Principles of pediatric lupus nephritis in a prospective contemporary multi-center cohort

Lupus ◽  
2021 ◽  
pp. 096120332110286
Author(s):  
Kathleen M Vazzana ◽  
Ankana Daga ◽  
Beatrice Goilav ◽  
Ekemini A Ogbu ◽  
Daryl M Okamura ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Kidney Function ◽  
Lupus Erythematosus ◽  
Extensive Literature ◽  
Short Term ◽  
Renal Outcomes ◽  
Childhood Arthritis ◽  
Black Patients ◽  
End Stage

Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort. After an extensive literature review, six principles of LN in cSLE were identified. The CARRA registry was queried to evaluate these principles in determining the rate of LN in cSLE, median time from cSLE diagnosis to LN, short-term renal outcomes, and frequency of rituximab as an induction therapy. Of the 677 cSLE patients in the CARRA registry, 32% had documented LN. Decline in kidney function was more common in Black cSLE patients than non-Black patients ( p = 0.04). Black race was associated with worse short-term renal outcomes. In short-term follow up, most children with LN had unchanged or improved kidney function, and end stage kidney disease (ESKD) was rare. Ongoing follow-up of cSLE patients in the CARRA registry will be necessary to evaluate long-term outcomes to inform risk, management, and prognosis of LN in cSLE.

scholarly journals Initial renal histology and early response predict outcomes of Brazilian lupus nephritis patients

Lupus ◽  
2019 ◽  
Vol 29 (1) ◽  
pp. 83-91
Author(s):  
G Vajgel ◽  
C B L Oliveira ◽  
D M N Costa ◽  
M A G M Cavalcante ◽  
L M Valente ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Lupus Nephritis ◽  
Confidence Interval ◽  
Odds Ratio ◽  
Estimated Glomerular Filtration Rate ◽  
Interstitial Fibrosis ◽  
End Stage ◽  
Class Iv

Objective We analyzed baseline and follow-up characteristics related to poorer renal outcomes in a Brazilian cohort of admixture race patients with lupus nephritis. Methods Overall, 280 outpatients with a diagnosis of systemic lupus erythematosus and previous kidney biopsy of lupus nephritis were recruited from August 2015 to December 2018 and had baseline laboratory and histologic data retrospectively analyzed; patients were then followed-up and data were recorded. The main outcome measure was the estimated glomerular filtration rate at last follow-up. Secondary analyses assessed the impact of initial kidney histology and treatment in long-term kidney survival. Results Median duration of lupus nephritis was 60 months (interquartile range: 27–120); 40 (14.3%) patients presented progressive chronic kidney disease (estimated glomerular filtration rate <30 and ≥10 ml/min/1.73 m2) or end-stage kidney disease at last visit. Adjusted logistic regression analysis showed that class IV lupus nephritis (odds ratio 14.91; 95% confidence interval 1.77–125.99; p = 0.01) and interstitial fibrosis ≥25% at initial biopsy (odds ratio 5.87; 95% confidence interval 1.32–26.16; p = 0.02), lack of complete or partial response at 12 months (odds ratio 16.3; 95% confidence interval 3.74–71.43; p < 0.001), and a second renal flare (odds ratio 4.49; 95% confidence interval 1.10–18.44; p = 0.04) were predictors of progressive chronic kidney disease. In a Kaplan-Meier survival curve we found that class IV lupus nephritis and interstitial fibrosis ≥25% were significantly associated with end-stage kidney disease throughout follow-up (hazard ratio 2.96; 95% confidence interval 1.3–7.0; p = 0.036 and hazard ratio 4.96; 95% confidence interval 1.9–12.9; p < 0.0001, respectively). Conclusion In this large cohort of admixture race patients, class IV lupus nephritis and chronic interstitial damage at initial renal biopsy together with non-response after 1 year of therapy and relapse were associated with worse long-term renal outcomes.

scholarly journals The spectrum of C4d deposition in renal biopsies of lupus nephritis patients

