Presentation Of Nephrotic Syndrome Research Articles

Nephrotic Syndrome and Acute Kidney Injury Presenting in an Older Adult with Uncontrolled Diabetes Mellitus Following Recent COVID-19 Infection Case Report

Nephrotic Syndrome and Acute Kidney Injury Presenting in an Older Adult with Uncontrolled Diabetes Mellitus Following Recent COVID-19 Infection Case Report

Clinical presentation and prognosis in children over 10-year-old with primary nephrotic syndrome

Objective: To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. Methods: The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. Results: There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) vs. 15.9% (65/408), χ2=4.66, P=0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (P>0.05), with minimal change disease the most common type (56.0% (14/25) vs. 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) vs. 23.3% (10/43), χ2=9.18, P=0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% vs. 0, χ2=14.68, P<0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 vs. 3/13, P=0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. Conclusions: Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis, but are liable to relapse in adulthood.

Clinical Course of Adult FSGS and Minimal Change Disease in North American and Japanese Cohorts.

Regional differences in presentation and clinical course of nephrotic syndrome (NS) have not been studied well because few studies directly compared the data from different intercontinental regions. We included adult nephrotic patients with Focal Segmental Glomerulosclerosis (FSGS) and Minimal Change Disease (MCD) who received immunosuppressive therapy (IST) in a North American (NEPTUNE, n=89) or Japanese (N-KDR, n=288) cohort. Baseline characteristics and rates of complete remission (CR) were compared. Factors associated with time to CR were evaluated by Cox regression models. NEPTUNE cases had more FSGS (53.9 vs. 17.0%) and family history of kidney disease (35.2 vs. 3.2%). N-KDR cases were older (median 56 vs. 43 years) and demonstrated greater levels of UPCR (7.73 vs. 6.65) and hypoalbuminemia (1.6 vs. 2.2 mg/dL). N-KDR cases showed higher proportion of CR (overall: 89.2 vs. 62.9%; FSGS: 67.3 vs. 43.7%; MCD: 93.7 vs. 85.4%). A multivariable model showed that FSGS (vs. MCD: HR=0.28, 95%CI 0.20-0.41), systolic blood pressure (per 10 mmHg: HR=0.93, 95%CI 0.86-0.99) and eGFR (per 10 mL/min/1.73m2: HR=1.16, 95%CI 1.09-1.24) were associated with time to CR. There were significant interactions in patient age (p=0.004) and eGFR (p=0.001) between the cohorts. The North American cohort had more FSGS and more frequent family history. Japanese patients showed more severe NS with better response to IST. FSGS, hypertension and lower eGFR were shared predictors of poor treatment response. Identifying shared and unique features across geographically diverse populations may help uncover biologically relevant subgroups, improve prediction of disease course and better design future multi-national clinical trials.

Predictive risk factors of steroid dependent nephrotic syndrome in children with idiopathic nephrotic syndrome

Introduction: Steroid dependent nephrotic syndrome (SDNS) is a difficult and troublesome presentation of idiopathic nephrotic syndrome (INS) in children, with complicated management and increased morbidity. Objectives: The aim of this study was to identify the predictive clinical and laboratory characteristics of patients with SDNS, for parents counseling, appropriate management and improving outcome. Patients and Methods: A total of 374 children with steroid sensitive INS were evaluated in two groups as steroid dependent (group 1=199) and non-steroid dependent (group 2= 175) INS. SDNS was defined as ≥2 relapses during steroid reducing treatment or 15 days after discontinuation of corticosteroids. Results: Mean age at presentation was significantly lower in children with SDNS than those without steroid dependency (P=0.022). Diagnostic age less than two years (P=0.016), total relapses (P&lt;0.001), relapse/year (P&lt;0.001), body mass index (BMI) (P=0.002) and serum cholesterol level (P=0.042) were significantly higher in children with SDNS, compared to those with low-frequent relapse. Mean relapse rate decreased significantly in SDNS with immunosuppressive treatment (P&lt;0.001). Conclusion: Age younger than two years at diagnosis, high BMI, high relapse rate/year and hypercholesterolemia at remission are suggested as predictors of SDNS in children with INS.

