scholarly journals P0362ROLE OF REPEATED RENAL BIOPSY IN ANCA VASCULITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
MAROUA BAAZIZ ◽  
Eric Thervet ◽  
Dominique Nochy ◽  
KARRAS Alexandre
Keyword(s):  
Renal Biopsy ◽  
Kidney Biopsy ◽  
Interstitial Fibrosis ◽  
Systemic Disease ◽  
Renal Pathology ◽  
Induction Treatment ◽  
Refractory Disease ◽  
Fibrinoid Necrosis ◽  
Renal Complication ◽  
Renal Relapse

Abstract Background and Aims Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a systemic disease characterized by inflammation of small vessels and presence of ANCA. Pauci-immune necrotizing crescentic glomerulonephritis is a severe renal complication of AAV. Despite immunosuppressive therapy, relapses are frequent during the course of the disease, affecting up to 50% of the patients. Kidney biopsy is routinely used to diagnose AAV at initial presentation and to predict renal prognosis. Although the activity of renal AAV is not easily evaluated by plasma or urine biomarkers, kidney biopsy is rarely performed when relapse is suspected. We herein analyze the clinical, laboratory and renal pathology data from patients who underwent repeated kidney biopsies during the course of AAV. Method We retrospectively reviewed data from 37 patients who underwent at least 2 kidney biopsies in our centre, between 2002 and 2018. The first renal biopsy (B1) was constantly performed at diagnosis. A follow-up biopsy (B2) was performed for purpose, either for suspicion of refractory disease or confirmation of renal relapse. Modifications of renal pathology between B1 and B2 were studied, by comparing presence of active and chronic lesions. Active lesions were defined by the presence of cellular crescents and/or fibrinoid necrosis. Chronic lesions were quantified by the percentage of globally sclerotic glomeruli and the percentage of interstitial fibrosis / tubular atrophy (IF/TA). Results Median age at diagnosis was 57 years, M/F ratio was 21/16. ANCA specificity was anti-MPO in 65% of cases, anti-PR3 in 32%. The median delay between B1 and B2 was 3,3 [0,9-5,8] years. B2 was done for suspicion of refractory disease (n=8) or of renal relapse (n=29). Causes of B2 were : persistence or reappearance of haematuria in 78% of cases, increase of creatinine in 43%, increase of ANCA titer in 67%. Systemic AAV activity was more important at B1 vs B2 (median BVAS 18 vs 9), as well as renal dysfunction (median sCreat 200 vs 156 μmol/l). Active glomerulonephritis was constantly found at B1 but was present in only 35% of B2. Presence of cellular crescents decreased from 71,4 to 29,7% (p = 0,002), whereas fibrinoid necrosis decreased from 80,6 to 35,1% (p = 0.0003). Five factors were significantly associated with the presence of active lesions : presence of at least one extra-renal AAV manifestation (p = 0.0006), increase of ANCA titer (p = 0.0022), CRP>30mg/l (p = 0.001), absence of IF/TA (p=0,02) and high percentage of normal glomeruli (p=0,014) at B1. Interestingly, level of proteinuria and persistence of hematuria were not associated with histological activity at B2 (p =0,64 and 0,22 respectively). In contrast, chronic lesions such as glomerulosclerosis and IF/TA were more severe at B2 compared to B1 (p <0,0001 and 0,014 respectively). The worsening of fibrotic lesions was not associated with ANCA specificity (anti-MPO vs -PR3). Conclusion This is the largest cohort reporting on repeated renal biopsy in AAV. Despite several clinical and laboratory signs suggesting active renal AAV, B2 revealed no relapse in 2/3 of cases, allowing avoidance of a new immunosuppressive induction treatment. Our results suggest that rebiopsy is crucial when renal relapse is suspected, as proteinuria and hematuria do not predict active disease, suggesting that their persistence could be explained by glomerular scarring.

