MO1006COMPARISON OF DIFFERENT DEFINITION OF PARTIAL AND COMPLETE REMISSION IN A COHORT OF CHILDREN WITH LUPUS NEPHRITIS*

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Chiara de Mutiis ◽  
Kjell Tullus

Abstract Background and Aims Lupus Nephritis (LN) occurs in up to 80% of children with SLE and it affects the long term outcome and the overall survival. Achieving and maintaining renal remission is crucial. However definition of remission in children is not clearly defined. We compared the outcomes using different published definitions of complete and partial remission. Method 248 children with biopsy proven LN class III or higher (ISN/RPS) diagnosed and treated in 23 international centers in the last 10 years were included. Data regarding their renal outcome were collected for twenty-four months after the start of induction therapy. We applied seven definitions of remission to compare the number of children achieving partial and complete remission. Definitions applied have been adapted from the Bristol-Myers Squibb (BMS) trial, the American College of Rheumatology (ACR) recommendations, the Lupus Nephritis Assesment with Rituximab (LUNAR) trial, the Aspreva Lupus Management Study (ALMS) trial, the Abatacept and Cyclophosphamide Combination: Efficacy and Safety Study (ACCESS) trial, the Kidney Disease Improving Global outcomes (KDIGO) guidelines and the Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis (BLISS-LN). We also focused on the BMS trial, the ACCESS trial and the KDIGO guidelines definitions to analyse the importance of gender, age, ethnicity and the economic income of the country (as defined by the World Bank) where patients had been treated. Results The mean age at diagnosis was 11 years and 4 month. 71.4% were females. They were mainly East-Asian (34.3%), South-Asian (24.6%) and Caucasian (18.6%). 42.7 % were from middle income countries and 57.3% high income countries. The kidney biopsies showed LN class III in 35.5%; class IV in 45.6% and class V in 18.9%. The different definitions varied significantly in terms of outcomes, with that of the ACCESS trial having the highest percentages of complete remission and the BMS trial the lowest (Figure 1). A relatively small percentage of children achieved partial remission during the follow-up for all the definitions (Figure 2). Focusing on the BMS, ACCESS and KDIGO definitions, we found no statistically significant differences of gender and age in the rate of children entering complete remission at 6, 12 and 24 months. East Asian children did however achieve remission more often than other ethnic groups (p < 0.05) (Figure 3). Children treated in high income countries showed a statistically significant higher percentage of complete remission at 12 and 24 months (p < 0.05) (Figure 4). Conclusion Rate of complete and partial remission varied considerably when using the different definitions. Ethnicity and income of the country where the patients were treated did influence outcome. The findings of our study can help in deciding how to define remission in urgently needed future treatment studies in children.

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1199.1-1199
Author(s):  
T. Knežević ◽  
I. Padjen ◽  
S. Bulimbašić ◽  
M. Ćorić ◽  
V. Ivković ◽  
...  

