Pathology Society Classification Research Articles

Using photovoice to investigate patient experiences of lupus nephritis in Canada

ObjectiveLupus nephritis (LN) is a major cause of morbidity and mortality, affecting up to 60% of patients with systemic lupus erythematosus (SLE). The perspectives of patients with SLE have been...

Histopathologic Remission of Severe Diabetic Nephropathy of Donor Kidney After Kidney Transplantation: A Case Report

Although diabetes is not an absolute contraindication for kidney donation, donor kidneys with severe diabetic nephropathy (DN) are frequently discarded. Current experience with the use of donor kidneys with severe DN is limited. Herein, we report a case of deceased donor kidney with Renal Pathology Society (RPS) classification scheme class III DN transplanted to a female recipient without post-operative hyperglycemia. Although kidney allograft biopsy still showed class III DN at 6 months, it turned to RPS class IIa DN combined with chronic antibody-mediated rejection at 4.5 years and 9 years. At the last follow-up approximately 10 years after transplantation, the patient's serum creatinine was 2.27 mg/dl. Our case indicates that with careful selection of potential recipients, favorable clinical outcomes can be achieved even with donor kidneys with severe DN. Thus, RPS class III diabetic donor kidneys deserve further evaluation and should not always be discarded.

Association of Autoantibody Concentrations and Trajectories With Lupus Nephritis Histologic Features and Treatment Response.

Autoantibodies are a hallmark of lupus nephritis (LN), but their association with LN classes and treatment response are not adequately known. In this study, we quantified circulating autoantibodies in the Accelerating Medicines Partnership LN longitudinal cohort to identify serological biomarkers of LN histologic classification and treatment response and how these biomarkers change over time based on treatment response. Peripheral blood samples were collected from 279 patients with systemic lupus erythematosus undergoing diagnostic kidney biopsy based on proteinuria. Of these, 268 were diagnosed with LN. Thirteen autoantibody specificities were measured by bead-based assays (Bio-Rad Bioplex 2200) and anti-C1q by enzyme-linked immunosorbent assay at the time of biopsy (baseline) and at 3, 6, and 12 months after biopsy. Clinical response was determined at 12 months. Proliferative LN (International Society of Nephrology/Renal Pathology Society class III/IV±V, n = 160) was associated with higher concentrations of anti-C1q, anti-chromatin, anti-double-stranded DNA (dsDNA), and anti-ribosomal P autoantibodies compared to nonproliferative LN (classes I/II/V/VI, n = 108). Anti-C1q and-dsDNA were independently associated with proliferative LN. In proliferative LN, higher baseline anti-C1q levels predicted complete response (area under the curve [AUC] 0.72; P = 0.002) better than baseline proteinuria (AUC 0.59; P = 0.21). Furthermore, all autoantibody levels except for anti-La/SSB decreased over 12 months in patients with proliferative, but not membranous, LN with a complete response. Baseline levels of anti-C1q and anti-dsDNA may serve as noninvasive biomarkers of proliferative LN, and anti-C1q may predict complete response at the time of kidney biopsy. In addition, tracking autoantibodies over time may provide further insights into treatment response and pathogenic mechanisms in patients with proliferative LN.

Canadian patient experiences of lupus nephritis: a qualitative analysis

ObjectiveLupus nephritis (LN) is one of the most severe manifestations of SLE; however, we know little about the lived experience of LN. This research investigates patient experiences and perspectives of...

Polar vasculosis is associated with better kidney outcome in type 2 diabetes with biopsy-proven diabetic kidney disease: A multicenter cohort study.