2020 ◽  
Author(s):  
Ying DING ◽  
Xiaojuan YU ◽  
Lihua WU ◽  
Ying TAN ◽  
Zhen QU ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Disease Activity ◽  
Hazard Analysis ◽  
Complement Factor ◽  
Pathological Features ◽  
Peritubular Capillary ◽  
Renal Outcomes ◽  
Renal Biopsies ◽  
C4d Staining

Abstract Objectives This study aims to determine the prevalence and localization of complement factor C4d in renal biopsies of lupus nephritis (LN) patients, as well as its association with the clinico-pathological features of the disease. Especially, the correlation between arteriolar C4d deposition and renal microvascular lesions (RVL) was further analyzed. Methods A total of 325 biopsy-proven lupus nephritis patients were enrolled and their clinico-pathological data were collected. C4d staining in renal biopsies was performed by immunohistochemistry. The association between C4d deposition and the clinico-pathological features was further analyzed. Results C4d deposition was present in most of renal specimens (98.8%) in our cohort. They were localized in the glomeruli (98.2%), tubular basement membrane (TBM) (43.7%), arterioles (31.4%) and peritubular capillary (33.8%), respectively. TBM C4d staining was closely related to the disease activity (SLEDAI) and NIH pathological activity and chronicity indices (P < 0.01). Patients with arteriolar C4d deposition were more likely to develop RVL (91.2%) in comparison to those negative (78.0%; P = 0.004), especially with two or more types of RVL (P < 0.001). During an average follow-up of 55.8 months, the presence of arteriolar C4d was related to worse renal outcomes (HR: 2.074, 95% CI 1.056–4.075, P = 0.034). Co-deposition of arteriolar C4d and C3c was an independent risk factor (HR: 2.539, 95% CI 1.130–5.705, P = 0.024) for predicting renal outcomes by the multivariate stepwise Cox hazard analysis Conclusions C4d deposition was common in renal tissues of lupus nephritis patients. TBM C4d deposition was related to the disease activity and arteriolar C4d deposition was associated with RVL and worse renal outcomes.

scholarly journals Clinical Outcomes of Patients With Primary Membranous Nephropathy and Subnephrotic Proteinuria

2021 ◽  
Vol 8 ◽  
Author(s):  
Peng He ◽  
Yang Zha ◽  
Jing Liu ◽  
Hanmin Wang ◽  
Lijie He
Keyword(s):  
Kidney Function ◽  
Membranous Nephropathy ◽  
Independent Predictor ◽  
Effective Management ◽  
Nephrotic Proteinuria ◽  
Hazard Ratios ◽  
Increased Risk ◽  
Course Outcomes ◽  
End Stage

Objectives: To update the information about the prognosis of patients with primary membranous nephropathy (MN) and subnephrotic proteinuria and identify the relevant predictors.Methods: In total, 474 cases of biopsy-proven primary MN with at least 18 months of follow-up were reviewed to determine the outcomes of the subgroup of patients that presented with subnephrotic proteinuria. Clinical data included initial proteinuria and microhematuria, defined as the average proteinuria/microhematuria of the first 6 months during the course. Outcomes included partial remission (PR), complete remission (CR), nephrotic proteinuria progression, and kidney function progression, defined as ≥50% loss of kidney function or end-stage kidney disease.Results: In total, 205 patients with primary MN and subnephrotic proteinuria at biopsy were eligible. During a median follow-up of 43 months, 200 (97.56%), 167 (81.46%), and 53 (25.85%) patients attained PR, CR, and nephrotic proteinuria progression, respectively. Only one patient (0.49%) progressed to the kidney function progression. By multivariate Cox hazards regression analyses, the initial proteinuria was identified as the independent predictor for PR, CR, and nephrotic proteinuria progression with adjusted hazard ratios (aHRs) of 0.67 (95% confidence interval, 0.56–0.80), 0.50 (95% CI, 0.40–0.63), and 2.97 (95% CI, 2.23–3.97), respectively. A higher level of initial microhematuria was also associated with an increased risk of nephrotic proteinuria progression. The corresponding aHR was 1.11 (95% CI, 1.05–1.17).Conclusion: Among patients with primary MN and subnephrotic proteinuria, although the overall prognosis is excellent, dynamic detection and effective management of proteinuria remain important. In addition, initial microhematuria may be another predictor of nephrotic proteinuria progression.

