Wei Wang

Background: Anti‐glomerular basement membrane (anti‐GBM) disease complicated by thrombotic microangiopathy (TMA) is rare and clinically challenging. In the setting of severe infection and active autoimmune disease, complement activation may reflect a secondary process rather than primary atypical hemolytic uremic syndrome (aHUS). Therapeutic plasma exchange (TPE) is standard therapy for anti‐GBM disease, but alternative antibody removal strategies are required when TPE is not tolerated. Case Presentation: We describe a 46‐year‐old female with anti‐GBM disease who developed TMA with preserved ADAMTS13 activity (72.04%) and 2.3% schistocytes on peripheral smear. Soluble C5b‐9 was elevated in the context of severe concurrent infection. TPE was discontinued due to intolerance (oxygen desaturation, fever, and dizziness). Seven sessions of protein A immunoadsorption (PAIA) were performed for selective antibody removal, and anti‐GBM antibody levels decreased from > 400 to 20.80 RU/mL during treatment. Eculizumab was administered on Days 7, 14, and 30, for persistent complement activation. Hematologic and biochemical parameters stabilized and gradually improved during combined therapy. Conclusion: This case suggests that PAIA may represent a feasible alternative antibody removal strategy in anti‐GBM disease intolerant to plasma exchange. In selected cases of secondary complement‐mediated TMA with ongoing complement activation, short‐term complement inhibition may contribute to disease stabilization; however, a definitive causal relationship cannot be established in a single case.

Background: Long-term antigen-specific data in PMN among Chinese populations remain limited. This study evaluated six target antigens and their clinical significance during extended follow-up. Methods: We retrospectively analyzed 132 treatment-naïve PMN patients diagnosed by biopsy (2010–2018) and followed for a median of 62.9 months. Renal tissue expression of PLA2R, THSD7A, NELL-1, PCDH7, EXT1, and EXT2 was assessed by immunohistochemistry, and serum anti-PLA2R antibodies were measured by ELISA. Associations between antigen profiles and 5-year outcomes (remission, renal survival, malignancy) were evaluated. Results: PLA2R was the predominant antigen (84.1%), followed by THSD7A (5.3%) and NELL-1 (0.76%); no PCDH7, EXT1, or EXT2 positivity was detected. PLA2R-negative patients were more often female (71.4% vs. 36.0%, P = 0.003), with better renal function and more frequent C1q deposition (38.1% vs. 13.5%, P = 0.016). Serum anti-PLA2R antibodies were detected in 55.3% of patients and strongly correlated with tissue PLA2R positivity (AUC = 0.851; optimal cutoff ≥17.47 RU/mL). Baseline antibody titers were not associated with remission ( P = 0.573). During 5-years follow-up, 42.4% achieved CR, 36.4% PR, and 21.2% had NR, with an estimated 5-year renal survival rate of 81.95%. No malignancy events were observed among the seven THSD7A-positive patients or the single NELL-1–positive patient in this cohort. Statistical power for rare antigen subgroups was limited. Conclusions: This >5-year Chinese PMN cohort provides the first comprehensive analysis of six target antigens. PLA2R remains predominant, while PLA2R-negative patients distinct immunopathologic features yet favorable long-term outcomes. A population-specific anti-PLA2R cutoff showed good diagnostic performance for predicting tissue antigen deposition. Rare antigens were infrequent and their malignancy associations require cautious interpretation. These findings provide long-term antigen-specific data supporting antigen-guided, population-adapted precision management of PMN.

Rationale: The coexistence of Guillain–Barré syndrome (GBS) and minimal change disease (MCD) is extremely rare. GBS is an autoimmune-mediated peripheral neuropathy that can occasionally be associated with renal complications such as nephrotic syndrome (NS). This case discusses a patient diagnosed with both GBS and MCD, as well as Sjögren syndrome kidney injury, focusing on the potential pathogenesis of these conditions and the role of autoantibodies in their development and treatment outcomes. Patient concerns: A young female patient presented with progressively worsening muscle weakness, sensory abnormalities, and edema. Further investigations revealed NS, characterized by proteinuria and hypoalbuminemia. While the neurological symptoms improved initially, the renal manifestations persisted, raising concerns about ongoing kidney damage. In addition, the patient was also found to have Sjögren syndrome kidney injury, along with positive perinuclear antineutrophil cytoplasmic antibody and antinuclear antibody, suggesting an autoimmune-mediated process contributing to the co-occurrence of these conditions. Diagnoses: GBS was diagnosed based on characteristic ascending paralysis and demyelination, as evidenced by nerve conduction studies. The diagnosis of MCD was supported by the patient’s clinical presentation of NS and kidney biopsy findings. The presence of clinical features such as dry mouth and dry eyes, coupled with positive anti-SSA/Ro52 and anti–Sjögren syndrome antigen B antibodies, pointed to Sjögren syndrome. Kidney biopsy results strongly suggested that kidney damage was likely due to Sjögren syndrome. Interventions: The patient was started on immunosuppressive therapy, including prednisone and cyclophosphamide, to address both the autoimmune neuropathy and renal issues. In addition, intravenous immunoglobulin was administered to treat the GBS. Supportive therapies, such as diuretics and albumin infusions, were used to manage edema and protein loss associated with NS. Outcomes: The patient showed significant improvement in neurological symptoms, including enhanced muscle strength and reduced sensory deficits. Proteinuria decreased, and renal function gradually stabilized. Lessons: This case illustrates the rare coexistence of GBS, MCD, and Sjögren syndrome kidney injury in a single patient. Autoimmune markers played a pivotal role in the pathogenesis of these diseases. Immunosuppressive therapy and intravenous immunoglobulin were essential in treating both neurological and renal complications. Further research is needed to deepen our understanding of the overlap of autoimmune diseases and to optimize treatment strategies for such complex cases.