Massive Pulmonary Embolism Dominating Section Initial Presentation of Nephrotic Syndrome- A Case Report

Hypercoagulability is a well-recognised feature of Nephrotic Syndrome (NS) and may manifest clinically in the form of Renal Vein Thrombosis (RVT), Deep Vein Thrombosis (DVT) and Pulmonary Embolism (PE); these may uncommonly be among the presenting features of the syndrome. Prompt recognition and management requires that patients with NS should be followed with a high index of suspicion for thromboembolism. Here, the authors report a case of 35-year-old male patient who, while under evaluation for proteinuria, presented with massive PE. He was eventually diagnosed with primary Membranous Nephropathy (MN); a cause of NS with the highest associated risk of thromboembolism. The patient was managed with mechanical ventilation, anticoagulation and supportive care. He also received Rituximab during hospital stay. He was discharged in stable condition on apixaban and advised close follow-up.

A case of thromboembolic events as first manifestation of membranous nephropathy

We present a case of idiopathic membranous glomerulonephritis that presented with venous thromboembolism and was successfully managed with corticosteroid, cyclosporin, appropriate anticoagulant therapy. A 59-year old male patient was referred to our nephrology department who had an outpatient history of swelling in the feet and flank pain was detected proteinuria and thrombosis in the inferior vena cava. Warfarin sodium treatment was started when thrombus was detected in the femoral vein, renal vein and inferior vena cava in the imaging. The patient had 12 g/day proteinuria, hypoalbuminemia, hyperlipidemia and antiphospholipase A2 receptor antibody positivity and treatment was started with the diagnosis of idiopathic membranous nephropathy. The patient who was discharged with oral steroid, cyclosporine and warfarin sodium, but warfarin sodium was changed to rivaroxaban in another clinic due to incompatibility problems, was presented again with dsypnea. Pulmonary embolism was detected. Rivaroxaban was discontinued and low molecular weight heparin treatment was started. There was a very high risk of morbidity and mortality and with appropriate treatment, clinical status and renal function were stabilized. In conclusion, although thromboembolism can be seen in nephrotic syndrome, it may rarely be encountered as the first presentation of nephrotic syndrome. This situation should not be overlooked, appropriate treatments should be provided without delay.

Clinical presentation and management of nephrotic syndrome in the first year of life: A report from the Pediatric Nephrology Research Consortium.

Background and objectivesNephrotic syndrome (NS) in the first year of life is called congenital (CNS) if diagnosed between 0–3 months, or infantile (INS) if diagnosed between 3–12 months of age. The aim of this study was to determine if there were clinically meaningful differences between CNS and INS patients, regarding clinical presentation, management and outcomes.Design, setting, participants, and measurementsEleven Pediatric Nephrology Research Consortium sites participated in the study, using IRB-approved retrospective chart reviews of CNS and INS patients born between 1998 and 2019. Data were collected on patient characteristics, pertinent laboratory tests, provided therapy, timing of unilateral/bilateral nephrectomy and initiation of renal replacement therapy (RRT).ResultsThe study included 69 patients, 49 with CNS and 20 with INS, with a median age at diagnosis of 1 and 6 months, respectively. Management for the two groups was similar regarding nutrition, thyroxin supplementation, immunoglobulin administration, and thrombosis prophylaxis. Within the first 2 months after diagnosis, daily albumin infusions were used more often in CNS vs. INS patients (79 vs. 30%; p = 0.006), while weekly infusions were more common in INS patients (INS vs. CNS: 50 vs. 3%; p = 0.001). During the 6 months preceding RRT, albumin infusions were more frequently prescribed in CNS vs. INS (51 vs. 15%; p = 0.007). Nephrectomy was performed more often in CNS (78%) than in INS (50%; p = 0.02). End-stage kidney disease tended to be more common in children with CNS (80%) vs. INS (60%; p = 0.09).ConclusionCompared to INS, patients with CNS had a more severe disease course, requiring more frequent albumin infusions, and earlier nephrectomy and RRT. Despite center-specific variations in patient care, 20–40% of these patients did not require nephrectomy or RRT.

