnormalities, 7 (5%) with HS nephritis and 26 (13%) with IgA nephropathy had heavy proteinuria and/or hypertension, and 20 (16%) with HS nephritis and 10 (5%) with IgA nephropathy had developed chronic renal failure. A worse outcome was significantly associated with the more severe clinical presentations and more severe glomerular changes by light microscopy in HS nephritis, whereas there was no relationship between the severity of clinical presentation and glomerular changes and prognosis in IgA nephropathy. These findings suggest that HS nephritis is an acute disease and prognosis is associated with the severity of glomerular changes at onset, while IgA nephropathy is a chronic, slowly progressive glomerular disease.     

The natural history of IgA nephritis in Singapore

The clinical and histological features of 151 patient with IgA nephritis were analyzed to determine the prognostic features of the disease. The mean duration of follow up examinations was 50 +/- 34 months (range 6 to 168 months). The majority of the patients were young males and showed no signs of IgA nephritis. The disease was detected by routine screening before induction into national service. The plot of the reciprocals of serum creatinine against time in the patients with progressive disease showed that the patients ran two different courses when they developed renal impairment; one was a slow progressive course over an average of 7.7 years before reaching end stage renal failure (ESRF), while the other was a more rapid decline to ESRF within an average of 3.3 years in which severe uncontrolled hypertension seemed to be the major adverse factor. Hypertension was present in 23% of patients. Nine percent had renal impairment at the end of the follow up period while 5% progressed to ESRF. The cumulative renal survival was 91% after 6 years with no further development of renal failure up to 14 years. Unfavorable long term prognostic indices were proteinuria of more than 2 gm, hypertension and presence of crescents on renal biopsy.     

Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression

This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median estimated glomerular filtration rate (eGFR) was 91.3 ml/min per 1.73 m², with the median urine protein creatinine ratio (UP:UC) 556 mg/mmol. The treatment protocol comprised daily steroids and cyclophosphamide for 8–12 weeks followed by azathioprine and a reducing regimen of alternate-day steroids for 8–12 months. After a mean follow-up period of 7 years following presentation, 37% made a complete recovery, 40.7% had persistent proteinuria, 7.4% had persistent proteinuria and were on antihypertensive therapy and 14.8% had progressed to end-stage kidney failure (ESKF). Children with poor outcome were older at presentation (p 0.005), had more crescents (p 0.015) and had heavier proteinuria 6 months post initial biopsy (p 0.023). All of the four children with ESKF had nephrotic range proteinuria and greater than 50% crescents on initial biopsy. Despite long-term immunosuppression, the majority of children with HSN grade 3b or higher will have persistent renal abnormalities on long-term follow-up.     

Prevalence and clinicopathologic findings of antiphospholipid syndrome nephropathy in Thai systemic lupus erythematosus patients who underwent renal biopsies

AIM: To determine the prevalence of antiphospholipid syndrome nephropathy (APSN) in Thai systemic lupus erythematosus (SLE) patients who underwent renal biopsy and to compare the relationship of renal histopathology and other significant clinical parameters between SLE patients with and without APSN. METHODS: A retrospective analysis was undertaken in systemic lupus erythematosus patients (n = 150, 44 <15 years old, 106 0e;15 years old) who underwent renal biopsy. The specimens were evaluated for histological features of APSN and other significant clinical parameters. The result of antiphospholipid antibodies, clinical course, and renal function from chart review were analysed. RESULTS: The prevalence of APSN in systemic lupus erythematosus patients who underwent renal biopsies was 34% (16% in <15-year-old group, 41.5% in > or =15-year-old group). APSN was associated with more severe hypertension (P = 0.002 for systolic and P = 0.004 for diastolic blood pressure), acute renal failure (P = 0.003), persistent heavy proteinuria (P < 0.001 for 4+ proteinuria), severe lupus nephritis (class III and IV, P = 0.014, high activity and chronicity indices, P < 0.001) and a tendency to progress to end-stage renal disease. CONCLUSION: Systemic lupus erythematosus patients who underwent renal biopsies in our institute showed a prevalence of APSN comparable to those in western countries. The presence of APSN was significantly higher in the adult than in the paediatric population. Its association with poor prognostic indicators suggests poor renal outcome. Clinicians should be aware of this condition in order to give proper care to systemic lupus erythematosus patients.     

Treatment of IgA nephritis in Singapore

Summary: Any form of therapy for IgA nephritis must be rational and practical. the least toxic agent should be chosen with a view to even life long therapy for patients with bad prognostic indices. Patients with IgA nephritis have evidence of platelet involvement and low-grade intravascular coagulation, with a tendency for increased thrombogenicity within the kidneys. the use of dipyridamole (an anti-platelet agent) and warfarin (an anti-thrombotic agent) has been successful in two separate trials in patients with IgA nephritis. the relative lack of side-effects and their low cost allow their long-term use for treatment in these patients. the indications for combination therapy with dipyridamole and low dose warfarin are the presence of any one of the following parameters: proteinuria < 1 g m/day, hypertension, renal impairment, glomerulosclerosis < 20% on renal biopsy, presence of even a single crescent, medial hyperplasia of blood vessels on biopsy. Treatment of patients with IgA nephritis with asymptomatic haematuria and proteinuria consist of control of systemic hypertension, use of ACE inhibitor for glomerular hyperfiltration, dipyridamole and low dose warfarin, low protein diet (0.8 g/kg per body weight/day) and control of serum cholesterol to prevent lipid induced glomerulosclerosis. Patients with IgA nephritis who have the nephrotic syndrome with selective proteinuria, should be treated with prednisolone. Those who do not respond to steroids can be given a course of cyclophosphamide. Patients with IgA nephritis with predominantly tubular interstitital lesions not in proportion to the degree of glomerular sclerosis should be treated with a low dose steroid regimen. While there is no cure for IgA nephritis such therapeutic measures serve to retard the progression to end-stage renal failure (ESRF). Future therapeutic strategies lie in immunomodulation using inhibitors of cytokines and possibly gene therapy.     

Lupus nephritis at the University Hospital of the West Indies, Kingston, Jamaica: a 10-year experience

The case records of 75 patients who had systemic lupus erythematosus (SLE) and renal involvement on renal histology were reviewed. There were 70 females and 5 males, with a ratio of 14:1. The majority of the patients were in their second and third decades of life. SLE nephritis was distinctly uncommon in patients below 10 years of age and above 50 years of age. In nearly 80% of the patients, SLE nephritis was diagnosed within the year prior to presentation. The most common presenting clinical features were arthritis in 78% and a rash in 48%. The two most common renal features were proteinuria in 60% and renal failure in 55%. Of these patients, 20%, 12%, 48%, and 17% fell into Class II (mesangial GN), Class III (focal GN), Class IV (diffuse GN), and Class V (membranous GN), respectively, when the renal histological abnormalities were categorized according to the World Health Organization classification. The remaining 3% of patients had sclerosis and as a result were unclassifiable. The two major clinical manifestations in the Class IV patients were an acute nephritis syndrome in 83% and renal failure in 61%. The nephrotic syndrome was seen in 62% of Class V (membranous GN) cases. There was 100% survival in Classes II (mesangial GN) and V (membranous GN), and 62% percent in Classes III (focal GN) and IV (diffuse GN) combined.