P0132DIAGNOSIS OF HYPEROXALURIA FROM RENAL BIOPSY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Marwa Omrane ◽  
Raja Aoudia ◽  
Mondher Ounissi ◽  
Nada Sellami ◽  
Mouna Jerbi ◽  
...  
Keyword(s):  
Renal Failure ◽  
Kidney Disease ◽  
Renal Biopsy ◽  
Kidney Biopsy ◽  
Interstitial Fibrosis ◽  
Renal Pathology ◽  
Crystal Deposition ◽  
Biopsy Specimens ◽  
Initial Symptoms ◽  
History Of

Abstract Background and Aims Crystal-induced kidney disease refers to kidney injury caused by intratubular crystal deposition. The most common forms of crystalline nephropathy encountered in renal pathology are nephrocalcinosis and oxalate nephropathy. The purpose of our study is to determine the epidemiological and clinical characteristics of hyperoxaluria diagnosed from renal biopsy. Method We retrospectively reviewed all kidney biopsy specimens evaluated at renal pathology laboratory, from 1976 to 2019. The biopsy specimens were received from multiple medical department and medical centers. We studied 8900 biopsy specimens and we were focused on patients whose diagnosis of hyperoxaluria was made from renal biopsy Results We identified 25 cases (15 children and 10 adults) with a sex ratio H / F of 0.9. Mean age at diagnosis was 17.2 years old [4 months-73 years old]. Most patients were offspring of consanguineous mating (14 of 25) with intermarriage of first-degree cousins being the most common pattern. A family history of chronic kidney disease was found in 13 patients: indeterminated nephropathy (n = 6) and renal stone (n = 5) and primary hyperoxaluria (n=2). Among our patients, five had a history of urolithiasis. One patient had a history of chronic diarrhea related to Crohn's disease and one patient had a history of cephalic pancreatectomy and ileal resection. Initial symptoms and signs were dominated by renal failure (n = 25) with mean creatinine of 789.5 μmol / l [306-1832μmol / l], associated with proteinuria in 10 patients and hematuria in 11 patients. Arterial hypertension was present in 4 patients. Oligo anuria was reported in 4 patients without dilation of the urinary excretory pathways. In our patients, the diagnosis of crystalin nephropathy was revealed by renal biopsy. In one case, the diagnosis was made after renal transplant. In 4 cases the diagnosis was made by postmortem kidney biopsy. In all cases, the kidney biopsy specimen showed extensive intratubular crystal deposition and tubulointerstitial mononuclear cell infiltration with features of tubular injury and interstitial fibrosis. Examination of histologic slides showed colorless refractile crystals of polygonal appearance. Multicolored birefringence under polarized light identified these crystals as calcium oxalate. After different investigations (genetic and biological analysis), the diagnosis of hyperoxaluria was confirmed. Hyperoxaluria was primary in 23 patients and secondary in 2 patients. Conclusion Hyperoxaluria is a rare condition, often serious, involving renal prognosis and sometimes life-threatening, especially in early-onset forms. Early diagnosis and treatment should be done as soon as possible to slow the progression to end-stage renal failure. In patients with renal insufficiency, the diagnosis of hyperoxaluria is difficult. Renal biopsy can help when clinical and radiological data are not sufficient.

A Validation of the 2018 Revision of International Society of Nephrology/Renal Pathology Society Classification for Lupus Nephritis: A Cohort Study from China

2020 ◽  
Vol 51 (6) ◽  
pp. 483-492 ◽  
Author(s):  
Juan Tao ◽  
Hui Wang ◽  
Xiao-Juan Yu ◽  
Ying Tan ◽  
Feng Yu ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Renal Biopsy ◽  
International Society ◽  
Interstitial Fibrosis ◽  
Activity Index ◽  
Renal Pathology ◽  
Tubular Atrophy ◽  
Female Ratio ◽  
Renal Outcomes