The Clinical and Genetic Features in Chinese Children With Steroid-Resistant or Early-Onset Nephrotic Syndrome: A Multicenter Cohort Study.

Steroid-resistant nephrotic syndrome (SRNS) is one of the major causes of end-stage kidney disease (ESKD) in children and young adults. For approximately 30% of children with SRNS results from a genetic cause. In this study, genotype-phenotype correlations in a cohort of 283 pediatric patients with SRNS or early-onset NS (nephrotic syndrome presenting within the first year of life) from 23 major pediatric nephrology centers in China were analyzed. All patients were performed with next-generation sequencing and Sanger sequencing. The overall mutation detection rate was 37.5% (106 of 283 patients). WT1 was the most frequently detected mutation, followed by NPHS1, NPHS2, and ADCK4, and these four major causative genes (WT1, NPHS1, NPHS2, and ADCK4) account for 73.6% of patients with monogenic SRNS. Thirteen of 106 individuals (12.3%) carried mutations in ADCK4 that function within the coenzyme Q10 biosynthesis pathway. In the higher frequently ADCK4-related SRNS, two mutations, c.737G>A (p.S246N) and c.748G>C (p.D250H), were the most prevalent. Our study provides not only definitive diagnosis but also facilitate available targeted treatment for SRNS, and prediction of prognosis and renal outcome. Our indications for genetic testing are patients with FSGS, initial SRNS, cases of positive family history or those with extra-renal manifestations.

Biopsy or Biomarker? Children With Minimal Change Disease Have a Distinct Profile of Urinary Epidermal Growth Factor.

Background: In this study, the profile of urinary EGF excretion (uEGF/uCreat) was mapped in children presenting with prolonged proteinuria or with nephrotic syndrome refractory to or dependent of steroids. We investigated whether uEGF/uCreat could be linked to the underlying biopsy result, taking into account its response to immunosuppressive medication and to ACE inhibition, as well as genetic predisposition.Methods: Ninety-eight pediatric patients with initial presentation of nephrotic syndrome or prolonged proteinuria were included in this study, along with 49 healthy controls and 20 pediatric Alport patients. All patients had a normal kidney function and were normotensive during the course of the study, whether or not under ACE inhibition. In repeated urine samples, uEGF was measured and concentration was normalized by urine creatinine. In order to compare diagnosis on kidney biopsy, genetic predisposition and response of uEGF/uCreat to immunosuppression and to ACE inhibition, uEGF/uCreat is studied in a linear mixed effects model.Results: Patients with Minimal Change Disease (MCD) showed a significantly different profile of uEGF/uCreat in comparison to healthy children, as well as compared to patients with Focal Segmental Glomerulosclerosis (FSGS) or another glomerulopathy on kidney biopsy. The response of uEGF/uCreat to ACE inhibition was absent in minimal change disease and contrasted with an impressive beneficial effect of ACE inhibition on uEGF/uCreat in FSGS and other proteinuric glomerulopathies. Absence of a genetic predisposition was also associated with a significantly lower uEGF/uCreat.Conclusions: Despite preserved kidney function, children with a proteinuric or nephrotic glomerular disease on kidney biopsy show a significantly lower uEGF/uCreat, indicative of early tubulo-interstitial damage, which appears reversible under ACE inhibition in any underlying glomerulopathy except in minimal change disease. In view of the distinct profile of uEGF/uCreat in minimal change disease compared to other glomerulopathies, and the link between genetic predisposition and uEGF/uCreat, our study suggests that uEGF/uCreat can be a helpful tool to decide on the need for a renal biopsy in order to differentiate minimal change disease from other proteinuric glomerular diseases.