This multicenter cohort study retrospectively assessed the association between polar vasculosis and the progression of diabetic kidney disease (DKD) in type 2 diabetes. We enrolled 811 patients with type 2 diabetes, biopsy-proven DKD, and proteinuria (≥0.15 g/g creatinine [g/day]). The association between polar vasculosis and other kidney lesions was explored. The outcome was DKD progression defined as a composite of renal replacement therapy initiation or 50% decline in estimated glomerular filtration rate (eGFR) from baseline. Of the 811 cases, 677 (83.5%) had polar vasculosis. In multivariate logistic regression analysis, subendothelial widening of the glomerular basement membrane, glomerulomegaly, glomerular class in the Renal Pathology Society classification ≥IIb, vascular lesions, age, eGFR, and hemoglobin A1c were positively associated with polar vasculosis, whereas interstitial fibrosis and tubular atrophy (IFTA) was negatively associated with polar vasculosis. During a median follow-up of 5.2 years, progression of DKD occurred in 322 of 677 (7.4 events/100 person-years) and 79 of 134 (11.4 events/100 person-years) cases with and without polar vasculosis, respectively. Kaplan-Meier analysis showed that polar vasculosis was associated with lower cumulative incidences of DKD progression. Multivariate Cox regression analyses showed that polar vasculosis was associated with a lower risk of DKD progression, regardless of eGFR or proteinuria subgroups. These associations between polar vasculosis and better kidney outcome were unchanged considering all-cause mortality before DKD progression as a competing event. This study showed that polar vasculosis of DKD was associated with less advanced IFTA and a better kidney outcome in type 2 diabetes with proteinuria.

Investigation of Lupus Nephritis Cases with Special Reference to Activity and Chronicity Indices

Background & Objectives: The International Society of Nephrology/ Renal Pathology Society (ISN/RPS) classification is based upon criteria which differentiate acute and chronic phases of Lupus Nephritis. Activity/Chronicity Index grants us a higher insight on the level of pathological lesions and treatment outcome by examining the reversibility of lesions .The present investigation was done in order to highlight the importance of activity and chronicity indices in the course of Lupus Nephritis.Materials & Methods: Seventy-three Kidney biopsy samples of Lupus Nephritis patients were examined. The information was recorded in a check-list and was then statistically analyzed.Results: Lupus Nephritis is importantly age-related, frequency of Lupus Nephritis increases with age until the age of 40 years; 21–40 years being the most frequent among studied patients. A prominent decline was seen after the age of 40. Regardless of age, the occurrence was more frequent in females. Class IV was most frequent in all ages and in both sexes. Both Activity and Chronicity scores were slightly higher in females. Activity Index was higher in ages of 11 to 30 years, whereas Chronicity Index was highest in 41 to 50 years of age. The highest Activity Index was reported in Class IV while the highest Chronicity Index was reported in Class V. The least Activity Index was shown in Class VI while Classes II and I had the lowest Chronicity Index. Endocapillary hypercellularity was the most frequent active lesion and tubular atrophy was the most frequent chronic lesion. It is noteworthy that fibrotic crescents were significantly less common among chronic lesions.Conclusion: Histopathological findings, clinical and para-clinical data could furnish more information on disease process, treatment, quality of life and mortality rate.

Mitochondrial Dynamics of Podocyte in Various International Society of Nephrology/Renal Pathology Society Class Lupus Nephritis Patients.

Morphological changes of podocyte mitochondria are observed in patients with mitochondrial cytopathy and nephrotic syndrome. However, whether mitochondrial dynamics involved in podocyte in lupus nephritis (LN) is still not clear. This study aims to investigate the associations between mitochondrial morphology and podocyte lesions and laboratory and pathological features in LN. The foot process width (FPW) and mitochondrial morphology were observed through electron microscope. Then the associations between mitochondrial morphology and podocyte lesions and laboratory features were explored in various International Society of Nephrology/Renal Pathology Society class LN patients. Foot process effacement and excessive mitochondria fission in podocyte were observed and proteinuria was positively correlated with FPW. Mitochondria area, circumference, and aspect ratio were negatively correlated with BUN, and 24h-UTP positively correlated with Alb. At the same time, Alb was negatively correlated with form factor. FPW, form factor, surface density, and numerical density on area were positively correlated with 24h-UTP. Excessive mitochondrial fission is associated with podocyte damage and proteinuria, whereas the mechanism still needs to be explored.

Metabolic phenotypes and risk of end-stage kidney disease in patients with type 2 diabetes.