The factors influencing diffuse crescentic glomerulonephritis renal survival and prognosis remain uncertain. Additionally, there’s no literature on the clinical outcomes of IgA nephropathy, lupus nephritis, and IgA vasculitis nephritis in type II patients. This study retrospectively examined 107 patients diagnosed with diffuse crescentic glomerulonephritis through biopsy. Analytical methods included Cox regression models and Kaplan-Meier survival analysis to assess the data. Among the 107 enrolled patients, 12 patients had Type I diffuse crescentic glomerulonephritis, 70 patients had Type II, and 25 patients had Type III. The respective 5-year kidney survival rates were 0%, 57.5%, and 18.6% for type I, type II, and type III. Furthermore, among Type II patients, IgA nephropathy emerged as the most prevalent condition. The cumulative 5-year kidney survival rates were 50% for patients with IgA nephropathy, 64% for lupus nephritis, and 70% for Henoch-Schönlein purpura nephritis. A significant association between the risk of ESKD development and several factors was revealed by a multivariate Cox regression analysis: estimated glomerular filtration rate (P = 0.004), initial kidney replacement therapy (KRT) at presentation (P = 0.002), global glomerulosclerosis (P<0.001). Type II diffuse crescentic glomerulonephritis was the most prevalent type in DCGN, and favors better renal prognosis than type I and III DCGN, in which IgA nephropathy was the most common entity of Type II DCGN. Additionally, estimated glomerular filtration rate, initial KRT at presentation and global glomerulosclerosis were identified as predictors of renal outcomes in diffuse crescentic glomerulonephritis.

Aim: To investigate the clinicopathological characteristics and prognosis among various types of diffuse crescentic glomerulonephritis(DCGN) and especially type II DCGN and to identify predictive factors for renal outcomes in these patients. Methods: This study retrospectively examined 107 patients diagnosed with diffuse crescentic glomerulonephritis through biopsy at the Sichuan Provincial People's Hospital, spanning from January 2012 to July 2022. The investigation primarily aimed to identify the occurrence of end-stage kidney disease (ESKD) as the primary endpoint. Analytical methods included Cox regression models and Kaplan-Meier survival analysis to assess the data. Results: Among the 107 enrolled patients, 12 (11.2%) patients had Type I diffuse crescentic glomerulonephritis, 70 (65.4%) patients had Type II (including immunoglobulin A [IgA] nephropathy, 40%; lupus nephritis, 38.6%), and 25 (23.4%) patients had Type III. Thirty-seven (34.6%) patients received kidney replacement therapy (KRT) at presentation, while 17 (15.9%) received plasmapheresis. In the follow-up cohort, 46 (47%) patients progressed to ESKD. For patients with diffuse crescentic glomerulonephritis (DCGN), the 5-year cumulative renal survival rates were markedly different across the three types: Type I had a rate of 0%, Type II had a rate of 57.5%, and Type III had a rate of 18.6%. Furthermore, among Type II patients, IgA nephropathy emerged as the most prevalent condition. The cumulative 5-year kidney survival rates were 50% for patients with IgA nephropathy, 64% for lupus nephritis, and 70% for Henoch-Schönlein purpura nephritis. A significant association between the risk of ESKD development and several factors was revealed by a multivariate Cox regression analysis: baseline serum creatinine level (P=0.001), initial kidney replacement therapy (KRT) at presentation (P=0.003), interstitial inflammation (P=0.023), global glomerulosclerosis (P=0.009), and the presence of fibrous crescents over 50% (P=0.033). Conclusion: Type II diffuse crescentic glomerulonephritis was the most prevalent type in DCGN, and favors better renal prognosis than type I and III DCGN, in which IgA nephropathy was the most common entity of Type II DCGN in western China. The prognosis for IgA nephropathy was worse than that for patients with lupus nephritis or Henoch-Schönlein purpura nephritis. Additionally, baseline serum creatinine level, initial KRT at presentation, interstitial inflammation, global glomerulosclerosis, and fibrous crescents >50% were identified as predictors of renal outcomes in diffuse crescentic glomerulonephritis .

