Coexistence of anti-glomerular basement membrane disease and IgA nephropathy: an illustrative case and comprehensive literature review.

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune condition characterized by the presence of positive anti-GBM autoantibodies, linear deposition of immunoglobulin G (IgG) along the GBM and severe kidney injury. In a limited number of cases, the association of anti-GBM disease with other glomerulonephritis has been reported. Herein, we present the case of a 66-year-old female patient with progressive worsen kidney function and decreased urine output. A renal biopsy revealed crescent glomerulonephritis with lineal IgG deposition along the GBM and mesangial IgA deposition, which supported the diagnosis of concurrent anti-GBM disease and IgA nephropathy (IgAN). In an extensive literature review, we identified a total of thirty-nine patients were reported anti-GBM disease combined with IgAN. The clinical characteristics of these patients demonstrate that the anti-GBM disease combined with IgAN tends to be milder with a more indolent course and a better prognosis than the classic anti-GBM disease, and its potential pathogenesis deserves to be further explored.

The SOX4/EZH2/SLC7A11 signaling axis mediates ferroptosis in calcium oxalate crystal deposition-induced kidney injury.

Epigenetic regulation is reported to play a significant role in the pathogenesis of various kidney diseases, including renal cell carcinoma, acute kidney injury, renal fibrosis, diabetic nephropathy, and lupus nephritis. However, the role of epigenetic regulation in calcium oxalate (CaOx) crystal deposition-induced kidney injury remains unclear. Our study demonstrated that the upregulation of enhancer of zeste homolog 2 (EZH2)-mediated ferroptosis facilitates CaOx-induced kidney injury. CaOx crystal deposition promoted ferroptosis in vivo and in vitro. Usage of liproxstatin-1 (Lip-1), a ferroptosis inhibitor, mitigated CaOx-induced kidney damage. Single-nucleus RNA-sequencing, RNA-sequencing, immunohistochemical and western blotting analyses revealed that EZH2 was upregulated in kidney stone patients, kidney stone mice, and oxalate-stimulated HK-2 cells. Experiments involving in vivo EZH2 knockout, in vitro EZH2 knockdown, and in vivo GSK-126 (an EZH2 inhibitor) treatment confirmed the protective effects of EZH2 inhibition on kidney injury and ferroptosis. Mechanistically, the results of RNA-sequencing and chromatin immunoprecipitation assays demonstrated that EZH2 regulates ferroptosis by suppressing solute carrier family 7, member 11 (SLC7A11) expression through trimethylation of histone H3 lysine 27 (H3K27me3) modification. Additionally, SOX4 regulated ferroptosis by directly modulating EZH2 expression. Thus, this study demonstrated that SOX4 facilitates ferroptosis in CaOx-induced kidney injury through EZH2/H3K27me3-mediated suppression of SLC7A11.

A pilot study of intensive short-time continuous renal replacement therapy to substitute maintenance hemodialysis during transitional period of COVID-19.

INTRODUCTION: There is a lack of perfect solutions for maintenance hemodialysis (MHD) in patients with a high transmission risk of SARS-CoV-2. METHODS: MHD patients with a high risk of SARS-CoV-2 transmission from April 1 to June 30, 2022, were recruited. We performed 4-h continuous renal replacement therapy with Prismaflex dialysis machine and ST100 suite using continuous venovenous hemodiafiltration (CVVHDF) mode with a fluid exchange volume of 8000 mL/h. RESULTS: Forty-five MHD patients were included with a median dialysis age of 91 months. Overall spKt/V reached 0.96 ± 0.19. Urea reduction ratio was 50.29 ± 7.60% with the ultrafiltration of 2.18 ± 0.79 kg. Dry weight was significantly inversely correlated with spKt/V (R = -0.563, p < 0.001). Female gender was a significant positive factor of spKt/V. Preheating of replacement solution using an incubator solved the complication of shivering in most patients. CONCLUSION: Intensive short-time CVVHDF may be considered as an alternative for routine MHD during COVID-19 transitional period.

Plant-derived compounds for treating autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic hereditary kidney disease, is the fourth leading cause of end-stage kidney disease worldwide. In recent years, significant progress has been made in delaying ADPKD progression with different kinds of chemical drugs, such as tolvaptan, rapamycin, and somatostatin. Meanwhile, numerous plant-derived compounds have been investigated for their beneficial effects on slowing ADPKD progression. Among them, saikosaponin-d, Ganoderma triterpenes, curcumin, ginkgolide B, steviol, resveratrol, Sparganum stoloniferum Buch.-Ham, Cordyceps sinensis, triptolide, quercitrin, naringin, cardamonin, gambogic acid, and olive leaf extract have been found to retard renal cyst development by inhibiting cell proliferation or promoting cell apoptosis in renal cyst-lining epithelial cells. Metformin, a synthesized compound derived from French lilac or goat's rue (Galega officinalis), has been proven to retard the progression of ADPKD. This review focuses on the roles and mechanisms of plant-derived compounds in treating ADPKD, which may constitute promising new therapeutics in the future.

Misdiagnosis of renal pelvic unicentric Castleman disease: a case report.