Background: A revision of the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification for lupus nephritis has been published in 2018. The current study aimed to verify the utility of this system. Materials and Methods: A total of 101 lupus nephritis patients from a large Chinese cohort who underwent renal biopsy in Peking University First Hospital were reevaluated by 2 renal pathologists, who had no knowledge of the clinical findings. The association between clinical data at the time of initial renal biopsy and follow-up and pathological features were further analyzed on all patients selected. Results: The mean age of the cohort was 33 years with a male/female ratio of 1:9, and a median follow-up period of 128 months. The presence and extent of mesangial hypercellularity, endocapillary hypercellularity, global and segmental glomerulosclerosis, neutrophil exudation/karyorrhexis, glomerular hyaline deposits, extracapillary proliferation (crescents), tubular atrophy/interstitial fibrosis, and interstitial inflammation were significantly correlated with several clinical renal injury indices (systemic lupus erythematosus disease activity index, serum creatinine value, proteinuria, and C3 level) at the time of biopsy. By multivariable Cox hazard analysis, fibrous crescents, tubular atrophy/interstitial fibrosis, and the modified National Institutes of Health chronicity index were independent risk factors for patients’ composite renal outcomes (hazard ratio [HR] 4.100 [95% CI 1.544–10.890], p = 0.005; HR 8.584 [95% CI 2.509–29.367], p = 0.001; and HR 3.218 [95% CI 1.138–9.099], p = 0.028; respectively). Conclusions: The 2018 revision of the ISN/RPS classification for lupus nephritis has utility for prediction of clinical renal outcomes.

MO308COULD THE PRESENCE OF ANCAS IN IGA NEPHROPATHY WITH CRESCENTS HAVE A CLINICAL IMPLICATION?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Zaira Castañeda Amado ◽  
Alejandra Gabaldon ◽  
María Teresa Sanz ◽  
Roxana Bury ◽  
Cinthia Baldallo ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Kidney Biopsy ◽  
Clinical Presentation ◽  
Fibrinoid Necrosis ◽  
Oral Steroids ◽  
Common Clinical Presentation ◽  
The Mean ◽  
End Stage

Abstract Background and Aims IgA nephropathy (IgAN) is the most common glomerulonephritis. The presence of ANCAs in this pathology represents a rare coincidence. However, it is not clear if the presence of IgA or IgG ANCAs in these patients could have clinical significance. We aim to describe the presence of IgA and IgG ANCAs in patients diagnosed with IgAN with crescents, and its possible clinical implications. Method Retrospective study from 2013 to 2020, it included all patients diagnosed by kidney biopsy of IgAN with extracapillary proliferation. Outpatient follow-up time was up to 24 months. Demographics and clinicopathologic data, ANCAs subtype, characteristics of the biopsy and treatment at the time of diagnosis/follow up was recollected. Results From 2013 to 2020, 17 adults were diagnosed with IgAN and extracapillary proliferation. 5 patients presented ANCAs, 3 (17%) were IgA ANCAs and 2 (11%) were IgG ANCAs. At diagnosis, the median age was 48 years old (27-75 years, sd. 15), with 9 women (52%). At the time of diagnosis, the most common clinical presentation was hypertension (71%). The laboratory analysis showed that median hemoglobin was 11.7 mg/dl (8.4-14.9 mg/dL, sd. 1.5), median creatinine was 2.2 mg/dL (0.55-5.7 mg/dL, sd. 1.4) and median proteinuria was 3.5 g/mgCr (0.1-12 g/mgCr, sd. 3.5). 7 patients (41%) presented extracapillary proliferation less than 25%, 7 patients presented it between 25% and 50%, and 3 patients (17%) had it in more than 50%. 5 (30%) patients presented fibrinoid necrosis. 1 (6%) patient needed renal replacement therapy upon admission. In terms of treatment, all patients with ANCAs IgAN received endovenous steroids and cyclophosphamide. The mean follow-up time was 6 months. Oral steroids (59%) and mycophenolate (41%) were the most frequent treatments. At six months, the median creatinine was 1.9 mg/dL (0.4-7, sd. 1.78) and the median proteinuria was 1.45 g/gCr (0.12-5.9, sd. 1.84 g/gCr). 3 patients developed end-stage chronic kidney disease and requiring substitute renal therapy; 4 patients died. Statistical analysis did not show differences in clinical characteristics, demographics, kidney function, proteinuria, need for renal therapy replacement or mortality according to the presence or subtype of ANCA. ANCA negative patients presented less than 25% of extracapillary proliferation in renal biopsy (p = 0.04). ANCA positive patients presented more fibrinoid necrosis than ANCA negative patients (p=0.01). Conclusion Given the limited size of our sample, our results do not allow us to be conclusive, showing no significant differences between the ANCA subtypes. However, from the point of renal biopsy, it is observed that patients with negative ANCAs present less extracapillary proliferation; and that patients ANCA positive presented more fibrinoid necrosis.

