AB0398 TNF-LIKE WEAK INDUCER OF APOPTOSIS / FGF INDUCIBLE MOLECULE 14 PATHWAY IN UNTREATED LUPUS NEPHRITIS: SERUM OR URINE TWEAK LEVELS MORE ACCURATE IN RELATION TO RENAL ACTIVITY?

Background:Lupus nephritis (LN) is one of the most serious manifestations of SLE, affecting 70% of patients and the most critical predictor of morbidity and mortality of the disease [1].Tumor necrosis factor-like weak inducer of apoptosis/ fibroblast growth factor inducible molecule 14 (TWEAK/Fn14) activation is involved in various pathological processes that occur locally in kidneys, facilitating the pathogenesis of LN [2].Objectives:To assess serum and urine TWEAK levels as well as renal Fn14 expression in newly diagnosed patients with LN and its correlation to disease activity.Methods:The present study included 30 selected newly diagnosed previously untreated SLE patients divided into 2 groups; 15 patients with LN and 15 without LN as well as 30 age and sex matched healthy subjects who served as control group. Written consent was obtained from all patients and controls after a full explanation of the study. Clinical assessment of disease activity by SLE Disease activity index (SLEDAI) [3]. Lupus nephritis was assessed clinically with the renal SLE disease activity index (rSLEDAI).Indicated renal biopsies were taken from the patients with LN and were classified according to the International Society of Nephrology and the Renal Pathology Society (ISN/RPS) classification [4].Serum and urinary levels of TWEAK were measured for the patients and controls using enzyme-linked immunosorbent assay (ELISA). Fn 14 was examined in renal biopsies from LN group by immunohistochemistry.Results:A significantly higher uTWEAK level on comparing SLE patients with LN to those without LN and controls (F = 149.2,P< 0.001). uTWEAK had a highly significant positive correlation with, proteinuria (r = 0.755,P< 0.001), a significant positive correlation with SLEDAI and rSLEADI (r = 0.217,P< 0.037) (r = 0.476,P< 0.024) respectively. uTWEAK had a significant negative correlation with anti-dsDNA titres, C3 and C4 (r = -0.579,P< 0.008) (r = -0.456,P< 0.011) (r = -0.552,P< 0.002). Although sTWEAK level was higher in SLE patients than controls, it was not found to be associated with the presence of LN (F = 4.963, P =0.012). Fn14 expression was detected in glomerular and tubular cells in LN patients.Expression of Fn14 in renal biopsies from LN patients was examined.Immunohistochemistry for Fn14 was detected in(A) glomerular endothelial cells,(B, C) some specimens showed moderate and intense staining for Fn14 in renal tubular cells. In the controls [renal biopsies from patients with renal cell carcinoma(D)], there was very slight staining for Fn14 in renal tubular cells.Conclusion:Urinary TWEAK is a specific and sensitive biomarker for detection of active LN in newly diagnosed untreated SLE patients.References:[1]Kwok SK and Tsokos GC. New insights into the role of renal resident cells in the pathogenesis of lupus nephritis. Korean J Intern Med 2018; 33 (2): 284-289.[2]Xia Y, Herlitz LC, Gindea S, Wen J, Pawar RD, Misharin A, et al. Deficiency of Fibroblast Growth Factor-Inducible 14 (Fn14) Preserves the Filtration Barrier and Ameliorates Lupus Nephritis. J Am Soc Nephrol 2015; 26(5):1053-1070.[3]Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH, Derivation of the SLEDAI. A disease activity index of lupus patients. Arthritis Rheum 1992; 35: 630-40.[4]Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol 2004; 15(2): 241-250.Disclosure of Interests:None declared

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The Spectrum of C4d Deposition in Renal Biopsies of Lupus Nephritis Patients

Frontiers in Immunology ◽

10.3389/fimmu.2021.654652 ◽

2021 ◽

Vol 12 ◽

Author(s):

