Primary Sjögren’s disease (pSjD) is a chronic systemic autoimmune disease in which immune-mediated pathology is not confined to exocrine glands but also affects multiple organ systems. Renal involvement constitutes a clinically meaningful manifestation of pSjD and may exert a considerable impact on disease progression, prognosis, and treatment selection. However, the pathogenic basis of renal injury in pSjD is still incompletely understood, and existing therapeutic approaches remain largely empirical. Available studies suggest that renal involvement in pSjD arises from the interaction of multiple determinants, including inherited susceptibility, environmental factors, and endocrine dysregulation. At the core of these pathogenic mechanisms lies persistent activation of the type I interferon (IFN-I) system and immune dysregulation driven by excessive responses of T and B lymphocytes. Such immune abnormalities favor sustained autoantibody production and ectopic germinal center (EGC) formation, thereby amplifying autoimmune inflammation and promoting renal structural and functional injury. Here, this review integrates relevant literature to examine the multifactorial pathogenic mechanisms described above and to systematically elucidate how these mechanisms drive autoimmune responses through diverse immune cell populations. In addition, the potential application prospects of relevant novel targeted therapeutic strategies in pSjD-related renal damage are discussed.

AimThis study aims to evaluate the efficacy and safety of telitacicept addition to standard therapy in adults with relapsing lupus nephritis (LN).MethodsFrom 2021 to 2024, patients with relapsing LN were identified and divided into two groups based on telitacicept administration. Laboratory indicators, renal remission status, Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores, glucocorticoid dosages, and the incidence of renal flares were evaluated. Multivariate regression was used to assess the baseline predictors of complete renal remission (CRR) and adverse events (AEs) were recorded.ResultsCompared to the standard therapy group (n=20), the proportion of patients who achieved a CRR and the primary efficacy renal response were significantly increased in the telitacicept group (n=20) at 6, 9, and 12 months, while the reduction rates of 24-hour urinary protein from baseline at 1, 3, and 9 months were significantly higher. The telitacicept group showed notable improvement in treatment responses in the median SLEDAI-2K score and glucocorticoid dose. Multivariate Cox regression analysis revealed that add-on telitacicept was associated with achieving early CRR. At the end of the follow-up period, the cumulative relapse rate in the telitacicept group was significantly lower than that in the standard treatment group, with no increase in the incidence of AEs.ConclusionsAs an add-on therapy, telitacicept is associated with early disease remission in patients with relapsing LN with reduced disease activity, lower glucocorticoid dosage, and fewer relapses. It also showed a favorable safety profile.

Immunoglobulin G4-related disease (IgG4-RD) is a rare, multisystemic fibro-inflammatory condition affecting various organs, including kidneys, lungs, nasal cavity, pancreas, salivary glands, and orbit. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) is a multi-systemic inflammatory vascular disease encompassing eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and granulomatosis with polyangiitis (GPA). It often overlaps with the organs or tissues affected by IgG4-RD. Clinically, some individuals with IgG4-RD are ANCA-positive, while some with AAV exhibit elevated IgG4 levels or IgG4-positive plasma cell infiltration, making these conditions difficult to distinguish. Reports have documented cases of overlap syndromes involving IgG4-RD and AAV, highlighting shared pathogenic mechanisms that may include macrophages, B cells, CD4+T cells, and inflammatory cytokines. However, the pathophysiological mechanism underlying these overlap syndromes remains unclear. This review examines potential pathophysiological links between IgG4-RD and AAVs (GPA/MPA) overlap syndromes.