MO310LIPID DISORDERS IN NEPHROTIC SYNDROME

Abstract Background and Aims Lipid disorders are a characteristic manifestation that accompanies the presentation of nephrotic syndrome (NS). The pathophysiology underlying its origin is debated in the literature. It is important to collect large series of patients to accurately characterize these manifestations. The aim of this study was to carry out an analysis of the lipid alterations detected in the presentation of NS, as well as its evolution, in a large cohort of patients treated in the Nephrology Service of a tertiary referral hospital. Method 111 NS outbreaks corresponding to 71 patients seen in the last 12 years were analyzed. Results 53 patients had a single outbreak. 18 patients (25.35%) had 2 or more outbreaks. 63.1% of the outbreaks affected males. Mean age 54.76 ± 18.46 years (17-85). Charlson comorbidity index 2.62 ± 2.43 points (0-8). The mean of drugs ingested daily prior to NS was 4 ± 3.88 (0-13) There were no significant differences between men and women regarding these three parameters. A renal biopsy was performed in the first outbreak in 67 patients with the result of: 21 membranous nephropathy, 11 minimal change nephropathy, 17 mesangial glomerulonephritis, 8 focal segmental glomerulosclerosis, 2 IgA nephropathy, 5 AA amyloidosis, 3 AL amyloidosis. 90.1% of the patients had high cholesterol levels (&amp;gt; 200 mg/dL). 73% of the patients had high LDL cholesterol (&amp;gt; 160 mg/dL). 72.1% of the patients had triglycerides (TG) above normal levels (&amp;gt; 150 mg/dL). 47.75% of the patients had a high atherogenic index (&amp;gt; 5). The mean levels at the presentation of NS were: total cholesterol 338.07 ± 111.61 mg/dL; HDL cholesterol 67.92 ± 25.46 mg/dL; LDL cholesterol 227.76 ± 99.28 mg/dL; TG 215.48 ± 97.27; atherogenic index 5.12 ± 2.47. There were no significant differences regarding these variables and the various glomerular diseases. Patients with prior dyslipidemia history, showed significantly lower cholesterol levels, 309.69 ± 98.08 mg/dL vs 363.53 ± 115.55 mg/dL, perhaps because they were already taking statins (we do not have this data). There is a significant correlation between total cholesterol and LDL cholesterol with serum albumin, but not between total cholesterol or LDL with proteinuria. There is a correlation between TG with both albumin and proteinuria. There is a significant inverse correlation between the neutrophil/lymphocyte ratio (NLR) and total cholesterol and LDL cholesterol. The higher the NLR, the lower the cholesterol. It gives the impression that the sicker/inflamed the patient is, the lower the ability to synthesize cholesterol. In our series, patients with acute kidney injury (AKI) or previous chronic kidney disease (CKD) had significantly lower cholesterol levels. AKI 306.84 ± 105.24 mg/dL vs no AKI 354.04 ± 110.50 mg/dL. CKD 293 ± 124.15 mg/dL vs no CKD 347.07 ± 106.27 mg/dL. In multivariate analysis, the variables associated with the level of total cholesterol and LDL cholesterol were serum albumin and the Charlson comorbidity index. Regarding the triglyceride level, the associated variables were serum albumin and proteinuria. In the evolution of the patients, both total cholesterol and triglycerides improved significantly after reaching NS remission: final cholesterol 190 mg/dL; Final triglycerides 141 mg/dL. Conclusion As in other series, we detected a high prevalence of lipid alterations in our population of adult patients with NS. Hypoalbuminemia appears as the factor that is independently associated with cholesterol and triglyceride levels. The lipid alterations improve in a parallel way as the NS picture does.

Rare thrombotic complications as an initial presentation of nephrotic syndrome

Thrombosis in nephrotic syndrome (NS) is rare event with approximate incidence being 2–3%. Mostly these complications follow NS with involvement of superficial and deep venous system. Cerebral venous sinus thrombosis (CVST) as an initial presentation of NS is rarely reported. Here, we described two cases; an 8-year-old boy and 9-year-old girl, who developed life threatening CVST and renal vein thrombosis, respectively, as initial presentation of NS. Both cases were diagnosed with early imaging studies and treated with appropriate anticoagulant therapy with complete resolution on repeat imaging.