Ying Ding ◽

Xiaojuan Yu ◽

Lihua Wu ◽

Ying Tan ◽

Zhen Qu ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Activity Index ◽

Hazard Analysis ◽

Disease Activity Index ◽

Complement Factor ◽

Pathological Features ◽

Renal Outcomes ◽

Renal Biopsies ◽

C4d Staining

ObjectivesThis study aimed to determine the prevalence and localization of complement factor C4d in renal biopsies from patients with lupus nephritis (LN), as well as its associations with the disease’s clinico-pathological features. The correlation between arteriolar C4d deposition and renal microvascular lesions (RVLs) was further analyzed.MethodsA total of 325 biopsy-proven LN patients were enrolled, and their clinico-pathological data were collected. C4d staining of renal biopsies was performed by immunohistochemistry. The associations between C4d deposition and the clinico-pathological features were further analyzed.ResultsC4d deposition was present in most (98.8%) renal specimens in our cohort. These deposits were localized in the glomeruli (98.2%), tubular basement membrane (TBM) (43.7%), arterioles (31.4%), and peritubular capillary (33.8%). Patients with TBM C4d staining had higher disease activity (measured with the Systemic Lupus Erythematous Disease Activity Index) and higher National Institutes of Health pathological activity and chronicity indices (all P < 0.01). Patients with arteriolar C4d deposition were more likely to develop RVLs (91.2%) compared to those with no arteriolar C4d deposition (78.0%; P = 0.004), especially with two or more types of RVLs (P < 0.001). During the mean follow-up of 55.8 months, arteriolar C4d was related to worse renal outcomes [hazard ration (HR): 2.074, 95% confidence interval (CI) 1.056–4.075, P = 0.034]. Multivariate Cox hazard analysis showed that co-deposition of arteriolar C4d and C3c was an independent risk factor (HR: 3.681, 95% CI 1.519–8.921, P = 0.004) for predicting renal outcomes.ConclusionsC4d deposition was common in renal tissues from LN patients. TBM C4d deposition was related to the disease activity, and arteriolar C4d deposition was associated with RVLs and worse renal outcomes.

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Urinary Neutrophil Gelatinase-Associated Lipocalin to Monitor Lupus Nephritis Disease Activity

Biomarker Insights ◽

10.4137/bmi.s27625 ◽

2015 ◽

Vol 10 ◽

pp. BMI.S27625 ◽

Cited By ~ 1

Author(s):

Hani Susianti ◽

Jullyanny W. Wijaya ◽

Ati Rastini ◽

Kusworini Handono ◽

Atma Gunawan ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Transforming Growth Factor ◽

Activity Index ◽

Disease Activity Index ◽

Transforming Growth Factor Β1 ◽

Sledai Score ◽

Neutrophil Gelatinase Associated Lipocalin ◽

Neutrophil Gelatinase

Background This study was conducted to determine whether there is an association between urinary neutrophil gelatinase-associated lipocalin (uNGAL) and urinary transforming growth factor-β1 (uTGF-β1) with lupus nephritis (LN) disease activity. Methods Urine samples from 18 LN patients were collected every month for six months then examined for uNGAL, uTGF-β1, and renal domain Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score. Results The uNGAL levels were significantly different between active and inactive LN (P < 0.05). uTGF-β1 levels were not different between active and inactive LN (P > 0.05). There was a significant correlation between uNGAL levels and renal domain SLEDAI score (r= 0.417, P < 0.05). There was no correlation between uTGF-β1 levels and renal domain SLEDAI score (r = 0.031, P > 0.05). Conclusion uNGAL is better than uTGF-β1 for differentiation of active and inactive LN. uNGAL can be considered as a biomarker to monitor LN disease activity.