Obesity often initiates or coexists with metabolic abnormalities. This study aimed to investigate the pathological characteristics and the independent or mutual relations of obesity and metabolic abnormalities with end-stage kidney disease (ESKD) in patients with type 2 diabetes (T2D) and associated diabetic kidney disease (DKD). A total of 495 Chinese patients with T2D and biopsy-confirmed DKD between 2003 and 2020 were enrolled in this retrospective study. The metabolic phenotypes were based on the body weight index (BMI)-based categories (obesity, BMI ≥ 25.0 kg/m2) and metabolic status (metabolically unhealthy status, ≥ 1 criterion National Cholesterol Education Program Adult Treatment Panel III (NCEP/ATP III) excluding waist circumference and hyperglycemia) and were categorized into four types: metabolically healthy non-obesity (MHNO), metabolically healthy obesity (MHO), metabolically unhealthy non-obesity (MUNO), and metabolically unhealthy obesity (MUO). The pathological findings were defined by the Renal Pathology Society classification. Cox proportional hazards models were used to estimate hazard ratios (HRs) for ESKD. There are 56 (11.3%) MHNO patients, 28 (5.7%) MHO patients, 176 (35.6%) MUNO patients, and 235 (47.5%) MUO patients. The high prevalence of the Kimmelstiel-Wilson nodule and severe mesangial expansion were associated with obesity, whereas severe IFTA was related to metabolically unhealthy status. In the multivariate analysis, the adjusted HR (aHR) was 2.09 [95% confidence interval (CI) 0.99-4.88] in the MHO group, 2.16 (95% CI 1.20-3.88) in the MUNO group, and 2.31 (95% CI 1.27-4.20) in the MUO group compared with the MHNO group. Furthermore, the presence of obesity was insignificantly associated with ESKD compared with non-obese patients (aHR 1.22, 95% CI 0.88-1.68), while the metabolically unhealthy status was significantly associated with ESKD compared to the metabolically healthy status in the multivariate analysis (aHR 1.69, 95% CI 1.10-2.60). Obesity itself was insignificantly associated with ESKD; however, adding a metabolically unhealthy status to obesity increased the risk for progression to ESKD in T2D and biopsy-proven DKD.

Renal Histopathology Associated With Kidney Failure and Mortality in Patients With Lupus Nephritis: A Long-Term Real-World Data Study.

Lupus nephritis (LN), a common manifestation of systemic lupus erythematosus, is associated with a higher risk of kidney failure and death. The renal pathology of LN helps elucidate the severity of inflammation and the extent of irreversible damage. We aimed to identify histologic variables that correlate with risks of kidney failure and mortality. Between 2006 and 2019, a total of 526 patients with LN were enrolled. Renal pathology was classified according to the International Society of Nephrology/Renal Pathology Society classification. Components of activity and chronicity indices were analyzed to determine which variables correlated with an increased risk of kidney failure and death, with the adjustment of potential confounders. During the follow-up period (median 7.5, IQR 3.5-10.7 years), 58 patients progressed to kidney failure and 64 died. In the multivariate Cox regression analysis, tubular atrophy (hazard ratio [HR] 2.28, 95% CI 1.66-3.14) and tubulointerstitial inflammation (HR 3.13, 95% CI 1.34-7.33) predicted kidney failure. The renal outcome was even worse if tubular atrophy and tubulointerstitial inflammation coexisted (10-year kidney survival rate: 63.22%). The presence of cellular crescents was associated with an increased risk of death in male patients with LN (HR 1.91, 95% CI 1.02-3.57), whereas the presence of fibrous crescents predicted death in female patients with LN (HR 5.70, 95% CI 1.61-20.25). Histologic variables of renal biopsy in LN could be regarded as prognostic indicators for kidney failure and mortality.

Histologic evaluation of activity and chronicity of lupus nephritis and its clinical significance.

The National Institutes of Health (NIH) lupus nephritis activity and chronicity indices, which comprise six activity scores and four chronicity scores, have a long development history. The 2018 revised International Society of Nephrology/Renal Pathology Society classification for lupus nephritis adopted the most recent NIH indices to replace subclasses A, C, and A/C. Although an evidence-based approach should further evaluate the clinical significance of the modified NIH indices, recent validation studies demonstrated that the modified chronicity indices have a strong correlation with kidney outcome of lupus nephritis.