IntroductionWe aimed to investigate the relationship between nuclear factor erythroid 2-related factor 2 (Nrf2) protein expression levels, lupus nephritis (LN) disease activity, and the degree of renal injury (based on the estimated glomerular filtration rate [eGFR]) in patients with LN.MethodsWe selected 40 healthy control participants and 102 patients with LN who were treated in the Second Hospital of Jilin University, China, for inclusion in this study. Patients with LN were classified into LN with high-eGFR and LN with low-eGFR groups. Nrf2 protein levels were measured in the serum and renal tissues of the participants in both groups to assess the correlation between Nrf2 protein levels and different LN disease states.ResultsThere was a significantly positive correlation between serum Nrf2 protein levels, the degree of renal injury, and systemic lupus erythematosus disease activity index (SLEDAI) scores in patients with LN. Nrf2 protein levels were higher in the LN with high-eGFR group than in the healthy control and LN with low-eGFR groups. In follow-up patients in the LN high eGFR group, Nrf2 protein levels decreased significantly after remission of disease activity.ConclusionNrf2 protein expression has a dual role in patients with LN. Nrf2 protein levels not only correlate with disease activity in patients with LN, but also with the degree of kidney injury. Before implementing targeted therapy for Nrf2, evaluating both Nrf2 protein expression and the disease state in patients with LN is necessary to better identify and place each patient in an appropriate patient group.

A complication of pediatric systemic lupus erythematosus (pSLE) is immune thrombocytopenia (ITP). Although corticosteroids and immunoglobulins are frequently used as preliminary treatments, some patients do not respond to them. Rituximab has been reported to be safe and effective in the treatment of pSLE complicated with refractory ITP. Research is currently underway to determine the optimal rituximab dose for these individuals. We report a case of a child with SLE-associated ITP (SLE-ITP) who was successfully treated with rituximab. Rituximab is likely the most viable therapeutic option for refractory SLE-ITP. Furthermore, a comprehensive review of the relevant literature was performed and a concise overview of the pathogenesis and available treatment modalities for pediatric patients diagnosed with SLE and concurrent ITP was provided.

Because of some similarities in organ involvement, clinical manifestations, and histopathological features, IgG4-related disease (IgG4-RD) may occur concurrently with some clinicopathologic variants of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). An overlap syndrome of IgG4-RD and AAV has recently been proposed in clinical and/or histopathological studies, indicating that there may be some potential pathophysiological associations between the two disease entities; however, the mechanisms underlying these are incompletely understood. Here, we describe a rare case of a 63-year-old man with IgG4-related tubulointerstitial nephritis (IgG4-TIN) and microscopic polyangiitis-associated glomerulonephritis (MPA-GN) overlap syndrome. The clinical diagnosis of MPA was based on the 2022 American College of Rheumatology (ACR)/European League Against Rheumatology (EULAR) classification criteria. Remission induction therapy with intravenous methylprednisolone was initiated, followed by oral prednisone maintenance therapy with gradual tapering. The patient remained asymptomatic and his renal function was essentially normalized within 3.5 months of follow-up. The serum IgG4 levels decreased to 5 g/L. We also conducted a literature review to identify clinical findings, treatment options, and outcomes of patients with concurrent IgG4-RD and MPA and briefly discussed the potential pathophysiological association between IgG4-RD and MPA. Our findings enrich the database of this rare overlap syndrome and provide a basis for the diagnosis and early intervention in both diseases. These results provide some insights for clinicians to recognize and treat this overlap syndrome.

Objective We investigated the correlation between zinc levels and Nrf2 expression and potential effects on the clinicopathology of patients with diabetic nephropathy (DN). Methods We selected 30 patients with DN, diagnosed via renal biopsy at our hospital from March 2018 to November 2019, and enrolled 30 healthy individuals from a medical examination center as the control group. Patients with DN were divided into normal-zinc and low-zinc groups. We detected the levels of zinc, copper, and Nrf2 mRNA in their serum, and collected the clinical and pathological data of DN patients. Results Serum zinc level and Nrf2 mRNA expression were significantly decreased in patients with DN compared to those of healthy people ( P < 0.05). Of the 30 patients, 16 had low zinc (53.3%) and 14 had normal zinc levels (46.7%). There was no significant difference in the blood Nrf2 mRNA expression between the two groups ( P > 0.05). However, the expression of Nrf2 in the kidney tissue of the low-zinc group was significantly lower compared to the normal-zinc group ( P < 0.05). Diastolic blood pressure and copper levels were significantly higher in the low-zinc group ( P < 0.05). In contrast, body mass index, red blood cell count, Hb level, and the ratio of zinc to copper were significantly lower in the low-zinc group ( P < 0.05). The pathological classifications of the low-zinc group were more severe ( P < 0.05). Conclusion Patients with DN were more likely to have zinc deficiency and lower expression of Nrf2. Additionally, DN patients with zinc deficiency were prone to have more severe clinical and pathological manifestations.