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Understanding the dynamics of hydroxychloroquine blood levels in lupus nephritis

Lupus ◽

10.1177/0961203320912832 ◽

2020 ◽

Vol 29 (6) ◽

pp. 560-568 ◽

Cited By ~ 3

Author(s):

Tatiana N Pedrosa ◽

Sandra G Pasoto ◽

Nadia E Aikawa ◽

Emily FN Yuki ◽

Eduardo F Borba ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Blood Level ◽

Lupus Erythematosus ◽

Activity Index ◽

Disease Activity Index ◽

Blood Levels ◽

Persistent Pattern ◽

Liquid Chromatography Tandem Mass ◽

Group 5

Objectives It is unknown if hydroxychloroquine blood level dynamics impact flare rates in lupus nephritis patients. We prospectively evaluated hydroxychloroquine levels to determine which blood-based patterns are more associated with disease activity. Methods In total, 82 lupus nephritis patients under a prescribed hydroxychloroquine dose of 4–5.5 mg/kg actual body weight (maximum 400 mg/day) for ≥3 months were evaluated at baseline and 7 months. Hydroxychloroquine blood levels were determined by liquid chromatography-tandem mass spectrometry. Flare was defined as increase ≥3 in the Systemic Lupus Erythematosus Disease Activity Index 2000 score and/or a change or increase in therapy. Results Overall, 9/82(11%) patients had flares during follow-up and had lower baseline hydroxychloroquine blood levels than those without flares (220.4 (53.5–1471.1) vs. 1006.3 (53.5–2137.8) ng/ml, p = 0.013). The hydroxychloroquine blood level cut-off that best predicted flares was 613.5 ng/ml (odds ratio = 8.67, 95% confidence interval: 1.66–45.18, p = 0.006). For 77 (94%) patients, the 7-month hydroxychloroquine level dynamics was evaluated and revealed: 59/77 (77%) had a persistent pattern of adequate (41/77(53%)) or fluctuating (18/77 (23%)) levels, with a low and comparable risk of flares (2/41 (5%) vs. 1/18 (5%), p = 1.000). The remaining group had persistent low levels (18/77 (23%)) and their flare frequency was significantly higher than the adequate group (5/18 (28%) vs. 2/41 (5%), p = 0.023). The frequencies of adequate/inadequate hydroxychloroquine blood levels in patients were comparable at baseline and 7 months (McNemar’s test, p = 0.480). Conclusion We provide novel evidence that hydroxychloroquine blood-level patterns (persistently low, adequate, or intermittent) have distinct impacts on flare rates in lupus nephritis patients. These findings reinforce the need of routine hydroxychloroquine measurements to maintain the appropriate blood levels.

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Progranulin and insulin-like Growth Factor Binding Protein-2 as Biomarkers of Disease Activity and Pathological Changes in Lupus Nephritis

Aktuelle Rheumatologie ◽

10.1055/s-0043-121629 ◽

2017 ◽

Vol 44 (02) ◽

pp. 121-127

Author(s):

Samar Goma ◽

Marwa Abdelaziz ◽

Eman El-Hakeim ◽

Mona El Zohri ◽

Sohair Sayed

Keyword(s):