Kidney biopsy chronicity grading in antineutrophil cytoplasmic antibody-associated vasculitis

2021 ◽  
Author(s):  
Marta Casal Moura ◽  
Fernando C Fervenza ◽  
Ulrich Specks ◽  
Sanjeev Sethi
Keyword(s):  
Renal Function ◽  
Kidney Biopsy ◽  
Interstitial Fibrosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Response To Treatment ◽  
Remission Induction ◽  
Induction Treatment ◽  
Tubular Atrophy ◽  
Renal Outcomes ◽  
The Impact

Abstract Background Kidney biopsy is valuable for prognostic assessment of renal outcomes in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with glomerulonephritis (AAV-GN) but the impact of chronic changes is not determined. Methods We conducted a retrospective cohort study of myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA-positive patients with AAV and active renal disease. We applied the Mayo Clinic Chronicity Score (MCCS) and validated and evaluated its implications on outcome prediction in AAV-GN. Results We analyzed 329 patients with kidney biopsies available to score. The extent of chronicity was graded by MCCS as minimal [102 (31.0%)], mild [106 (32.2%)], moderate [86 (26.1%)] and severe [35 (10.6%)]. The MCCS grades correlated with the degree of renal function impairment at presentation [mean estimated glomerular filtration rate (eGFR) 48.3 versus 29.2 versus 23.7 versus 18.5 mL/min/1.73 m2, respectively; P < 0.0001]. Higher degrees of the individual components of the MCCS (glomerulosclerosis, interstitial fibrosis, tubular atrophy and arteriosclerosis) were associated with lower median eGFR (P < 0.0001) and decreased event-free [kidney failure (KF) and death] survival (P = 0.002, P < 0.0001, P < 0.0001 and P = 0.017, respectively). Patients with lower MCCS grades recovered renal function more frequently (P < 0.0001). Increasing MCCS grades were associated with decreased renal recovery (P = 0.001), more frequent events and shorter time to KF (P < 0.0001), KF and death (P < 0.0001) and death (P = 0.042), independent of the remission induction treatment used (cyclophosphamide or rituximab). The MCCS stratified renal outcomes for each MCCS grade and can be used in clinical practice as a cutoff for KF prediction (MCCS ≥4). Conclusions Chronic changes on kidney histology independently predict renal function, outcomes and response to treatment in AAV-GN.

scholarly journals Explanatory histological findings for urinary protein and serum creatinine levels at renal biopsy in lupus nephritis: A cross-sectional study

2020 ◽  
Author(s):  
Eri Katsuyama ◽  
Yoshia Miyawaki ◽  
Kenei Sada ◽  
Yosuke Asano ◽  
Keigo Hayashi ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Serum Creatinine ◽  
Renal Biopsy ◽  
Multiple Regression ◽  
Interstitial Fibrosis ◽  
Urinary Protein ◽  
Glomerular Sclerosis ◽  
Fibrinoid Necrosis ◽  
Class Iii ◽  
The Mean

Abstract Background To evaluate histological active and chronic lesions associated with proteinuria and serum creatinine (SCr) level as common clinical endpoints in many clinical trials for lupus nephritis (LN).Methods One hundred and nineteen patients from 1990 to 2015 with LN class III, IV, and V, as defined by the International Society of Nephrology/Renal Pathology Society classification, were enrolled. Multiple regression analysis was performed to explore semiquantitative histological variables related to urinary protein and SCr levels.Results The mean age of enrolled patients was 45 years and 79% were female. The mean SCr level was 0.87 mg/dl and mean urinary protein was 3.00 g/gCr at the time of the renal biopsy. Class IV (71%) was the most common type, followed by class III (17%) and class V (13%). Multicollinearity was confirmed between monocellular infiltration (variance inflation factor [VIF] = 10.22) and interstitial fibrosis (VIF = 10.29) and between karyorrhexis (VIF = 4.14) and fibrinoid necrosis (VIF = 4.29). After excluding fibrinoid necrosis and monocellular infiltration because of multicollinearity, only urinary protein level was correlated with wire loop (β−coefficient [β]: 1.09 and confidence interval [CI]: 0.35 to 1.83), and SCr level was correlated with glomerular sclerosis (β: 1.08 and CI: 0.43 to 1.74) by multiple regression analysis.Conclusion As urinary protein and SCr levels could not reflect active lesions quantitatively, they might be difficult to be evaluated for response to induction remission treatments in patients with LN.