Pathological validation of the Japanese Renal Pathology Society classification and challenges in predicting renal prognosis in patients with diabetic nephropathy.

BackgroundDiabetic nephropathy (DN) accounts for approximately half of all cases of chronic kidney disease (CKD) and end-stage kidney disease worldwide. The Renal Pathology Society (RPS) classification has been used to predict the renal prognosis in DN. In 2018, the Japanese Renal Pathology Society (JRPS) proposed a comprehensive classification system that included pathological changes in the kidney. The clinical significance of the JRPS classification system was comparatively evaluated in the present study.MethodsA total of 93 cases diagnosed with DN from 2009 to 2019 were enrolled. JRPS scores (J-scores) were calculated by scoring the pathological factors in the JRPS classification system and comparing them with clinical parameters.ResultsMost pathological factors constituting the J-score were significantly correlated with clinical factors. Laminated nodules were inversely correlated with estimated glomerular filtration rate. After adjusting for age, sex, body mass index, hemoglobin A1c, diabetes duration, and hypertension, CKD stage was significantly correlated with JRPS grade, nodular lesions, and exudative lesions in the multivariate logistic regression analysis. However, receiver operating characteristic curve analysis revealed that the J-score (area under the curve [AUC] = 0.639) had lower clinical significance than the traditional RPS classification system (AUC = 0.675).ConclusionThe JRPS classification can more comprehensively reflect renal changes than the RPS classification and is correlated with renal survival. When creating a new pathological classification, arteriolar hyalinosis should not be included, whereas laminated nodules should be included.

Tubulointerstitial nephritis in cases of lupus nephritis: An experience from a tertiary care referral center of Eastern India

Context: Up to 60% of patients with systemic lupus erythematosus develop lupus nephritis (LN). Tubulointerstitial nephritis (TIN) includes interstitial inflammation, tubulitis, tubular atrophy (TA), and interstitial fibrosis. These are considered independent risk factors for renal outcome. Aims: To evaluate the occurrence of TIN in LN and to correlate it with clinical and histopathological variables and renal outcome. Settings and Design: It was a prospective, single-center study. Subjects and Methods: One hundred and thirty-two LN cases were evaluated. Light microscopic scoring of interstitial inflammation, fibrosis, and TA was done as follows: 0 (nil); 1+ (mild - <25% of the area of observed cortex); 2+ (moderate - >25% to 50% of the area of observed cortex); 3+ (severe - >50% of the area of observed cortex). For direct immunofluorescence study, fluorescein isothiocyanate-conjugated polyclonal rabbit antisera against human IgG, IgA, IgM, C3c, C1q, kappa, and lambda antibodies (DAKO, Germany) were used. Statistical Analysis Used: Statistical software GraphPad Prism version 6.1. Results: Significant TIN was present in 6% of cases associated with high National Institutes of Health activity and chronicity indices irrespective of the modified International Society of Nephrology and Renal Pathology Society class of LN. In the cases where inflammation and fibrosis are marked, significantly raised serum creatinine, low estimated glomerular filtration rate, high 24-h urinary protein excretion, and reduced survival without any complete remission were seen. Severe interstitial and tubular inflammations without chronicity were also associated with low survival rate due to frequent relapse and significant hypertension. Conclusions: Activity and chronicity indices describing TIN components become essential to predict the survival, therapeutic response, and disease prognosis in LN.

Dissecting the histological features of lupus nephritis highlights new common patterns of injury in class III/IV

ObjectiveThe International Society of Nephrology/Renal Pathology Society classification is the gold standard for the characterisation of lupus nephritis (LN) on renal biopsy, with therapeutic repercussions. Its recent revision simplified the...

Interstitial Inflammation in the ISN/RPS 2018 Classification of Lupus Nephritis Predicts Renal Outcomes and is Associated With Bcl-2 Expression.