Growth Factor ◽

Kidney Disease ◽

Lupus Nephritis ◽

Disease Activity ◽

Renal Biopsy ◽

Activity Index ◽

Binding Protein ◽

Disease Activity Index ◽

Insulin Like Growth Factor ◽

Factor Binding

Abstract Background Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease, characterised by the production of auto-antibodies and the formation of immune complexes due to the polyclonal activation of T and B lymphocytes, which results in tissue and organ damage. During inflammation, neutrophils and macrophages release serine proteases to cleave progranulin (PGRN) into granulin, which exerts its pro-inflammatory effects that counteract the anti-inflammatory effects of intact PGRN. It is suggested that insulin-like growth factor binding protein-2 (IGFBP-2) is a dependable biomarker of renal deterioration but it is still unclear if it has high sensitivity and specificity for discriminating SLE-caused kidney disease from other-cause kidney disease.This study aimed to investigate the diagnostic value of PGRN and ILGFBP-2 in patients with lupus nephritis (LN) and the correlation of these biomarkers with disease activity and renal biopsy pathology. Patients and methods Patients with SLE (n=25) and chronic kidney disease (CKD) (n=25), and age- and sex-matched controls (n=25) were enrolled in the study. Serum PGRN and ILGFBP-2 levels were measured for each group. Results Disease duration was 4.78±4.26 years in the SLE patients. The mean SLE Disease Activity Index score was 15.04±7.54. All renal biopsy results were class 2, 3, and 5 with a percentage of 32, 24, and 44% respectively. PGRN and ILGFBP-2 were significantly higher in SLE patients (p<0.001 all) than in the CKD and control groups. All patients with high levels of biomarkers showed higher values of SLE disease activity. No significant difference was noted between active and inactive LN or classes of renal biopsy with PGRN and ILGFBP-2. Conclusion PGRN and ILGFBP-2 are significantly elevated in SLE compared to CKD and the general population and were associated with the SLE Disease Activity Index but not with active LN or classes of renal biopsy.

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Association of Intrarenal B-Cell Infiltrates with Clinical Outcome in Lupus Nephritis: A Study of 192 Cases

Clinical and Developmental Immunology ◽

10.1155/2012/967584 ◽

2012 ◽

Vol 2012 ◽

pp. 1-7 ◽

Cited By ~ 20

Author(s):

Yan Shen ◽

Chuan-Yin Sun ◽

Feng-Xia Wu ◽

Yi Chen ◽

Min Dai ◽

...

Keyword(s):

B Cells ◽

Lupus Nephritis ◽

B Cell ◽

Lupus Erythematosus ◽

Kidney Diseases ◽

Activity Index ◽

Disease Activity Index ◽

Immunological Responses ◽

Renal Biopsies ◽

Stage Renal Disease

Background. Lupus nephritis (LN) remains a major cause of morbidity and end-stage renal disease. Dysfunction of B lymphocytes is thought to be important in the pathogenesis of SLE/LN. Intrarenal B cells have been found in several forms of inflammatory kidney diseases although their role in LN renal is not well defined.Methods. Intrarenal B cells were analyzed in 192 renal biopsies from patients diagnosed with lupus nephritis. Immunohistochemical staining of serial sections was performed for each LN patient using CD20, CD3, and CD21 antibodies.Results. Intrarenal B cells were more likely to be associated with class IV LN and were mainly distributed in the renal interstitium, with very few in the glomerulus. The systemic lupus erythematosus disease activity index (SLEDAI), blood urea nitrogen, and serum creatinine levels were all significantly greater in the LN-B cell groups (allP<0.05). LN renal activity and chronicity indices correlated with B-cells infiltrates (allP<0.0001). Renal biopsies were classified into four distinct categories according to the organizational grade of inflammatory cell infiltrates. Germinal center- (GC-) like structures were not identified in any LN biopsies.Conclusion. It is hypothesized that intrarenal B cells enhance immunological responses and exaggerate the local immune response to persisting autoimmune damage in the tubulointerstitium.

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Intrarenal and Urinary Th9 and Th22 Cytokine Gene Expression in Lupus Nephritis

The Journal of Rheumatology ◽

10.3899/jrheum.140954 ◽

2015 ◽

Vol 42 (7) ◽

pp. 1150-1155 ◽

Cited By ~ 7

Author(s):

Cathy Choi-Wan Luk ◽

Lai-Shan Tam ◽

Bonnie Ching-Ha Kwan ◽

Priscilla Ching-Han Wong ◽

Terry King-Wing Ma ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Mrna Level ◽