Introduction: The impact of early-onset peritonitis (EOP) on patients with diabetes undergoing peritoneal dialysis (PD) has not been adequately addressed. We therefore sought to investigate the effects of EOP on the therapeutic response to management and long-term prognostic outcomes in patients with diabetes undergoing PD. Methods: For this retrospective cohort study, we analyzed the data for patients with end-stage renal disease, who were also suffering from diabetes mellitus and had undergone PD between January 1, 2013, and December 31, 2018. EOP was defined as the first episode of peritoneal dialysis-related peritonitis (PDAP) occurring within 12 months of PD initiation. All patients were divided into an EOP group and a later-onset peritonitis (LOP) group. Clinical data, treatment results, and outcomes were compared between groups. Results: Ultimately, 202 patients were enrolled for the analysis. Compared to the EOP group, the LOP group had more Streptococcus (p = 0.033) and Pseudomonas (p = 0.048). Patients with diabetes in the EOP group were less likely to have PDAP-related death (OR 0.13, CI: 0.02-0.82, p = 0.030). Patients with diabetes in the EOP group were more likely to have multiple episodes of PDAP and had higher rates of technical failure and poorer patient survival than those in the LOP group, as indicated by Kaplan-Meier analysis (p = 0.019, p = 0.004, and p < 0.001). In the multivariate Cox proportional hazards model, EOP was a significant predictor for multiple PDAP (HR 4.20, CI: 1.48-11.96, p = 0.007), technical failure (HR 6.37, CI: 2.21-18.38, p = 0.001), and poorer patient survival (HR 3.09, CI: 1.45-6.58, p = 0.003). Conclusions: The occurrence of EOP is significantly associated with lower rates of PDAP-related death and poorer clinical outcomes in patients with diabetes undergoing PD.

Introduction Early-onset peritonitis (EOP) is a risk factor for mortality in peritoneal dialysis (PD) patients. This study investigates the clinical features and outcomes of EOP in elderly patients. Methods This multicenter retrospective study evaluated 433 elderly PD patients with end-stage renal disease. The cohort was divided into nonperitonitis group (n = 239), EOP group (<= 12 months, n = 109) and late-onset peritonitis (LOP) group (>12 months, n = 85). Clinical data, treatment results, and outcomes were compared between the groups. Results Compared with LOP group, there were no significant intergroup differences in the rate of primary recovery, complete cure, relapse, catheter removal, or death from PDAP (p >0.05) in the most recent PDAP episode. However, Kaplan-Meier analysis showed that patients in the EOP group were likely to have multiple episodes of PD-associated peritonitis (PDAP), technique failure, all-cause death, and composite endpoint in the long-term prognostic outcomes (p <0.001). Conclusions EOP is significantly associated with poorer clinical outcomes in older PD patients.

Clear cell renal cell carcinoma (ccRCC) is the most common type of renal cell carcinoma and has a poor prognosis. However, its underlying mechanisms remain unclear. The present study aimed to evaluate the role of small proline-rich repeat protein 3 (SPRR3) in the proliferation, migration and invasion of ccRCC cells and to investigate its upstream and downstream regulatory mechanisms. Survival analysis was performed using the UALCAN website based on the The Cancer Genome Atlas database. Normal renal cell line HK-2 and ccRCC cell lines (786-O, CaKi-1 and UMRC-2) were used. Reverse transcription-quantitative PCR (RT-qPCR) was used to detect mRNA and microRNA (miRNA) levels. Western blotting was used to detect protein levels. Cell Counting Kit-8 and colony formation assays, a wound healing assay and a Transwell invasion assay were used to assess the proliferation, migration and invasion of ccRCC cells, respectively. Transfection of overexpression plasmids and small interfering RNAs were used to upregulate and knockdown SPRR3 expression, respectively. Transfection of miRNA-mimics was used to overexpress miR-338-3p. A luciferase reporter gene assay was used to verify the predicted binding relationship between SPRR3 mRNA and miR-338-3p. The results indicated the following: i) SPRR3 was a risk factor for the survival of patients with ccRCC, and was upregulated in ccRCC cell lines; ii) SPRR3 promoted the proliferation, migration and invasion of ccRCC cells; iii) SPRR3 regulated the tumor phenotypes of ccRCC cells via the PI3K/Akt pathway; iv) miR-338-3p directly targeted SPRR3 mRNA and negatively regulated SPRR3 expression; and v) miR-338-3p inhibited the PI3K/Akt pathway and the tumor phenotypes of ccRCC cells by downregulating SPRR3. In conclusion, SPRR3, as a novel target of miR-338-3p, regulated the proliferation, migration and invasion of ccRCC cells via the PI3K/Akt pathway; this finding not only enriches our understanding of the mechanism underlying ccRCC development, but also demonstrates a potential novel therapeutic target for this disease.