Clinical Characteristics of Acute Glomerulonephritis with Presentation of Nephrotic Syndrome at Onset in Children

Clinical Characteristics of Acute Glomerulonephritis with Presentation of Nephrotic Syndrome at Onset in Children

Rare Presentation of Nephrotic Syndrome in 10-Year-Old Male

Rare Presentation of Nephrotic Syndrome in 10-Year-Old Male

Steroid Sensitive Nephrotic Syndrome Presented as Posterior Reversible Encephalopathy Syndrome - A Rare Case Report

Posterior reversible encephalopathy syndrome (PRES) is a rare serious syndrome of central nervous system that can develop in both adults and children. It is characterised by acute onset of headache, confusion, seizures, or focal neurological deficits along with radiological abnormalities in the parietal and occipital lobes. In the past, this syndrome has been mainly described in adults but rare in children. However, it is not uncommon in paediatric nephrology. Hypertension, renal disease, immunosuppression, and chemotherapy of malignancies are triggers for PRES. Here, we report a case of 12 years old boy with steroid-sensitive nephrotic syndrome presenting as PRES.

Clinical characteristics of acute glomerulonephritis with presentation of nephrotic syndrome at onset in children

BackgroundAcute glomerulonephritis (AGN) is a common disease in children, which places a huge burden on developing countries. The prognosis of it may not always be good. However, the clinical characteristics of AGN with nephrotic syndrome (NS) at onset have not been fully clarified. MethodsOne hundred and thirteen cases were analyzed retrospectively. Clinical data, pathological results and prognosis between AGN with NS (AGN-NS) and AGN without NS (AGN-no-NS) were compared. ResultsTwenty (17.7%) of 113 patients were AGN-NS. The patients with AGN-NS were more likely to have hypertension (55.0% vs. 25.8%) and acute kidney injury (AKI) (50.0% vs. 17.2%). AKI was significantly related to the manifestation of AGN-NS in children (OR = 3.812, P = 0.040). Compared with the AGN-no-NS, the immunosuppressive treatments were more common in AGN-NS. A more severe pathological grade was significantly related to lower C3 fraction, estimated glomerular filtration rate (eGFR), and AKI, but not to the performance of AGN-NS. There was no difference in prognosis between the two groups. ConclusionsAKI was significantly associated with AGN-NS. The prognosis of AGN-NS and AGN-no-NS in our study was almost good. Given the fact that AGN-NS patients are more likely to use immunosuppressive therapy, the long-term outcome of AGN-NS warrants further research.

Atypical Presentation of Cytomegalovirus-Related Infantile Nephrotic Syndrome

Nephrotic syndrome is the most common glomerular disease in children. Most cases are idiopathic and the first episode is rarely related to cytomegalovirus infection, particularly after 3 months of age. We present a 7-month-old infant who developed atypical presentation of nephrotic syndrome secondary to cytomegalovirus infection. The patient was referred to undergo orchipexy due to right-sided undescended testis. Following the surgery, he developed fever, gastroenteritis and renal failure. A few days later, generalized edema and proteinuria were detected. Due to positive test results for cytomegalovirus, ganciclovir was administered. Remission of nephrotic syndrome was obtained within the first two weeks of the treatment. No relapse of nephrotic syndrome was detected during 12 months of follow up. We may conclude that in unexplained infantile nephrotic syndrome, CMV should be considered as one of the possible etiologies. Keywords: Nephrotic Syndrome; Cytomegalovirus; Infant; Ganciclovir; Edema; Proteinuria; Gastroenteritis.