Disease Activity Index ◽

Cytokine Gene Expression ◽

Mrna Levels ◽

Active Lupus Nephritis ◽

Histological Activity

Objective.We studied the urinary sediment mRNA level of Th9- and Th22-related cytokines in patients with systemic lupus erythematosus (SLE).Methods.We quantified urinary mRNA levels of interleukin (IL) 9, IL-10, IL-22, and their corresponding transcription factors in 73 patients with active lupus nephritis, 13 patients with hypertensive nephrosclerosis (HTN), and 25 healthy subjects.Results.There was no detectable IL-9 mRNA in all samples. Patients with proliferative lupus nephritis had significantly lower urinary IL-22 mRNA levels than those with nonproliferative nephritis (2.2 ± 5.4 vs 8.6 ± 20.0 copies, p = 0.019), and urinary IL-22 mRNA level inversely correlated with the histological activity index (r = −0.427, p < 0.0001). In contrast, patients with lupus nephritis had significantly higher urinary IL-10 mRNA levels than patients with HTN (7.8 ± 18.5 vs 1.9 ± 4.0 copies, p = 0.012), and urinary IL-10 mRNA levels correlated with its intrarenal mRNA levels (r = 0.337, p = 0.004) and SLE disease activity index (r = 0.277, p = 0.018). Urinary IL-10 mRNA level was significantly lower among patients who achieved complete remission than those with partial remission or no response (4.1 ± 6.5 vs 14.1 ± 28.0 copies, p = 0.036).Conclusion.Urinary IL-22 mRNA level is decreased in patients with SLE with proliferative nephritis, while urinary IL-10 mRNA levels correlates with its intrarenal mRNA level and disease activity. Urinary IL-10 mRNA levels may also predict treatment response. These results suggest that urinary mRNA levels of IL-10 and IL-22 might be used as biomarkers for assessing disease activity and risk stratification in lupus nephritis.

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Antineutrophil Cytoplasmic Antibody in Lupus Nephritis: Correlation with Clinicopathological Characteristics and Disease Activity

Current Rheumatology Reviews ◽

10.2174/1573397116999201208213422 ◽

2020 ◽

Vol 16 ◽

Author(s):

Dina Said ◽

Nearmeen Mohammed Rashad ◽

Nora Said Abdelrahmanc ◽

Ghada Aboelsaud Dawaa

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Rapidly Progressive Glomerulonephritis ◽

Disease Activity Index ◽

Antineutrophil Cytoplasmic Antibody ◽

Systemic Lupus ◽

Cross Sectional ◽

Class Iv

Background:: Lupus nephritis (LN) represents 40%–50% of all systemic lupus erythematosus (SLE) patients, and rapidly progressive glomerulonephritis is associated with significant morbidity and mortality. Antineutrophil cytoplasmic antibody (ANCA) might be involved in the pathogenesis of LN. Objective:: We evaluated the role of myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA, and anti-glomerular basement membrane autoantibodies (anti-GBM autoAb) for the diagnosis of LN. Methods:: In this cross-sectional study, 95 SLE patients were divided into 2 subgroups: LN group (n = 60) and non-LN group (n = 35). For further analysis, we subclassified the LN group into ANCA-positive (n = 16) and ANCA-negative (n = 44) LN patients. The entire Non-LN group was ANCA-negative. The SLE disease activity index (SLEDAI) was reported for each patient. Determination of MPO-ANCA, PR3-ANCA, and anti-GBM autoAb was performed using a novel multiplex bead-based technology in all patients. Data analyses were done using SPSS, version 20. Approval was obtained from the institutional review board of Zagazig University (ZU-IRB#6000). Results:: Of 95 patients with SLE, 16 patients (16.84%) had ANCA-positive LN, all of which were MPO-ANCA. There was a positive correlation between MPO-ANCA and SLEDAI, as well as with class IV LN. Receiver operating characteristic analyses revealed that the sensitivity and specificity of MPO-ANCA were 81.3% and 99.8%, respectively, in discriminating LN from systemic lupus without nephritis. Conclusion:: MPO-ANCA level was significantly correlated with SLEDAI, inflammatory markers, kidney function tests, and LN class IV.