Introduction: The objective was to evaluate the poisoning severity score (PSS) as an early prognostic predictor in patients with wasp stings and identify associated clinical characteristics and risk factors for mortality. Methods: A total of 363 patients with wasp stings at Suining Central Hospital between January 2016 and December 2018 were enrolled. Within the first 24 h of admission, the poisoning severity score (PSS) and the Chinese expert consensus on standardized diagnosis and treatment of wasp stings (CECC) were utilized for severity classification, and their correlation was examined. Patients were then divided into survival and death groups based on discharge status. Logistic regression analysis was employed to analyze factors influencing patients’ outcomes. Results: The mortality of wasp sting patients was 3.9%. The PSS and CECC were found to correlate for severity classification. Additionally, female gender, age, number of stings, and PSS were identified as independent risk factors for mortality in wasp sting patients. Combining these four factors yielded an AUC of 0.962 for predicting death. Conclusions: PSS aids in early severity classification of wasp stings. Female gender, age, number of stings, and PSS were independent mortality risk factors in these patients.

Background: It is still controversial whether the proportion of crescents below 50% can be an independent predictive risk factor for poor prognosis in IgAN patients. We reported the significance of different proportions of crescents on the clinical features and the cut-off value of crescents in predicting the occurrence of end-stage kidney disease (ESKD) in patients with IgAN.Methods: We retrospectively analyzed biopsy-proven primary IgAN patients in Sichuan Provincial People’s Hospital from 2007 to 2019. The patients were divided into 5 groups on the basis of crescent proportion as follows: 0 (n = 647), < 10% (n = 221), 10 to 24% (n = 272), 25 to 49% (n = 80), and ≥50% (n = 22). The primary endpoint was defined as ESKD, and the secondary endpoint was the combined renal endpoint (≥50% reduction in eGFR or ESKD). A validation cohort of 346 patients were enrolled from Affiliated Hospital of Southwest Medical University. Cox regression model and Kaplan-Meier survival analysis were performed.Results: A total of 1242 eligible patients with biopsy-proven IgAN were recorded in the database, compared with the non-crescent group, patients in the crescent group had lower levels of hemoglobin (Hb) and albumin (Alb), higher levels of blood urea nitrogen (BUN), 24h urinary protein and hematuria, a higher proportion of mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), and tubular atrophy/interstitial fibrosis (T1/T2) (p < 0.05). A higher crescent proportion was associated with lower levels of Hb, ALB, eGFR and serum IgG (p < 0.05), higher levels of SCr, BUN, increasing amounts of 24 h urinary protein, increasing proportion of M1 and E1, and increasing severity of interstitial inflammatory infiltration. During the median follow-up of 43 months (range 6-151), 63 individuals (7.0%) reached the primary outcome of ESKD and 99 patients (11.1%) reached the combined renal endpoint. 34(7.5%), 21 (13.3%), 24(12.2%), 14(21.5%) and 6(31.6%) patients reached the combined renal endpoint in the above five groups in crescents 0, p = 0.003) and crescents ≥25% was an independent risk factor for the combined renal endpoint(p < 0.001). The receiver operating characteristic curve showed that IgAN patients with crescents ≥43.7% had a higher risk of ESKD, even with immunosuppressants (Sensitivity = 75.7%,specificity = 89.6%,p < 0.001). This discovery cohort and the validation cohort further confirmed that patients with crescents p < 0.001).Conclusion: IgAN patients with crescents had more severe clinicopathological features and poorer prognosis. Crescents ≥50% was an independent risk factor for the progression of ESKD and crescents ≥25% was an independent risk factor for ≥50% reduction in eGFR or ESKD in treated and untreated IgAN patients. Crescents ≥43.7% was an independent risk factor for ESKD in those with immunosuppressants.

Background: We aimed to assess the utility of the poisoning severity score (PSS) as early prognostic predictors in patients with wasp stings, and to explore a reliable and simple predictive tool for short-term outcomes.Methods: From January 2016 to December 2018, 363 patients with wasp stings in Suining Central Hospital were taken as research subjects. In the first 24h of hospital admission, the PSS and Chinese expert consensus on standardized diagnosis and treatment of wasp stings (CECC) were used as the criterion for severity classification, and their correlation was analyzed. The patients were divided into survival and death groups according to the state of discharge. The factors that affect outcome were analyzed by logistic regression analysis. A clinical prognostic model of death was constructed according to the risk factors, and 1000 times repeated sampling was done to include the data to verify the model internally.Results: The mortality of wasp sting patients was 3.9%. There was a correlation between PSS and CECC (r=0.435, PPConclusions: PSS is helpful in the early classification of the severity of wasp stings. Sex, age, number of stings, and PSS were independent risk factors for death in wasp sting patients. The nomogram model established in this study can accurately predict the occurrence of the risk of death.

An amendment to this paper has been published and can be accessed via the original article.