To investigate the histopathological characteristics of patients with lupus nephritis in the 2018 revised International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification and assess the prognostic factors. This study enrolled 92 patients with lupus nephritis, who had conventional treatment and renal biopsy. Each renal tissue was evaluated according to 2018 ISN/RPS classification, and quantified apoptotic regulator protein, the B-cell lymphoma-2 protein (Bcl-2), expressions in selected lymphocyte subsets were measured using novel computational approaches using multicolor confocal images. Histopathological characteristics and prognostic factors of end-stage renal disease (ESRD) and chronic kidney disease (CKD) were compared. Follow-up data were obtained, and survival analysis was conducted. During follow-up period (average 74.3 months), 16 and 18 patients progressed ESRD and CKD, respectively. Multivariable analysis of age, sex, disease activity and pathological features in ISN/RPS demonstrated the extent of interstitial inflammation (grade 0~3) was significantly associated with both ESRD and CKD. When interstitial inflammation was divided into mild (grade 0, 1) and severe (grade 2, 3), Cox regression analysis showed that patients with severe interstitial inflammation were significantly increased risk of both ESRD and CKD (hazard ratio 4.67 and 3.8, respectively). Bcl-2 expression in CD4+ and CD20 cells was significantly higher in the severe interstitial inflammation group compared to in mild interstitial inflammation patients (p=0.006 and 0.010, respectively). The extent of interstitial inflammation can predict clinical renal outcomes. Significantly elevated Bcl-2 expression in both CD4+ and CD20 cells was found in severe interstitial inflammation compared with mild interstitial inflammation.

Complement Deposition Predicts Worsening Kidney Function and Underlines the Clinical Significance of the 2010 Renal Pathology Society Classification of Diabetic Nephropathy.

ObjectivesConverging evidence points towards a role of the complement system in the pathogenesis of diabetic nephropathy (DN). The classification system of diabetic kidney lesions devised by the Renal Pathology Society (RPS) in 2010 are based on the pathogenic process of DN. Therefore, we investigated the correlation between glomerular C3 deposits and RPS DN classification and the combined deleterious effects thereof on kidney function.MethodsThe study analyzed data from 217 diabetic patients who underwent renal biopsy between 2010 and 2021 and were found to have DN as the only glomerular disease. C3 deposition was considered positive if the glomerular C3 immunofluorescence intensity was at the trace or ≥1+ level. We divided DN into five glomerular lesion classes and separately evaluated the degree of interstitial and vascular involvement. The primary outcome was the composite of a ≥50% decline from the initial estimated glomerular filtration rate, end-stage renal disease, and death.ResultsNone of the patients were classified into class I, and few were classified into classes IIa (7.8%) and IV (9.2%). Most patients were classified as IIb (30.9%) and III (52.1%). C3 deposition was detected in 53.9% of patients. Multivariate logistic regression analysis showed that DN class was significantly correlated with C3 deposits [odds ratio, 1.59; 95% confidence interval (CI), 1.08–2.36; p = 0.02). During a median follow-up of 22 months, 123 (56.7%) patients reached the composite outcome. The endpoints occurred more frequently in patients with C3 deposition (69.2 vs. 42%) compared with those without C3 deposition. Patients with C3 deposition in either class IIb [hazards ratio (HR), 3.9 (95% CI, 1.14–13.17) vs. 2.46 (95% CI, 0.68–8.89)] or III [HR, 4.98 (95% CI, 1.53–16.23) vs. 2.63 (95% CI, 0.77–9.0)] had a higher risk of adverse kidney outcomes than those without C3 deposition. The prognostic accuracy of the combination of DN class and C3 deposits at 1 and 3 years was higher than that for DN class only.ConclusionsComplement deposition together with DN class predicts more rapid deterioration of kidney function in DN, which underlines the clinical significance of the DN phenotype according to the RPS classification.

