Clinicopathological correlations of paediatric lupus nephritis (original) (raw)
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Clinicopathology of childhood-onset renal systemic lupus erythematosus
Nephrology, 2007
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.
Clinical course, Morphology, and Treatment outcome of Childhood-onset Lupus Nephritis
Introduction: Lupus nephritis, more commonly known as lupus, is an inflammation of the kidney that is caused by systemic lupus erythematosus (SLE). This is an autoimmune disease. With lupus, the body's immune system targets its body tissues. Lupus nephritis happens when lupus involves the kidneys. The present study focused on the childhood onset of lupus nephritis, its clinical course, morphology, and treatment outcome. Aim of the study: To evaluate the clinical behavior, the treatment recommendations according to morphological changes, and to improve the management of childhood-onset lupus nephritis Methods: A total of 38 lupus nephritis children, less than 18 years, treated and admitted under the Pediatric Nephrology Department of Dhaka Shishu (Children) Hospital from July 2017 to December 2019 were included in our study. We recorded the clinical and demographic features, lab variables, treatment, and outcome. Result: Mean age at onset of lupus was 11 yrs (range 3-16 yrs). The Female: male ratio was 7:2. Proteinuria 36(94.7%) and hematuria 23(60.5%) were the commonest findings on admission. Hypertension was observed in 24 (63.2%) patients. 14 (36.8%) patients had renal impairment at onset of SLE. Common extrarenal findings were hematological (76.3%) and mucocutaneous (65.8%). 71% had anemia, reduced C3, C4 was present in 89.5% of patients. ANA was positive in 97.4% and anti-Ds DNA was positive in 100% of patients. Renal biopsy was done in 26 patients and diffuse proliferative lupus was the commonest histopathology. Activity scores were more eminent than chronicity scores. All patients received hydroxychloroquine and corticosteroid mostly methylprednisolone pulse for induction followed by oral prednisolone. 6 (15.8%) patients were treated with cyclophosphamide pulse during induction followed by MMF as maintenance therapy. 31(81.6%) patients were treated with MMF both during induction and maintenance. 6 (15.8%) patients need dialysis. 9 (23.7%) patients died during hospitalization. All deceased patients took MMF during induction along with methylprednisolone pulse and histopathologically they were diffuse proliferative lupus. Conclusion: Our study showed worse survival using MMF during the induction phase of therapy having diffuse proliferative glomerulopathy in biopsy although MMF was considered as a potential alternative to more toxic regimens for induction. The presence of hypertension, infection, diffuse proliferative lesion, and acute kidney injury was more frequent among deceased patients. Delayed diagnosis, referral, and delayed initiation of treatment due to low socioeconomic status were other factors of unfavorable outcomes in children.
Outcome of lupus nephritis in Iranian children: prognostic significance of certain features
Pediatric Nephrology, 2008
The objective of this study was to determine the clinical and histopathological features and outcome of children with lupus nephritis (LN). Of 84 children with systemic lupus erythematosus (SLE), we retrospectively studied 58 children (69%) under 15 years of age with biopsy-proven LN who had been followed between October 1989 and January 2005. The mean age at diagnosis or initial referral was 10.6±2.25 years, and the mean followup was 5.3±4.1 years. Class IV LN was observed in 34 (58.6%) patients. The 5-year patient and renal survival rates were 82.5 and 78.5%, respectively, in the total group, and 75 and 85.8%, respectively, in patients with Class IV LN. No independent predictor of unfavorable outcome, including renal histology, was detected by multivariate analysis. The mid-term patient and the renal survival rates of Iranian children with biopsy-proven LN are high. Within 5 years of follow-up, renal histology was not a predictor for survival.
Clinicopathological study of the WHO classification in childhood lupus nephritis
Pediatric Nephrology, 2004
Over the past 10 years, at our center, 25 children diagnosed with systemic lupus erythematosus (SLE) have undergone an early renal biopsy; 15 underwent a second biopsy. The objective of this study was to determine whether clinical and laboratory parameters used to evaluate lupus disease activity and nephritis correlated with the WHO class on biopsy. At diagnosis, the presence of proteinuria, hematuria, a lower serum albumin, and the need for blood pressure medication were all associated with a worse class of lupus nephritis (P<0.05). On follow-up biopsy, however, none of these parameters correlated with the WHO class. Thus, it appears that while the WHO classification is useful for categorizing disease at presentation, it may be less useful for the evaluation of disease progression. Other biopsy indices need to be evaluated in serial renal biopsies to better understand the progression of lupus nephritis once treatment has been initiated.