Background:Optimal prognostic predictors in lupus nephritis (LN) are still not clearly defined. Studies have recently shown that albumin-to-globulin ratio (A/G) is not only a good predictor of outcomes in malignancy, infection and renal disease, but is also a potential marker of increased immunoglobulin synthesis and LN activity and might be useful as a predictor of the development of LN in SLE patients. Studies on the role of A/G in predicting outcomes in LN are, however, lacking.Objectives:To assess a potential prognostic value of A/G at renal biopsy in predicting outcomes in LN.Methods:In this retrospective study we analyzed the demographic, histologic, laboratory and clinical characteristics of patients with biopsy-proven LN diagnosed between 2011 and 2020. All patients met the 1997 revised American College of Rheumatology classification criteria for SLE. Complete remission was defined as proteinuria <0.5 g/day and serum creatinine within 10% from baseline, and partial remission as more than 50% reduction in proteinuria to sub-nephrotic levels and serum creatinine within 10% from baseline.Results:We have included 55 patients (80% women) with biopsy-proven LN (age at biopsy 38±13 years). On renal histology, one patient was class I LN, two patients were class II, 7 were class III, 5 were class III+V, 23 were class IV, two were class IV+V, 11 class VI and 4 had other features. Median eGFR and proteinuria at biopsy were 69 (IQR: 44, 100) mL/min/1.73 m2 and 3.5 (2.3, 5.6) g/day, respectively. Baseline mean A/G was 1.12±0.29 and did not differ between LN classes (p=0.91). At one-year follow-up eGFR increased non-significantly (69 vs. 82 mL/min/1.73 m2, p=0.13) and proteinuria decreased (3.5 vs. 0.6 g/day, p<0.001). Eighteen patients achieved complete remission (CR), 16 partial remission and 21 did not achieve remission. None progressed to ESRD and two patients died, both from sepsis. A/G at the time of biopsy did not differ between group which achieved CR/PR vs. no remission (p>0.05; Figure 1) and was not a significant predictor of remission in a multivariate regression model (OR 5.9, 95% CI 0.6, 63.2).Conclusion:While being a potential marker for transition of SLE to LN, A/G was not showed to be a predictor of renal remission in LN.References:[1]Liu XR, et al. Lupus. 2021 Jan 6:961203320981139.[2]Kwon OC, et al. Semin Arthritis Rheum. 2018 Dec;48(3):462-466ckDisclosure of Interests:None declared


Lupus ◽  
2019 ◽  
Vol 28 (13) ◽  
pp. 1598-1603 ◽  
Author(s):  
S H Koubar ◽  
J Kort ◽  
S Kawtharani ◽  
M Chaaya ◽  
M Makki ◽  
...  

Introduction Systemic lupus erythematosus affects 4.8–78.5 people per 100,000 worldwide, 90% of whom are females. Geography and ethnicity have been shown to significantly affect the prevalence and natural history of the disease. Lupus nephritis affects around half of patients with systemic lupus erythematosus. Data about systemic lupus erythematosus and lupus nephritis in the Middle East are still scarce. In this study, we aimed to describe the characteristics of systemic lupus erythematosus and lupus nephritis at a tertiary care center in Lebanon. Methods This is a retrospective chart review of all biopsy-proven lupus nephritis patients admitted to the American University of Beirut medical center between January 2000 and December 2018. Patients above 12 years of age who had any International Society of Nephrology/Renal Pathology Society (ISN/RPS) class of lupus nephritis on their renal biopsy were included in the study. Results The study included 55 patients with lupus nephritis. Upon presentation of systemic lupus erythematosus, the most common clinical feature was arthritis, seen in 83% of patients, followed by anemia (82%) and malar rash (48%). In total 93% had positive ANA, 89% had positive anti-dsDNA and 98% of patients had proteinuria. The most common ISN/RPS class of lupus nephritis in our series was IV (49%). At the time of the biopsy 15% of patients underwent dialysis. At 6 months, 11/27 had complete remission, 6/27 had partial remission and 10/27 had no remission. At 1 year, 8/23 had complete remission, 4/23 had partial remission and 11/23 had no remission. During the study period, 15 out of 35 patients available for analysis had chronic kidney disease (CKD) and six out of 34 patients developed end-stage kidney disease requiring renal replacement therapy. In comparison to other series in the region, our series had more males affected, higher creatinine at the time of biopsy and greater degree of proteinuria. Conclusion Our study provided insight on the demographics, characteristics, and outcomes of lupus nephritis in Lebanon. Interestingly, male gender was present in a quarter of patients. This warrants further investigation and confirmation. We are hoping to expand this experience into a national prospective registry to further characterize this entity in our region.


Lupus ◽  
2017 ◽  
Vol 27 (3) ◽  
pp. 461-467 ◽  
Author(s):  
E S Park ◽  
S S Ahn ◽  
S M Jung ◽  
J J Song ◽  
Y-B Park ◽  
...  