scholarly journals AB0441 ASSOCIATION OF CO-POSITIVITY FOR ANTI-DSDNA, -NUCLEOSOME, AND -HISTONE ANTIBODIES AND DISEASE ACTIVITY IN PATIENTS WITH LUPUS NEPHRITIS: RESULTS FROM THE KORNET REGISTRY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1519.2-1519
Author(s):  
D. J. Park ◽  
S. E. Choi ◽  
H. Xu ◽  
J. H. Kang ◽  
S. S. Lee
Keyword(s):  
Lupus Nephritis ◽  
Disease Activity ◽  
Renal Biopsy ◽  
Lupus Erythematosus ◽  
Kidney Biopsy ◽  
Activity Index ◽  
Disease Activity Index ◽  
Induction Treatment ◽  
Rapid Decline

Background:Recent studies have shown that the simultaneous positivity of anti-double stranded DNA, -nucleosome, and -histone antibodies (3-pos) is prevalent in lupus nephritis (LN) patients compared to non-renal systemic lupus erythematosus (SLE) patients.Objectives:The aim of this study was to define the clinical, biologic, histopathologic, and prognostic differences according to the simultaneous reactivity to those antibodies in Korean patients with biopsy-proven LN.Methods:We studied 102 patients who underwent kidney biopsy prior to the start of induction treatment and who were subsequently treated with immunosuppressives and followed-up for more than 12 months. Sociodemographic, clinical, laboratory, and treatment-related data at the time of kidney biopsy and during follow-up were obtained by a review of patients’ charts. Antibodies were detected by immunoblot analysis or ELISA at the time of renal biopsy.Results:Fifty-eight (35.4%) of the total of 102 LN patients had 3-pos. In comparison with non-3-pos patients, the patients with 3-pos showed a higher SLE Disease Activity Index-2000 score (P=0.002), lower lymphocyte level (p=0.004), higher proportion of proteinuria >3.5 g/24 hr (p=0.005), and higher positivity of urinary sediments (p=0.005) at the time of renal biopsy. In the renal histopathologic findings, the patients with 3-pos had more proliferative LN (p=0.015) and also showed more endocapillary hypercellularity, sub-endothelial hyaline deposits, fibrinoid necrosis/karyorrhexis, and cellular crescents in the disease activity index (p=0.016, p=0.045, p=0.002, and p=0.022, respectively), as well as a higher activity score (p=0.011). After a median follow-up of 83.2 months, rapid glomerular filtration rate decline was frequently observed in patients with 3-pos compared to those without (p=0.012).Conclusion:Our findings suggest that 3-pos is related to severe LN and, furthermore, that patients with 3-pos show a rapid decline of renal function compared to those without.Disclosure of Interests:None declared

scholarly journals More than ancillary records: clinical implications of renal pathology examination in tumor nephrectomy specimens

2021 ◽  
Author(s):  
Johannes Philipp Kläger ◽  
Ahmad Al-Taleb ◽  
Mladen Pavlovic ◽  
Andrea Haitel ◽  
Eva Comperat ◽  
...  
Keyword(s):  
Interstitial Fibrosis ◽  
Renal Pathology ◽  
Vascular Lesions ◽  
Renal Tumors ◽  
Renal Diseases ◽  
Tumor Type ◽  
Neoplastic Tissue ◽  
Cell Renal Cell Carcinoma ◽  
Hypertensive Nephropathy ◽  
Tumor Nephrectomy