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SLE Plasma Profiling Identifies Unique Signatures of Lupus Nephritis and Discoid Lupus

Scientific Reports ◽

10.1038/s41598-019-50231-y ◽

2019 ◽

Vol 9 (1) ◽

Cited By ~ 1

Author(s):

Michael A. Smith ◽

Jill Henault ◽

Jodi L. Karnell ◽

Melissa L. Parker ◽

Jeffrey M. Riggs ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Disease Activity Index ◽

Complement C3 ◽

Type I ◽

Tissue Remodelling ◽

Organ Systems ◽

Organ Specific

Abstract Systemic lupus erythematosus (SLE) impacts multiple organ systems, although the causes of many individual SLE pathologies are poorly understood. This study was designed to elucidate organ-specific inflammation by identifying proteins that correlate with SLE organ involvement and to evaluate established biomarkers of disease activity across a diverse patient cohort. Plasma proteins and autoantibodies were measured across seven SLE manifestations. Comparative analyses between pathologies and correlation with the SLE Disease Activity Index (SLEDAI) were used to identify proteins associated with organ-specific and composite disease activity. Established biomarkers of composite disease activity, SLE-associated antibodies, type I interferon (IFN), and complement C3, correlated with composite SLEDAI, but did not significantly associate with many individual SLE pathologies. Two clusters of proteins were associated with renal disease in lupus nephritis samples. One cluster included markers of infiltrating leukocytes and the second cluster included markers of tissue remodelling. In patients with discoid lupus, a distinct signature consisting of elevated immunoglobulin A autoantibodies and interleukin-23 was observed. Our findings indicate that proteins from blood samples can be used to identify protein signatures that are distinct from established SLE biomarkers and SLEDAI and could be used to conveniently monitor multiple inflammatory pathways present in different organ systems.

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Monocyte Chemoattractant-1 as a Urinary Biomarker for the Diagnosis of Activity of Lupus Nephritis in Brazilian Patients

The Journal of Rheumatology ◽

10.3899/jrheum.110201 ◽

2012 ◽

Vol 39 (10) ◽

pp. 1948-1954 ◽

Cited By ~ 30

Author(s):

RENATA FERREIRA ROSA ◽

KIOKO TAKEI ◽

NAFICE C. ARAÚJO ◽

SÔNIA M.A. LODUCA ◽

JOSÉ C.M. SZAJUBOK ◽

...

Keyword(s):

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Sandwich Elisa ◽

Disease Activity Index ◽

High Specificity ◽

Control Group ◽

Kidney Involvement ◽

Active Lupus Nephritis

Objective.Monocyte chemotactic protein (MCP-1), involved in the pathogenesis of lupus nephritis (LN), has recently been indicated as a new biomarker of kidney activity in systemic lupus erythematosus (SLE). Our aim was to assess urinary MCP-1 (uMCP-1) as a biomarker of renal activity in patients with SLE and to compare it to other disease activity markers, using the ELISA.Methods.Seventy-five female Brazilian patients with SLE and a control group participated in our study. Patients with SLE were distributed among 3 groups according to kidney involvement and classified according to disease activity based on clinical and laboratory measures such as urinary sediment, proteinuria, kidney function, C3, C4, anti-dsDNA, disease activity index, and renal SLE disease activity index. The serum and uMCP-1 concentrations were measured by sandwich ELISA.Results.In the A-LN group (active lupus nephritis: SLE with kidney involvement), the concentration of uMCP-1 was significantly higher than in other groups. A cutoff point was established using the results of the control group to apply this test in the detection of LN. A-LN had a higher frequency of positive results for uMCP-1 in comparison to the other groups (p < 0.001). To detect disease activity in patients with LN, a new cutoff was determined based on the results of patients with SLE with kidney involvement. Setting specificity at 90%, the sensitivity of the test was 50%.Conclusion.The high specificity makes uMCP-1 a useful test as a predictor of kidney activity in SLE, especially when associated to other measures used in clinical practice.