A Case of Nephrotic Syndrome Presenting With Pulmonary Embolus in a Kidney Transplant Patient

A Case of Nephrotic Syndrome Presenting With Pulmonary Embolus in a Kidney Transplant Patient

PULMONARY EMBOLISM AS PRESENTING FEATURE OF MEMBRANOUS NEPHROPATHY INDUCED NEPHROTIC SYNDROME

Severe pulmonary embolism as first-time presentation of Nephrotic syndrome is rare but Nephrotic syndrome secondary to membranous nephropathy (MN) may impose a greater thrombotic risk for unclear reasons. Here, we report the case of a 36-year-old female patient presented with complaints of sudden onset of shortness of breath since 4-5 days and features of right-sided heart failure. There was no preceding history of any chronic disease or renal disease. She was diagnosed as having a bilateral severe pulmonary embolism. Extensive workup and renal biopsy were done which was suggestive of primary membranous nephropathy.

Outcomes of Older Patients (≥60 years) with New-Onset Idiopathic Nephrotic Syndrome Receiving Immunosuppressive Regimen: A Multicentre Study of 116 Patients.

Because of its rarity, renal presentation and outcomes of idiopathic nephrotic syndrome (INS; minimal changes disease or focal and segmental glomerulosclerosis) has poorly been described in elderly patients, precluding an individualized therapy procedure. Whether immunosuppressive regimens formerly designed in children and young adults are safe and efficient in elderly remains elusive. In a large multicentric retrospective study that included 116 patients with INS and onset ≥ 60 years of age, we showed that cumulative incidence of renal response was 95% after frontline therapy, with an age-dependent median time-to-response (60 days before 70 years of age at the onset vs. 120 days after; p = 0.03). Cumulative incidence of relapse was 90% at 7 years, with relapse occurring continuously over time. After a median follow-up of 34 months (IQR (12; 57)), 7 patients had died (6%) and 5 reached end-stage renal disease. Complications were highly prevalent: diabetes mellitus (23.3%), hypertension (24.1%), infection requiring hospitalization (21.6%) and acute kidney injury (9.5%). Thus, in older patients with INS and receiving steroids, renal response is delayed and relapse is the rule. Alternative immunosuppressive regimens, including B-cells depleting agents as frontline therapy, should be tested in this subset of patients to improve the mid- to long-term outcomes.

The Clinicopathological Characteristics of Henoch-Schönlein Purpura Nephritis with Presentation of Nephrotic Syndrome

Background: Henoch-Schönlein purpura nephritis (HSPN) is a common vasculitis involving the kidneys, with a lower incidence in adults. Meanwhile, nephrotic syndrome (NS) can appear in HSPN. However, the clinicopathological features and renal outcome of adult-onset HSPN presenting with NS (NS-HSPN) have not been well clarified. Methods: A total of 191 HSPN patients were prospectively analyzed and comparisons were made between NS-HSPN and non-NS-HSPN. Multivariate Cox regression analysis was carried out to find the unfavorable factors of renal outcome of NS-HSPN. Results: Among the 191 patients, 44 (23.0%) had NS-HSPN. Apart from edema and abdominal pain, patients with NS-HSPN tended to have lower levels of erythrocytes and hemoglobulin in blood as well as a greater number of erythrocytes in urine (p < 0.05). Mesangial proliferation was the most common pathological lesion in HSPN and the rates of crescent formation were significantly different, with 54.5% in NS-HSPN and 33.3% in non-NS-HSPN (p < 0.05). Notably, 18.2 and 4.8% of patients reached the composite endpoints in the NS-HSPN and non-NS-HSPN groups, respectively (p < 0.05), demonstrating that NS-HSPN patients were more likely to progress to end-stage renal disease and had a worse outcome. We also found that hypertension, estimated glomerular filtration rate (eGFR), cystatin, and tubular atrophy/interstitial fibrosis (HR > 1, p < 0.05) at onset were correlated with adverse outcome in NS-HSPN. Conclusion: NS-HSPN had more severe clinicopathological manifestations and poorer prognosis. The adverse predictors of NS-HSPN principally depend on clinicopathological presentation rather than on different therapies, and hypertension, eGFR, cystatin, and tubular atrophy/interstitial fibrosis can serve as independent risk factors in NS-HSPN.