Abstract Background Nephrectomy is the management of choice for the treatment of renal tumors. Surgical pathologists primarily focus on tumor diagnosis and investigations relating to prognosis or therapy. Pathological changes in non-neoplastic tissue may, however, be relevant for further management and should be thoroughly assessed. Methods Here, we examined the non-neoplastic renal parenchyma in 206 tumor nephrectomy specimens for the presence of glomerular, tubulo-interstitial, or vascular lesions, and correlated them with clinical parameters and outcome of renal function. Results We analyzed 188 malignant and 18 benign or pseudo-tumorous lesions. The most common tumor type was clear cell renal cell carcinoma (CCRCC, n = 106) followed by papillary or urothelial carcinomas (n = 25). Renal pathology examination revealed the presence of kidney disease in 39 cases (18.9%). Glomerulonephritis was found in 15 cases (7.3%), and the most frequent was IgA nephropathy (n = 6; 2.9%). Vasculitis was found in two cases (0.9%). In 15 cases we found tubulo-interstitial nephritis, and in 9 severe diabetic or hypertensive nephropathy. Partial nephrectomy was not linked to better eGFR at follow-up. Age, vascular nephropathy, glomerular scarring and interstitial fibrosis were the leading independent negative factors influencing eGFR at time of surgery, whereas proteinuria was associated with reduced eGFR at 1 year. Conclusion Our large study population indicates a high incidence of renal diseases potentially relevant for the postoperative management of patients with renal neoplasia. Consistent and systematic reporting of non-neoplastic renal pathology in tumor nephrectomy specimens should therefore be mandatory.

scholarly journals MP085DEVELOPMENT OF A NEW RENAL PATHOLOGY CODING LIST FOR THE FLEMISH RENAL BIOPSY (FCGG) REGISTRY

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii457-iii457
Author(s):  
Amélie Dendooven ◽  
Wim Laurens ◽  
Mark Helbert ◽  
Pascale De Paepe ◽  
Evelyne Lerut ◽  
...  
Keyword(s):  
Renal Biopsy ◽  
Renal Pathology

scholarly journals MO638ANEMIA IN DIABETIC PATIENTS REFLECTS SEVERE TUBULOINTERSTITIAL INJURY AND ASSISTS CLINICALLY IN PREDICTING DIAGNOSIS OF DIABETIC NEPHROPATHY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Soichiro Yokota ◽  
Kenji Ito ◽  
Maho Watanabe ◽  
Koji Takahashi ◽  
Naoko Himuro ◽  
...  
Keyword(s):  
Renal Function ◽  
Regression Analysis ◽  
Diabetic Nephropathy ◽  
Kidney Disease ◽  
Confidence Interval ◽  
Odds Ratio ◽  
Kidney Biopsy ◽  
Renal Pathology ◽  
Diabetic Patients ◽  
Pathological Findings

Abstract Background and Aims Diabetic nephropathy (DN) is currently a leading cause of end-stage kidney disease worldwide. Kidney biopsy is generally performed in diabetic patients to discriminate between DN and non-diabetic kidney disease (NDKD), and to provide more specific treatments. In addition to conventional predicting factors of DN, recent studies suggested the predictive value of anemia in the diagnosis of DN, however detailed pathophysiology and the significance of anemia in renal pathology are not fully understood. This study aimed to investigate the impact of anemia on renal pathology and clinical course in patients who underwent kidney biopsy. Method We reviewed 81 patients (60.4 ± 13.7 years, 54 men and 27 women) with type 2 diabetes who underwent percutaneous kidney biopsy in Fukuoka University Hospital from January 2001 through March 2020. DN was diagnosed by mesangial expansion or nodular glomerulosclerosis observed under a light microscope, and immunofluorescence assisted in differentiating NDKD from DN. Anemia was defined as hemoglobin level <13 g/dL in males and <12 g/dL in females in accordance with the World Health Organization standards. Laboratory and pathological findings, and clinical courses were investigated. Results According to their pathological findings, patients were classified into two groups: isolated DN (DN group, n=30) and NDKD alone or concurrent DN (NDKD group, n=51). There were 11 types of NDKD. Of these, membranous nephropathy was the most common (23.5%), followed by IgA nephropathy (17.6%), and crescentic glomerulonephritis (13.7%). In multiple logistic regression analysis, absence of severe hematuria (odds ratio (OR) 11.66, 95% confidence interval (CI) 1.68 - 89.9) and presence of anemia (OR 11.38, 95% CI 2.51 - 51.52) were significantly related with the diagnosis of DN. Akaike’s information criterion (AIC) and net reclassification improvement (NRI) analyses revealed improved predictive performance by adding anemia to the conventional factors (AIC 100.152 to 91.844; NRI 27.0%). The tissues of patients in the DN group demonstrated more severe interstitial fibrosis and tubular atrophy (IF/TA) than the NDKD group (p<0.05) regardless of the rate of global glomerulosclerosis (figure), and IF/TA was related to the prevalence of anemia (odds ratio: 7.31, 95% confidence interval: 2.33 - 23.00) in multivariate regression analysis. These results suggest DM-associated severe IF/TA (compared with NDKD) impaired erythropoietin production, resulting in earlier anemia, independent of glomerular injuries and renal function. Furthermore, the renal prognosis was significantly better in the NDKD group than in the DN group using Log-rank test (p<0.05). Conclusion DN is associated with anemia because of severe IF/TA regardless of renal function, and anemia helps clinician discriminate clinically between isolated DN and NDKD.