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Assessment of SLEDAI Score in Children with Lupus Nephritis Class III-IV in Vietnam National Children’s Hospital

VNU Journal of Science Medical and Pharmaceutical Sciences ◽

10.25073/2588-1132/vnumps.4238 ◽

2020 ◽

Vol 36 (2) ◽

Author(s):

Duong Thi Thanh Binh ◽

Nguyen Thu Huong ◽

Nguyen Thi Kien ◽

Pham Van Dem ◽

Tran Minh Dien

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Disease Activity Index ◽

Class Iii ◽

Systemic Lupus ◽

Sledai Score ◽

Pediatric Systemic Lupus Erythematosus

This study describes clinical, paraclinical characteristics and treatment response in children with nephritis class II-IV caused by systemic lupus erythematosus and validates SLEDAI for the evaluation of disease activity and the appropriate treatment strategy. A cross-sectional descriptive study was carried out on 40 children, 37 girls (92%) and 3 boys (8%), with an average age of 11.7 years with lupus nephritis class III- IV in Vietnam National Children’s Hospital in 2019. The study results show that the average score of SLEDAI in the children with pericardial and pleural effusions was 20.94 ± 4.09; high blood pressure, 20.89 ± 4.23; and gross hematuria, 20.29 ± 5.03, which were higher than those in children without these manifestations with p< 0.05. The most common kidney manifestations were nephrotic-range nephritis with renal failure (40%) and Glomerulonephritis (35%), corresponding to an average SLEDAI score of 24.25 ± 5.52 and 24.33 ± 3.2, respectively (p = 0.001). SLEDAI had an inverse correlation with the C3 complement value (r -0.315, p <0.05). The average SLEDAI score decreased gradually from 18.75 ± 4.22 to 3.38 ± 3.95 points (p <0.001) after 12 months of treatment. The study concludes that SLEDAI score was higher in patients with pleural and/or pericardial effusions, hypertension and gross hematuria, nephrotic-range nephritis with kidney failure or glomerulonephritis. SLEDAI score corresponded with the C3 complement value and the average SLEDAI score decreased gradually with treatment. Keywords: Lupus Nephritis class III- IV, SLEDAI. References [1] George Bertsias, Ricard Cervera và Dimitrios T Boumpas, Systemic Lupus Erythematosus: Pathogenesis and Clinical Features<sample chapter 20_mod 17_Systemic Lupus nephritis 2012.pdf> (2012), EULAR Textbook on Rheumatic Diseases, EULAR, 476-505.[2] D.M. Levy and S. Kamphuis, Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am59(2) (2012)345-64.[3] Thai Thien Nam, 2018, Lupus in National Children,s Hospital, [4] C.Bombardier, M.B. Hurwitz et al, Derivation of the SLEDAI: A disease activity index for lupus patients. The committee on prognosis studies in SLE, Arthritis Rheum 35(6) (1992) 630-640.[5] R. Shamim, S. Farman, S. Batool et al, Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus. Pak J Med Sci. 36(3) (2020) 467-472.[6] Le Thuy Hang, Assesment of SLEDAI score and panthology in children with lupus nephritis, 2016, Pediatrician thesis, Hanoi Medical University.[7] S.K.S.M. Nazri, K.K. Wong and W.Z.W.A. Hamid, Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score, Saudi Med J. 39(6) (2018) 627-631. [8] Nguyen Thuy Duong, clinical, paraclinical and pathology characteristics in children with nephritis caused by systemic lupus erythematosus, 2011, Master thesis, Hanoi Medical University.[9] S.N. Wong, W.K. Chan, J.Hui et al, Membranous lupus nephritis in Chinese children--a case series and review of the literature. Pediatr Nephrol, 24(10)(2009) 1989-1996.[10] N.T.N. Dung, H.T. Loan, S. Nielsen et al, Juvenile systemic lupus erythematosus onset patterns in Vietnamese children: a descriptive study of 45 children. Pediatric Rheumatology Online Journal, 10 (2010) 38-48.[11] T. Pusongchai, J. Jungthirapanich, S. Khositseth, Pediatric Systemic Lupus Erythematosus in Thammasat University Hospital, J Med Assoc Thai. 93(12) (2010) 283-290.

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