We investigated renal outcome of kidney-transplantation in 19 Korean recipients with biopsy-proven lupus nephritis and compared it with 18 Korean age- and gender-matched recipients without lupus nephritis who were diagnosed with end-stage renal disease caused by renal diseases other than lupus nephritis in a single centre. We reviewed histological findings of kidneys and calculated cumulative dose of immunosuppressive agents. We assessed renal flare of systemic lupus erythematosus, recurrence of lupus nephritis and graft failure as prognosis. The mean age of recipients with lupus nephritis was 43.5 years and all patients were female. Six patients had class III, 10 had class IV and three had class V. There were no meaningful differences in demographic data, renal replacement modality, cumulative doses of immunosuppressants and prognosis between recipients with and without lupus nephritis. Eight patients experienced renal flare of systemic lupus erythematosus, but there were no cases of recurrence of lupus nephritis or graft failure in recipients with lupus nephritis. Kidney-recipients with class IV lupus nephritis exhibited a lower cumulative renal flare of systemic lupus erythematosus free survival rate than those with class III lupus nephritis. In conclusion, renal outcome of kidney-transplantation in patients with lupus nephritis is similar to that in those without lupus nephritis, and class IV was associated with renal flare of systemic lupus erythematosus.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Asaka Hachiya ◽  
Munetoshi Karasawa ◽  
Takahiro Imaizumi ◽  
Noritoshi Kato ◽  
Takayuki Katsuno ◽  
...  

AbstractLupus nephritis (LN) is a life-threatening complication of systemic lupus erythematosus. The 2003 pathological classification of LN was revised in 2016; it quantitatively evaluates the interstitium in addition to the glomeruli. We performed a retrospective multi-centre cohort study and investigated the utility of the 2016 classification—including the activity index (AI), chronicity index (CI), and each pathological component to predict complete remission or renal function decline, defined as 1.5-fold increase in serum creatinine levels—and compare with that of the 2003 classification. Ninety-one consecutive adult patients with first-onset class III/IV LN who were newly prescribed any immunosuppressants were enrolled and followed up for a median of 51 months from January 2004. Cox regression analysis demonstrated the subclasses based on the 2003 classification, which mainly evaluate glomerular lesions, were not associated with clinical outcomes. After adjustments for estimated glomerular filtration rate and urinary protein levels, higher CI and higher interstitial fibrosis and lower hyaline deposit scores were associated with renal functional decline. Similarly, higher CI and interstitial inflammation scores were associated with failure to achieve complete remission. Therefore, the 2016 classification can predict the clinical outcomes more precisely than the 2003 classification.


2008 ◽  
Vol 61 (5-6) ◽  
pp. 270-273
Author(s):  
Dejan Celic ◽  
Tatjana Ilic ◽  
Tatjana Djurdjevic-Mirkovic ◽  
Igor Mitic ◽  
Biljana Milic ◽  
...  

Introduction Mycophenolate mofetil is an immunosupressive agent used in transplantation and subsequently in a variety of autoimmune conditions. It inhibits both B and T lymphocyte proliferation, and also has nonimmune effects on the kidney. The major experience in systemic lupus erythematosus has focused on proliferative lupus nephritis. Materials and methods In our study we treated 8 female patients having proliferative lupus nefritis with combination therapy of prednisone (1mg/kg body weight) and mycophenolate mofetil (2g per day). Complete remission was defined as a value for urinary protein excretion that was less than 0.5g per 24 hours, with normal urinary sediment, a normal serum albumin concentration and improved or stable serum creatinine. Partial remission was defined as a daily proteinuria below 2g in the previosly nephrotic patient or minimum 30% from starting values, with normal urinary sediment, serum albumin of minimum 30g/L and stable serum creatinine. Results Two patients had a complete remission after 7 and 2 months respectively. Five patients had a partial remission after 5.2?4.3 months of therapy. One patient did not react to therapy. There were no side effects during the course of therapy. Discussion Considering the fact that 7/8 patients have had nephrotic range proteinuria and that 50% of patients were refractory on standard induction therapy, the results of this study are a good indicator of value of mycophenolate mofetil in the therapy of proliferative forms of lupus nephritis. Conclusion Mycophenolate mofetil has satisfactory results in the treatment of proliferative forms of lupus nephritis with minimal side effects.