Approach to Classification and Management of Childhood-Onset Lupus Nephritis
Current Pediatrics Reports, 2020
Purpose of Review (1) To discuss the relevance of the 2018 International Society of Nephrology/Renal Pathology Society (ISN/ RPS) classification system for nephritis in childhood-onset systemic lupus erythematosus (SLE). (2) To highlight current practices not addressed in the 2013 Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plan (CTP) for pediatric lupus nephritis (LN). Recent Findings In addition to class of glomerular involvement, multiple features on kidney biopsy can be assessed for prognosis and therapeutic decision-making. There is pediatric evidence and expertise for optimization of LN classification. Yet, treatment is based primarily on whether nephritis is proliferative or membranous. Moreover, alternative strategies for induction therapy are more commonly prescribed for children. Summary There is a need for pediatric data to evaluate/validate LN classification systems. Updates are needed to CTPs for LN induction therapy. Pediatric nephrologists should be enrolling LN patients into existing prospective registries to build the evidence base for treatment decisions.
Nephrology Dialysis Transplantation, 2009
Introduction. Lupus nephritis (LN) is the major indicator of morbidity and mortality in systemic lupus erythematosus (SLE). Many studies have found a significantly worse patient survival rate in patients with LN class IV than patients with other LN classes. Objective. The aim was to describe the severity and outcomes of LN in a group of Thai children. Methods. We retrospectively reviewed the patient files of children diagnosed with SLE aged ≤18 years in Songklanagarind Hospital, Southern Thailand, from 1985 to 2007. Results. Of 216 SLE patients, 180 had renal biopsy results, and the others were excluded from analysis. There were 33 males and 147 females, average age 11.8 ± 2.6 years (range 3.6-18.0), with a median follow-up period of 3.9 years (range 9 days to 19.4 years). Using the WHO LN classification, there were 9, 55, 5, 94 and 14 patients of classes I-V, respectively, as well as 2 with end-stage renal disease and 1 with IgM nephropathy. The mortality rate was 23% (42/180). Patients with LN class II had a similar renal and patient survival compared to patients with LN class IV (P = 0.3 and 0.2, respectively). Cox proportional hazard regression analysis in 177 patients (3 patients who had a renal biopsy result outside the WHO classification were omitted) showed that gender was an independent risk factor for survival. Males had 2.6 times the hazard rate compared to females (95% CI 1.2-5.7, P = 0.03), but LN classification, age and timing of the renal biopsy were not significant. Conclusion. Renal and patient survival in LN classes II and IV were similar. Gender was the only independent risk factor of mortality, with males at greater risk than females.
Renal involvement in childhood-onset systemic lupus erythematosus in Egypt
Rheumatology International, 2012
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 childhoodonset 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.
Severe paediatric systemic lupus erythematosus nephritis--a single-centre experience
Nephrology Dialysis Transplantation, 2010
Background. Paediatric patients with systemic lupus erythematosus (SLE) often have severe presentations including lupus nephritis (LN). Few paediatric studies have evaluated the anticardiolipin antibody (aCL) and renal histology. The purpose of this study was to evaluate clinicopathologic features, including aCL, short-term clinical and renal histologic outcomes of paediatric patients with new-onset SLE nephritis. Methods. We conducted a single centre, retrospective inception cohort study. Charts were reviewed at presentation (initial renal biopsy), 6-month (follow-up biopsy) and 12month follow-up. Results. The population consisted of 21 patients (median age, 14.5 years): 19/21 were female, 6/21 African American, 3/21 Asian, 9/21 Caucasian and 3/21 Hispanic. At presentation, 19/21 had elevated aCL, 15/21 hypertensive, 12/21 nephrotic and 7/21 required haemodialysis (HD)-2/7 HD patients had thrombotic microangiopathy, 1/7 crescentic glomerulonephritis. Two patients had thromboembolism: both had aCL, were taking oral contraceptives and required HD, one was nephrotic and the other had elevated lupus anticoagulant. Initial biopsies revealed 6/21 ISN/RPS class II nephritis, 3/21 class III, 7/21 class IV and 5/21 class V. Treatment consisted of methylprednisolone, corticosteroids, cyclophosphamide or mycophenolate mofetil. Follow-up biopsies revealed 12/13 to have improved histology. Indication for a follow-up biopsy was severe illness at presentation. At 12-month follow-up, no patients were nephrotic (P < 0.001) or required HD (P < 0.001), and 3/14 had elevated aCL (P < 0.001). Conclusion. Elevated aCL, hypertension, nephrotic syndrome and need for HD were common presentations among our paediatric SLE nephritis population. Renal histology and aCL were helpful in the therapeutic management.