Objective The aim of this study was to evaluate the efficacy of atorvastatin plus disease-modifying antirheumatic drugs (DMARDs) in patients with rheumatoid arthritis (RA). Methods We queried the PubMed, Embase, Web of Science, and the CENTRAL (Cochrane Central Register of Controlled Trials) databases for this study. The pooled efficacy was evaluated using standardized mean differences. The inverse of the variance model was used for data pooling. Results Based on the search, we identified 9 randomized controlled trials. The trials included 258 patients in the atorvastatin plus DMARD groups and 246 patients in the DMARD alone groups. The primary outcome was the change from baseline in the 2018 (209:228 Disease Activity Score in 28 Joints). Based on the Disease Activity Score in 28 Joints, disease activity in RA patients decreased significantly in patients given atorvastatin plus DMARD compared with patients given DMARD alone (standardized mean difference, −2.46; 95% confidence interval, −3.98 to −0.95; p = 0.0015; I 2 = 97%; p < 0.01). Subgroup analysis did not identify any confounding factors, and no publication bias was detected in the meta-analysis. Conclusions The result supports that atorvastatin could be added to DMARDs to treat patients with RA.

In 2018, a renal risk score of antineutrophil cytoplasmic antibody (ANCA)–associated glomerulonephritis (AAGN) based on estimated glomerular filtration rate (eGFR), normal glomeruli, and tubular atrophy/interstitial fibrosis (TA/IF) was proposed to predict renal outcomes. We aimed to evaluate this renal risk score in a myeloperoxidase (MPO)–ANCA predominant population in Northeast China. We retrospectively analyzed the clinicopathologic data of 65 patients biopsy-proven from a Chinese medical center. Each patient was assessed by eGFR, normal glomeruli, and TA/IF, and the renal outcome was evaluated using the renal risk score. In our study, 95.4% of patients were ANCA positive (78.5% MPO-ANCA positive and 16.9% proteinase 3-ANCA positive). The average follow-up period was 14.3 months. Thirty-four patients (52.3%) reached end-stage renal disease (ESRD). Based on the renal risk score, 8 (12.3%), 31 (47.7%), and 26 (40%) patients were divided into the low-risk, medium-risk, and high-risk groups, respectively. Kaplan–Meier survival curves revealed the high-risk group had worse renal outcomes than the low-risk group (p<0.01) and the medium-risk group (p<0.01), but the renal outcome did not differ between the low-risk and medium-risk groups (p>0.017). Similar results were obtained by the competitive survival analysis. The AUC for 3-year overall ESRD predictions was 0.845. In the regression analysis, the renal risk score was a favorable predictor for the development of ESRD (HR 3.13, 95%CI 1.58–6.19, p=0.001). The renal risk score is a preferred index that can predict ESRD in Chinese AAGN patients, especially in the high-risk group with worse renal outcomes.