scholarly journals Change of Renal Gallium Uptake Correlated with Change of Inflammation Activity in Renal Pathology in Lupus Nephritis Patients

2021 ◽  
Vol 10 (20) ◽  
pp. 4654
Author(s):  
Tsu-Yi Hsieh ◽  
Yi-Ching Lin ◽  
Wei-Ting Hung ◽  
Yi-Ming Chen ◽  
Mei-Chin Wen ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Left Kidney ◽  
Renal Pathology ◽  
Class Iii ◽  
Stage Renal Disease ◽  
End Stage ◽  
Active Inflammation ◽  
Histological Results

Background: Lupus nephritis (LN) often lead to end-stage renal disease in systemic lupus erythematosus patients. This study aimed to investigate the clinical application of renal gallium-67 scans for determining renal histological parameters in LN patients. Methods: Between 2006 and 2018, 237 biopsy-proven and 35 repeat biopsies LN patients who underwent renal gallium scans before or after biopsy were included for analysis. The classification and scoring of LN were assessed according to the International Society of Nephrology/Renal Pathology Society. A delayed 48-h gallium scan was performed and interpreted by semiquantitative methods using left kidney/spine (K/S) ratio. The renal histological results were compared with gallium uptake. Results: Out of 237 participants, 180 (76%) had proliferative LN. Baseline gallium left K/S ratio was significantly higher in class IV LN as compared to class III (median (interquartile range, IQR): 1.16 (1.0–1.3), 0.95 (0.9–1.1), respectively, p < 0.001). Furthermore, changes in gallium uptake between two biopsies were positively correlated with changes activity index (r = 0.357, p = 0.035), endocapillary hypercellularity (r = 0.385, p = 0.032), and neutrophils infiltration (r = 0.390, p = 0.030) in renal pathology. Conclusions: Renal gallium uptake is associated with active inflammation in LN. Changes in renal gallium uptake positively correlated with changes in activity index in renal pathology.

scholarly journals P0442RELATED FACTORS IN THE DEVELOPMENT OF LUPUS NEPHRITIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Andres Ribas ◽  
Isabel Galcerán ◽  
Sara Outón ◽  
Tarek Salman ◽  
Clara Barrios ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Protective Factor ◽  
Analytical Data ◽  
Class Iii ◽  
Systemic Lupus ◽  
Complement Factors ◽  
Patients At Risk

Abstract Background and Aims Lupus Nephritis (LN) is a severe complication of Systemic Lupus Erytemathosus (SLE). This is the main reason why identifying predisposing factors to differentiate patients at risk of developing LN is so important. Method Retrospective study of patients with SLE diagnosed between years 2008-2018 in our center. Demographic, clinical and analytical data have been collected. Results We included 171 patients, 48 (28%) with diagnose of LN. Age at diagnose of SLE was 39,51 ± 15,40 years, being more frequent in women 151 (87,5%). Time of follow-up since SLE diagnose until development of LN was of 3 ± 5, 3 years. Respectful to the LN classification we found: 4 (8%) class I LN, 6 (12.5%) class II LN, 15 (31.2%) class III LN, 19 (39.5%) class IV LN and 4 (8%) class V LN. At diagnose of SLE, the following variables, where related to developing LN: CH50 [HR: 1,039; CI (95%): 1,004-1,064; p=0,024], C3 [HR: 1,029; CI (95%): 1,016-1,042; p&lt;0,001, titer of Anti- DNACrithidia [HR: 4,364; CI(95%): 1,26-15,064; p=0,02], AntiSM [HR: 4,634, CI (95%) 1,76-12,17, p=0,002], ACA IgG [HR: 7,5; CI (95%): 2,3 -24,449; p=0,001] and Lupus anticoagulant [HR: 4,97; CI (95%): 1,591-15,533; p=0,006]. Treatment with hidroxicloroquine is a protective factor against developing LN [HR: 0,17; CI (95%): 0,063-0,511; p=0.001]. At diagnose of LN, complement factors and titer of anti-DNA crithidia show a positive correlation when compared to the initial determinations: C3 [r= 0,605 (p&lt;0,001]); C1q [r= 0,861 (p=0,006)]; CH50 [r= 0,981 (p&lt;0,001), anti- DNACrithidia [r= 0,529 (p&lt;0,001)], anti-Sm [r=0.8, )p=0.001)]. Conclusion Consumption of complement factors, high titers of anti-DNAcrithidia, Anti-SM, ACA IgG and Lupus anticoagulant are related to a future LN development at SLE diagnose. Moreover, we see an increase of their titer once we diagnose LN. Otherwise, treatment with hidroxicloroquine seems to be a protective factor.