IDF21-0222 Prevalence and clinical predictors of Non-diabetic kidney disease in Type 2 Diabetes with renal involvement

Background: Renal Involvement in individuals with Type-2 Diabetes (T2DM) may be due to diabetes per se i.e., Diabetes kidney disease (DKD) or to other cause other than T2DM i.e., non-Diabetic kidney disease (NDKD) and in a group of individuals may have both (DKD+NDKD). Differentiation between the same is only possible with renal biopsy. Specific treatment options are available for NDKD which could potentially change outcomes. Data regarding the proportion of subtypes of renal involvement in diabetes is limited and flawed due to biases in performing renal biopsy. Most previous studies of renal biopsies are retrospective in nature. With increasing burden of T2DM worldwide it is imperative that we to establish non-invasive markers which could help to identify those who might benefit from renal biopsy to establish diagnosis of NDKD. Aim: To determine proportion of patients with T2D with renal involvement who have DKD, NDKD or mixed disease. To identify possible clinical predictor/s which could help decision making for targeted renal biopsy to identify those with NDKD component (NDKD and mixed group). Method: We recruited consecutive patient with renal involvement (eGFR >30ml/min/m2 and/or urinary albumin: creatinine excretion >/=300mg/g. Clinical history, demography of patients, Lipid profile, Liver function test, electrolytes, serum creatinine, urea, Albumin, globulin, Uric acid, 24hr urinary protein (by autoanalyzer), Glycated hemoglobin (HbA1C) Complete blood count (CBC) (automated hematology analyzer), Urine RE/ME (dipstick method), retinopathy (Digital fundus camera) and neuropathy (VPT) were documented. Histopathological classification of renal biopsy sample was undertaken as per International Society of Nephrology and the Renal Pathology Society classification. Results: Total 110 were included of whom 31% were female. 73 patients (66.4%) had DKD 20 patients (18.2%) had NDKD and in 17 patients (15.4%) had an overlap (Mixed group). Amongst NDKD IgA nephropathy was commonest cause of NDKD (25%). Duration of diabetes (p<0.001), late age of onset (p=0.03), BMI (p=0.02), presence of hematuria (p=0.04), retinopathy (p=0.003) and neuropathy(p=0.002) were present in different proportions in the DKD vs the other two groups combined. Multivariable regression analysis however showed that only duration of diabetes was able to discriminate DKD from the other two groups. (AOR=0.9, CI 0.945 0.996, P=0.02), but it had sensitivity of 54% and specificity of 81% (AUC 0.765, P <0.001). Discussion: Almost 25% of our patients with T2DM had some evidence of NDKD (either pure NDKD or mixed) and warrants biopsy for appropriate diagnosis and management. Amongst currently used discriminators, only duration of diabetes was able to predict (not very robust) which patients needed biopsy to identify NDKD.

Clinical characteristics and long-term outcomes in patients with mixed Class III/IV + V and pure proliferative lupus nephritis: A single-center experience

Proliferative lupus nephritis (LN) is a crucial complication in systemic lupus erythematosus (SLE). This study evaluated the clinical implications of coexistence of membranous LN in proliferative LN in terms of clinical characteristics and long-term outcome. We retrospectively reviewed the medical records of patients with SLE who underwent renal biopsy between 2005 and 2018. Patients with proliferative LN based on the 2003 International Society of Nephrology/Renal Pathology Society classification were subclassified into pure (Class III or IV only) and mixed (Class III or IV + Class V) proliferative LN. The clinical features at the time of renal biopsy, incidence of end-stage renal disease (ESRD), and all-cause mortality were compared between patients with mixed or pure proliferative LN. Of the 171 patients, 30 and 141 were classified into mixed and pure proliferative LN groups, respectively. Patients with pure proliferative LN showed higher anti-dsDNA antibody and lower hemoglobin, platelet, and complement 3 levels than patients with mixed proliferative LN. The SLE disease activity index was also higher in patients with pure proliferative LN (p = 0.047). The pure proliferative LN group showed a higher proportion of Class IV and higher histologic activity index scores (p < 0.001 and p = 0.004, respectively). During the follow-up period of 58.3months, 18 patients developed ESRD and 15 patients died. ESRD was exclusively observed in patients with pure proliferative LN, although the incidence of ESRD was not statistically different (p = 0.055). All-cause mortality was comparable between the two groups. Pure proliferative LN was associated with higher clinical and histological activities and modestly increased risk of ESRD. Active immunosuppressive treatment would be required to control the renal inflammation in patients with proliferative LN, regardless of the coexistence of membranous LN.