scholarly journals In Patients with Membranous Lupus Nephritis, Exostosin-Positivity and Exostosin-Negativity Represent Two Different Phenotypes

2021 ◽  
pp. ASN.2020081181 ◽  
Author(s):  
Aishwarya Ravindran ◽  
Marta Casal Moura ◽  
Fernando C. Fervenza ◽  
Samih H. Nasr ◽  
Mariam P. Alexander ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Kidney Biopsy ◽  
Interstitial Fibrosis ◽  
Study Cohort ◽  
Lower Serum ◽  
Tubular Atrophy ◽  
Novel Proteins ◽  
Biopsy Specimens ◽  
Membranous Lupus Nephritis

BackgroundIn patients with secondary (autoimmune) membranous nephropathy, two novel proteins, Exostosin 1 and Exostosin 2 (EXT1/EXT2), are potential disease antigens, biomarkers, or both. In this study, we validate the EXT1/EXT2 findings in a large cohort of membranous lupus nephritis.MethodsWe conducted a retrospective cohort study of patients with membranous lupus nephritis, and performed immunohistochemistry studies on the kidney biopsy specimens against EXT1 and EXT2. Clinicopathologic features and outcomes of EXT1/EXT2-positive versus EXT1/EXT2-negative patients were compared.ResultsOur study cohort included 374 biopsy-proven membranous lupus nephritis cases, of which 122 (32.6%) were EXT1/EXT2-positive and 252 (67.4%) were EXT1/EXT2-negative. EXT1/EXT2-positive patients were significantly younger (P=0.01), had significantly lower serum creatinine levels (P=0.02), were significantly more likely to present with proteinuria ≥3.5 g/24 h (P=0.009), and had significantly less chronicity features (glomerulosclerosis, P=0.001 or interstitial fibrosis and tubular atrophy, P<0.001) on kidney biopsy. Clinical follow-up data were available for 160 patients, of which 64 (40%) biopsy results were EXT1/EXT2-positive and 96 (60%) were EXT1/EXT2-negative. The proportion of patients with class 3/4 lupus nephritis coexisting with membranous lupus nephritis was not different between the EXT1/EXT2-positive and EXT1/EXT2-negative groups (25.0% versus 32.3%; P=0.32). The patients who were EXT1/EXT2-negative evolved to ESKD faster and more frequently compared with EXT1/EXT2-positive patients (18.8% versus 3.1%; P=0.003).ConclusionsThe prevalence of EXT1/EXT2 positivity was 32.6% in our cohort of membranous lupus nephritis. Compared with EXT1/EXT2-negative membranous lupus nephritis, EXT1/EXT2-positive disease appears to represent a subgroup with favorable kidney biopsy findings with respect to chronicity indices. Cases of membranous lupus nephritis that are EXT1/EXT2-negative are more likely to progress to ESKD compared with those that are EXT1/EXT2-positive.

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