Idiopathic membranous nephropathy preceding membranous lupus nephritis: a case report

Lupus ◽  
2020 ◽  
Vol 29 (3) ◽  
pp. 340-343
Author(s):  
C Gökalp ◽  
G Aygun ◽  
A F Dogan ◽  
U Usta ◽  
I Kurultak ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Membranous Nephropathy ◽  
Adult Population ◽  
Idiopathic Membranous Nephropathy ◽  
Systemic Lupus ◽  
Common Causes ◽  
Membranous Lupus Nephritis

Membranous nephropathy is one of the most common causes of nephrotic syndrome in the adult population. According to the underlying etiology, membranous nephropathy is classified as either primary or secondary. Systemic lupus erythematosus is an autoimmune disease that can affect the kidneys in 50% of patients in the course of the disease. Renal disease may be the first manifestation of systemic lupus erythematosus and the development of systemic findings may be delayed for about 1–5 years following the diagnosis of lupus nephritis. We present a 59-year-old male patient who had a diagnosis of idiopathic membranous nephropathy since 2007 and developed membranous lupus nephritis during the 12-year follow-up without any extrarenal systemic lupus erythematosus findings.

Therapeutic potential of allogeneic mesenchymal stromal cells transplantation for lupus nephritis

Lupus ◽  
2018 ◽  
Vol 27 (13) ◽  
pp. 2161-2165 ◽  
Author(s):  
J Barbado ◽  
S Tabera ◽  
A Sánchez ◽  
J García-Sancho
Keyword(s):  
Lupus Nephritis ◽  
Mesenchymal Stromal Cells ◽  
Lupus Erythematosus ◽  
Stromal Cells ◽  
Therapeutic Potential ◽  
Activity Index ◽  
Disease Activity Index ◽  
Controlled Clinical Trial ◽  
Allogeneic Mscs

Animal and human studies have suggested the potential of mesenchymal stromal cells (MSCs) to treat systemic lupus erythematosus (SLE). Here, we present the results of compassionate MSC treatments for three SLE patients to provide the proof of concept for a randomized and controlled clinical trial. Three patients of different ethnicities who suffer from chronic SLE, and who presented with class IV active proliferative nephritis confirmed by biopsy, were treated with allogeneic MSCs from healthy donors. Ninety million cells were infused intravenously into each patient during high and very high activity disease flare-ups and follow-up was continued for 9 months. Multi-organic affectation was quantified by the SLE disease activity index (SLEDAI), and indicators of lupus nephritis activity, such as proteinuria, as well as lymphocyte and monocyte antigens and anti-HLA antibodies were measured at 1, 3, 6, and 9 months after treatment. Proteinuria levels improved dramatically during the 1st month after treatment and the ameliorations were sustained throughout the follow-up period. SLEDAI scores revealed early, durable, and substantial remissions that were complete for two patients and partial for the third patient and that permitted medication doses to be reduced 50–90%. These favourable outcomes support completion of the randomized and controlled MSC trial for SLE.