The Copy Cat Disease A Case of Lupus Podocytopathy

Systemic Lupus Erythematosus (SLE) is a chronic inflammatory disease that can affect various organs. Specifically, when the kidneys are affected this can result in&nbsp;nephritic or nephrotic syndrome. Lupus podocytopathy (LP) is a type of nephrotic syndrome associated with SLE. This case looks at a 44 year old female who presented to the emergency department (ED) with bilateral lower extremity edema and was noted to have hypoalbuminemia and an elevated total protein/creatinine ratio. She was discharged from the ED with a diagnosis of nephrotic syndrome and advised to follow up with nephrology as an outpatient. Her outpatient labs were positive for antinuclear antibodies (ANA), anti-ribonucleoprotein antibodies (anti-RNP), and anti-smith antibodies. She returned to the ED two weeks later for hand cramping and was admitted for an acute kidney injury. She had an inpatient renal biopsy performed which showed 100% podocyte effacement, concerning for minimal change disease (MCD). However, the biopsy also showed a positive tissue ANA with no immune complex deposition. In the context of MCD and a positive tissue ANA, this patient was diagnosed with LP. She was treated with steroids and hydroxychloroquine with improvement in her kidney function and proteinuria. Lupus podocytopathy is a new entity that is not classified in the 2018 International Society of Nephrology/Renal pathology Society (ISN/RPS) classification system [1]. &nbsp;It is treated similarly to MCD, with steroids and possibly immunosuppressive agents. Clinicians should keep autoimmune conditions on their differential when evaluating and treating patients with nephrotic syndrome.&nbsp;

POS-488 IS CHRONICITY A PREDICTOR OF TREATMENT RESPONSE IN CLASS III AND IV LUPUS NEPHRITIS TREATED WITH LOW DOSE CYCLOPHOSPHAMIDE? A RETROSPECTIVE STUDY

Lupus nephritis (LN)is classified histologically according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification. Within this classification class III and IV LN have the worst prognosis and warrant intense immunosuppression with potential for more severe adverse effects. . The first line treatment for class III and IV LN in our setting is low-dose intravenous cyclophosphamide as per the Eurolupus protocol(CYC). The purpose of this study was to identify clinicopathological features at presentation which would correlate with response to treatment.

The relationship between the modified National Institute of Health activity and chronicity scoring system, and the long-term prognosis for lupus nephritis: A retrospective single-center study

The revision of International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification guidelines for lupus nephritis (LN) was suggested by a working group, who recommended a modified National Institute of Health (NIH) activity and chronicity scoring system to evaluate active and chronic LN lesions. However, whether this approach was useful for estimating long-term prognosis for LN patients is unclear. We conducted a retrospective cohort study in Japanese subjects with biopsy-proven LN, between 1977 and 2018. Pathologic lesions were evaluated based on ISN/RPS 2003 classifications and the modified NIH scoring system. Patients were grouped by activity index (low, 0-5; moderate, 6-11; high, 12-24), and chronicity index (low, 0-2; moderate, 3-5; high, 6-12). The primary outcome was a composite of end-stage kidney disease (ESKD) or all-cause death, and the secondary outcome was ESKD alone. Sixty-six subjects with a median age of 31 years were included. During median follow-up (11.5 years), 15 patients reached the primary outcome: 10 had ESKD, four had died, and one had ESKD and died. Kaplan-Meier analysis showed that the cumulative primary outcome incidence increased with a higher chronicity index (log-rank trend p < 0.001). From multivariable survival analysis, moderate (hazard ratio [HR] 6.17, 95% confidence interval [CI] 1.14 to 33.20; p = 0.034) and high chronicity indices (HR 20.20, 95% CI 1.13 to 359.82; p = 0.041) were risk factors for the primary outcome. Moderate and high chronicity indices were associated with an increased ESKD risk for LN.