The present study aimed to detect the levels of microRNA (miR)-33a-5p in the renal tissue, serum and urine of patients with primary IgA nephropathy (IgAN), thereby preliminarily exploring the association between the levels of miR-33a-5p and the condition of primary IgAN to provide evidence for the expression of miR-33a-5p in the serum and urine of IgAN patients as a clinical marker. Reverse-transcription quantitative PCR was performed to evaluate the level of miR-33a-5p in IgAN patients according to severity and pathological classification. The results suggested that the levels of miR-33a-5p in the serum, urine and kidney tissues of patients with IgAN were lower than those of the control tissues obtained from cancer patients (0.28±0.25 vs. 1.00±0.45, P<0.05; 0.34±0.28 vs. 1.00±0.53, P<0.05; 0.47±0.27 vs. 1.00±0.38, P<0.05, respectively). Receiver operating characteristic curve analysis suggested that the serum and urine levels of miR-33a-5p may be used as a marker to differentiate renal injury in IgAN patients from healthy individuals. At the same time, according to the estimated glomerular filtration rate (eGFR) and Lee classification of nephropathy, it was determined that with the progression of renal failure and the increase of the pathological grade of kidney tissue, the relative level of miR-33a-5p in kidney tissue also decreased (eGFR <50 ml/min vs. eGFR ≥50 ml/min/1.73 m2 group: 0.38±0.27 vs. 1.00±0.34, P<0.001; Lee grade ≤3 group vs. Lee grade >3: 1.00±0.48 vs. 0.38±0.45, P<0.05). This result suggested that the levels of miR-33a-5p in serum, urine and kidney tissues decreased with the severity of renal injury and the progression of renal failure in patients with IgAN. Hence, miR-33a-5p detected in the serum and urine may be used as a non-invasive biomarker to reflect the progression of renal injury and renal failure in patients with IgAN.

Collagen type III is commonly detected in the renal interstitium and vasculature; however, it is absent in healthy glomeruli. Deposition of collagen type III in the glomerular mesangium and capillary basement membranes may arise in two rare diseases, namely collagen type III glomerulopathy (CG) and nail patella syndrome. CG is a rare glomerular disease with no specific treatment, although supportive measures for control of hypertension and edema may help to relieve symptoms. With progression to end-stage renal disease, patients with CG may come to require dialysis and/or renal transplantation. The present study reported on a 59-year-old male who was diagnosed with CG nephrotic syndrome by immunohistochemical and electron microscopic examination of biopsy material. To the best of our knowledge, this is the first case reported in northeastern China. The angiotensin II blocker telmisartan was successfully used to alleviate renal symptoms and a literature review was performed. The present case supports the use of telmisartan as a first choice of treatment for CG.

The characteristic features of diabetic nephropathy include thickening of the glomerular basement membranes, expansion of mesangium, and appearance of albumin in the urine (microalbuminuria and macroalbuminuria). Experimental studies have documented that 12-lipoxygenase (12-LOX) and its metabolite 12(S)-hydroxyeicosatetraenoic acid (HETE) play an important role in the pathogenesis of diabetic nephropathy. 12(S)-HETE may work in association with angiotensin II and transforming growth factor- β (TGF-β) reciprocally to induce fibrotic changes in the diabetic kidneys. The fibrotic actions of angiotensin II on the kidneys are mediated indirectly through an increase in the synthesis of 12(S)-HETE. Conversely, 12(S)-HETE may also enhance the actions of angiotensin II by upregulating the expression of AT1 receptors on the glomerulus, mesangium, and podocytes. 12(S)-HETE may also cross-talk with TGF-β in a reciprocal manner to induce the fibrotic changes in the diabetic kidney. 12(S)-HETE-triggered signaling pathways may involve activation of p38 mitogen-activated protein (p38MAP) kinase, increase in cAMP-responsive element-binding protein (CREB) transcriptional activity, epigenetic changes involving histone methylation through an increase in histone methyltransferase activity along with an upregulation of cyclin-kinase inhibitors including, p16, p21, and p27. The present review discusses the role of 12-LOX in the pathogenesis of diabetic nephropathy along with the possible mechanisms.

Hemodialysis (HD) is the most important treatment for patients with end‐stage renal disease (ESRD). Thrombocytopenia is a potential treatment complication related to dialysis. Under normal circumstances, the platelet count would slightly decrease within the first hour of HD, but get restored towards the end of procedure. In most patients, the platelet count can be maintained within the normal range, and the occurrence of thrombocytopenia is relatively rare in clinical practice. Therefore, the possibility of thrombocytopenia in HD patients is often ignored. Moreover, thrombocytopenia might be misdiagnosed and mistreated. At present, almost all articles on the subject, apart from some case reports, focus on pseudothrombocytopenia and heparin‐induced thrombocytopenia. In this review, we summarized various underlying causes, mechanisms, and diagnostic approaches to thrombocytopenia in HD patients. The review aims to provide a guide for clinicians interested in the causes and adequate treatment of thrombocytopenia.