2021 ◽  
Vol 8 (1) ◽  
pp. e000533
Author(s):  
Valérie Pirson ◽  
Antoine Enfrein ◽  
Frédéric A Houssiau ◽  
Farah Tamirou

BackgroundThe very long-term consequences of absence of remission in lupus nephritis (LN) remain understudied.MethodsIn this retrospective analysis, we studied a selected cohort of 128 patients with biopsy-proven class III, IV or V incident LN followed for a median period of 134 months (minimum 25). Remission was defined as a urine protein to creatinine (uP:C) ratio <0.5 g/g and a serum creatinine value <120% of baseline. Renal relapse was defined as the reappearance of a uP:C >1 g/g, leading to a repeat kidney biopsy and treatment change. Poor long-term renal outcome was defined as the presence of chronic kidney disease (CKD).ResultsTwenty per cent of patients never achieved renal remission. Their baseline characteristics did not differ from those who did. Absence of renal remission was associated with a threefold higher risk of CKD (48% vs 16%) and a 10-fold higher risk of end-stage renal disease (20% vs 2%). Patients achieving early remission had significantly higher estimated glomerular filtration rate (eGFR) at last follow-up compared with late remitters. Accordingly, patients with CKD at last follow-up had statistically longer time to remission. Among patients who achieved remission, 32% relapsed, with a negative impact on renal outcome, that is, lower eGFR values and higher proportion of CKD (33% vs 8%).ConclusionEarly remission should be achieved to better preserve long-term renal function.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1787-1787
Author(s):  
Svetlana Asenova ◽  
Ulrike Bacher ◽  
Andreas Gerritzen ◽  
Axel R. Zander ◽  
Nicolaus Kröger

Abstract Abstract 1787 Poster Board I-813 Introduction The qualitative assay for free light chain has been reported to be sensitive and specific for detecting and monitoring diseases caused by monoclonal gammopathies such as multiple myeloma. More recently the International Myeloma Working Group proposed uniform response criteria including a new definition of stringent complete remission (sCR). The definition of stringent complete remission requires beside absence of clonal cells in bone marrow by immunohistochemistry or immunofluorescence also normalization of free light chain ratio in serum. Patients and Methods We evaluate the value of free light chain assay to determine stringent CR by monitoring 87 patients with multiple myeloma who achieved complete remission (n=52) or very good partial remission (n=35) between January 2003 and December 2008. Free light chain measurements were performed with the commercially available Free liteTM Kit (Binding Site, Heidelberg, Germany). Because of the shorter half-life of free light chain assay ratio, only patients were included, if the complete or very good partial remission remains stable for at least 3 months. The comparison between immunofixation and free light chain ratio was performed at least 6 weeks after immunofixation becomes negative for the first time. 87 patients (50 mal and 37 female) were included, 67 had intact immunoglobulin and 11 had light chain immunoglobulin at time of diagnosis. The remission status was determined either after allogeneic (n = 73) or autologous (n = 7) stem cell transplantation or after conventional bortezomib or lenalidomide containing chemotherapy (n = 7). Results 35 out of 87 patients achieved a very good partial or near complete remission with still positive immunofixation. In 22 out of those 35 patients the free light chain kappa ratio was within the normal range of 0.26 – 1.65 mg/l (63 %). Only in 13 patients with persistent immunofixation positivity the free light chain ratio was outside the normal range (37%). 52 patients achieved complete remission according to the EBMT criteria with negative immunofixation for at least 3 months. In those patients all (100 %) had a normal free light chain kappa/lambda ratio. In a subgroup of patients (n = 10) who relapsed during follow-up from complete remission sequential monitoring of immunofixation and free light assay was performed as recently described [4]. In 9 out of 10 patients a free light chain ratio became abnormal at a median 90 days before immunofixation became positive. Conclusions The free light chain assay ratio is a useful marker for a faster detection of remission or progression in myeloma patients. However, these results do not support additional value of free light chain ratio to determine the depth of remission in immunofixation negative patients. More sensitive methods such as imunophenotyping analysis by FACS or molecular primer should be used to determine depth of complete remission since these methods have shown relevant clinical impact. Disclosures No relevant conflicts of interest to declare.


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