Natural history of longitudinally extensive transverse myelitis in 35 Hispanic patients with systemic lupus erythematosus: good short-term functional outcome and paradoxical increase in long-term mortality

Lupus ◽  
2018 ◽  
Vol 27 (8) ◽  
pp. 1279-1286 ◽  
Author(s):  
F D Flores-Silva ◽  
O Longoria-Lozano ◽  
D Aguirre-Villarreal ◽  
H Sentíes-Madrid ◽  
F Vega-Boada ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Functional Outcome ◽  
Lupus Erythematosus ◽  
Transverse Myelitis ◽  
Systemic Lupus ◽  
Short Term ◽  
Term Outcome ◽  
Short Term Outcome

Background and objective Acute transverse myelitis (TM) is an infrequent neurological complication of systemic lupus erythematosus (SLE). Short-term outcome varies widely between cohorts. Little is known about the epidemiology and long-term functional outcome of TM associated to SLE. Methods Patients with SLE and acute TM were identified during hospital admission, visits to the Emergency Room or the Neurology Outpatient Clinic. We evaluated ambispectively those patients with SLE presenting with clinical myelopathy and corroborated with spinal MRI. Cases were divided as partial (non-paralyzing) or complete (paralyzing). We determined long-term functional outcome as well as mortality in those patients with follow-up periods of at least five years. Results We identified 35 patients (partial, n = 15; complete, n = 20) in which complete clinical and imaging data were available (26 with follow-up ≥ 5 years). Patients with complete TM were significantly older than those with partial forms. Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role. Surprisingly, functional recovery at one year was in general good; however, we observed a five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case). Conclusions Short-term outcome of SLE-related TM is generally good, and recurrence rate is low. However, we observed a long-term fatality rate of 31% for reasons unrelated to TM, suggesting that TM is a manifestation of severe immune dysregulation and a predictor of severity and mortality in patients with SLE.

scholarly journals Clinical And Morphological Improvement Of Lupus Nephritis Treated With Rituximab

Folia Medica ◽  
2014 ◽  
Vol 56 (4) ◽  
pp. 245-252 ◽  
Author(s):  
Maria E. Tsanyan ◽  
Sergey K. Soloviev ◽  
Stefka G. Radenska-Lopovok ◽  
Anna V. Torgashina ◽  
Ekaterina V. Nikolaeva ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Cell Therapy ◽  
Disease Activity ◽  
B Cell ◽  
Renal Biopsy ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Renal Biopsies

Abstract Aim: TO assess the effects of rituximab (RTM) therapy on clinical and morphologic activity of lupus nephritis (LN). Material and methods: The study included 45 patients with confirmed diagnosis of systemic lupus erythematosus (SLE), unaffected by previously received standard therapy with glucocorticoids (GCs) and cytostatics. The disease activity was assessed using Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K); to assess the LN activity we used the SLICC RA/RE index. Forty-five patients with LN were given puncture renal biopsy prior to prescribing RTM; 16 patients had repeated renal biopsy 1 year and more after beginning the anti-B-cell therapy. LN was graded histologically in accordance with the WHO classification (2003) with indices of activity (AI) and chronicity (CI). Results: The predominant number of patients had class III - IV of LN. The repeated renal biopsies demonstrated that LN had undergone a transition into a more favourable morphologic class, which was associated, in most of these cases, with a positive therapeutic effect. The follow-up dynamics showed a statistically significant reduction of AI (p=0.006), and no statistically significant changes in the CI (p = 0.14). Conclusion: The long-term follow-up in the study has showed that repeated courses of anti-B-cell therapy with RTM have a positive effect both on SLE activity and generally on the renal process. The reduction of the morphologic class of LN as assessed in the repeated renal biopsies is a convincing proof for this. Eleven out of 16 patients experienced transition of the morphologic class into a more favourable type, which in most cases was combined with lower AI (p = 0.006). We found no evidence of increase in the CI (p = 0.14).

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