IgA肾病（IgAN）是我国常见的原发性肾小球疾病（PGD）,约占PGD的45.26%,成人IgAN在10年内进展为终末期肾脏病（ESRD）比例为15%-20% [1] 。诸多因素参与IgAN进展,如高血压、大量蛋白尿等。文献报道伴高血压IgAN（IgAN-HBP）进展为ESRD的风险是不伴高血压IgAN（IgAN-NHBP）的2.5倍 [2] 。

Several studies have confirmed that the myeloid-derived suppressor cells (MDSCs) are closely associated with autoimmune diseases, but their exact role in these processes remains largely unclear. Here, we investigated the role MDSCs in patients with primary membranous nephropathy (PMN). Compared to healthy controls (HCs), PMN patients showed significantly increased number of HLA-DR-CD11b+CD33+ MDSCs in the peripheral blood, including both CD14+CD66b- monocytic and CD14-CD66b+ granulocytic MDSCs. The frequency of MDSCs was positively correlated with the level of serum anti-phospholipase A2 receptor (anti-PLA2R), 24-h urine protein quantification, and disease activity in PMN patients. Consistently, enhanced T helper 2 (Th2) and T helper 17 (Th17) immune responses were positively associated with plasma anti-PLA2R levels, 24-h urine protein quantification, and the disease activity in PMN patients. Moreover, compared to HCs, MDSCs from PMN patients exhibited significantly elevated arginase-1 (ARG-1) production and increased potential to promote Th17 differentiation in vitro in an ARG-1-dependent manner. This study directly demonstrates a pathogenic role for MDSCs in human PMN and provides a molecular mechanism for the pathogenesis of PMN. Our data show that MDSCs may promote PMN disease progression mainly by enhancing Th17 response. Therefore, MDSCs may be an important diagnostic, therapeutic, and prognostic marker for PMN diseases.

Background: The prevalence of Idiopathic Membranous Nephropathy (IMN) among patients with Primary Glomerular Disease (PGD) has increased in China. Objectives: This cross-sectional studyaimed to investigate changes in the clinicopathological features associated with IMN in northeast China. Methods: We analyzed clinical and pathological data of 1,198 patients with IMN among 4,083 patients hospitalized with PGD undergoing renal biopsy from January 1, 2005, to December 31, 2018 at the Second Hospital of Jilin University in northeast China. Enrollees were divided into two groups: those seen from 2005 to 2011 (Group 1) and those seen from 2012 to 2018 (Group 2). We compared the clinicopathological features between Group 1 and Group 2. Results: The percentage of patients with IMN among those with PGD increased over the 14 years of the study (P = 0.001). The male-to-female ratio, prevalence of microscopic hematuria, and proteinuria levels were higher in Group 2 than in Group 1 (P < 0.05). Among those with PGD, the percentages of patients with IMN-total, IMN-I, IMN-II, and IMN-III+IV, as well as Tubulointerstitial Lesion (TIL) scores, were higher in Group 2 than in Group 1 (P < 0.05). Finally, age (P < 0.001), degree of proteinuria (P < 0.001), and serum triglyceride level (P = 0.027) were significantly associated with TIL scores among patients with IMN. Conclusions: Membranous nephropathy is now the leading cause of PGD, with increased TIL scores during the study period. Advanced age, high proteinuria, and serum triglyceride levels represent the independent risk factors for severe TIL among patients with IMN.

Nephrotic syndrome, a rare complication of secondary syphilis and syphilitic minimal change disease, has only been fully documented in one case thus far. The current study describes two cases of nephrotic syndrome with minimal change disease due to syphilis infection. One case was of a 15-year-old boy while the other was a 47-year-old male. Both patients showed renal pathologic characteristics of minimal change disease and both were positive for the rapid plasma reagin (RPR). Prolonged antibiotic therapy with penicillin or ceftezole resulted in full remission in the two cases. In conclusion, it is important to recognize that syphilis is a reversible cause of nephrotic syndrome with minimal change disease. Antibiotic therapy can result in complete remission.