Castleman disease is a rare heterogeneous lymphoproliferative disorder of unknown etiology. Unicentric Castleman disease (UCD) is more common. UCD can occur at any site where lymphatic tissue exists, most commonly in the mediastinum, neck, and abdominal cavity, etc. in the current study, we reported a 46-year-old woman, who has left low back pain and discomfort. Magnetic resonance imaging (MRI) of the kidneys showed the left renal pelvis was occupied, left hydronephrosis, and the left renal hilum and retroperitoneal lymph nodes were enlarged. Enhanced kidney CT showed that the "pelvic tumor" was moderately enhanced in the bottom part in corticomedullary phase, while in nephrogenic phase, it was unevenly enhanced with a highly enhanced bottom part and weakly enhanced upper part. In excretory phase, reinforcement was decreased. "left renal pelvis tumor" was diagnosed and she underwent surgical treatment with left nephrectomy. However, histopathological examination indicated the UCD. We suggest that for renal pelvic tumors having imaging characteristics of homogeneous soft tissue density and heterogeneous CT enhancement, the hyaline vascular type of UCD could be taken into consideration for differential diagnosis.

Identification of distinct clinical phenotypes of cardiogenic shock using machine learning consensus clustering approach.

BACKGROUND: Cardiogenic shock (CS) is a complex state with many underlying causes and associated outcomes. It is still difficult to differentiate between various CS phenotypes. We investigated if the CS phenotypes with distinctive clinical profiles and prognoses might be found using the machine learning (ML) consensus clustering approach. METHODS: The current study included patients who were diagnosed with CS at the time of admission from the electronic ICU (eICU) Collaborative Research Database. Among 21,925 patients with CS, an unsupervised ML consensus clustering analysis was conducted. The optimal number of clusters was identified by means of the consensus matrix (CM) heat map, cumulative distribution function (CDF), cluster-consensus plots, and the proportion of ambiguously clustered pairs (PAC) analysis. We calculated the standardized mean difference (SMD) of each variable and used the cutoff of ± 0.3 to identify each cluster's key features. We examined the relationship between the phenotypes and several clinical endpoints utilizing logistic regression (LR) analysis. RESULTS: The consensus cluster analysis identified two clusters (Cluster 1: n = 9,848; Cluster 2: n = 12,077). The key features of patients in Cluster 1, compared with Cluster 2, included: lower blood pressure, lower eGFR (estimated glomerular filtration rate), higher BUN (blood urea nitrogen), higher creatinine, lower albumin, higher potassium, lower bicarbonate, lower red blood cell (RBC), higher red blood cell distribution width (RDW), higher SOFA score, higher APS III score, and higher APACHE IV score on admission. The results of LR analysis showed that the Cluster 2 was associated with lower in-hospital mortality (odds ratio [OR]: 0.374; 95% confidence interval [CI]: 0.347-0.402; P < 0.001), ICU mortality (OR: 0.349; 95% CI: 0.318-0.382; P < 0.001), and the incidence of acute kidney injury (AKI) after admission (OR: 0.478; 95% CI: 0.452-0.505; P < 0.001). CONCLUSIONS: ML consensus clustering analysis synthesized the pattern of clinical and laboratory data to reveal distinct CS phenotypes with different clinical outcomes.

Influence of SGLT2i and RAASi and Their Combination on Risk of Hyperkalemia in DKD: A Network Meta-Analysis.

BACKGROUND: This network meta-analysis investigated the effect of various combined regimens of sodium-glucose cotransporter-2 inhibitors (SGLT2is) and renin-angiotensin-aldosterone system inhibitors (RAASis) on the occurrence of hyperkalemia in diabetic kidney disease. METHODS: The risk of hyperkalemia was compared using the random-effects model of network meta-analysis, with results expressed as odds ratios (ORs) with 95% confidence intervals (CIs). The comparative effects of all medications and their combinations with placebo were ranked using the surface under the cumulative ranking probabilities. RESULTS: In total, 27 eligible studies involving 43,589 participants with diabetic kidney disease were included. Major findings showed that the use of mineralocorticoid receptor antagonists (MRAs) on top of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) prominently increased hyperkalemia incidence when compared with placebo (OR, 6.08; 95% CI, 2.30 to 16.08), ACEI (OR, 3.07; 95% CI, 1.14 to 8.31), ARB (OR, 2.57; 95% CI, 1.10 to 6.02), SGLT2i (OR, 9.22; 95% CI, 2.99 to 28.46), renin inhibitors+ACEI/ARB (OR, 2.23; 95% CI, 1.14 to 4.36), or SGLT2i+ACEI/ARB (OR, 4.10; 95% CI, 2.32 to 7.26). Subgroup analysis among different generations of MRA found that spironolactone had the strongest effect in combination with ACEI/ARB, even higher than the combined use of ACEI and ARB (OR, 2.89; 95% CI, 1.26 to 6.63). In addition, SGLT2i had a significantly lower incidence of hyperkalemia compared with ACEI (OR, 0.33; 95% CI, 0.12 to 0.91), ARB (OR, 0.28; 95% CI, 0.13 to 0.61), dual RAASi (ACEI combined with ARB; OR, 0.17; 95% CI, 0.06 to 0.47), or MRA or renin inhibitors combined with ACEI/ARB (OR, 0.11; 95% CI, 0.04 to 0.33; OR, 0.24; 95% CI, 0.08 to 0.76, respectively). Moreover, adding SGLT2i to the combination of MRA and ACEI/ARB, as well as the combinations of different RAASis, markedly reduced the occurrence of hyperkalemia. CONCLUSIONS: Among the therapeutic drugs with the potential risk of increasing serum potassium in patients with diabetic kidney disease, MRA added an extra risk of hyperkalemia while SGLT2i had the opposite effect and could even reverse the elevation of serum potassium caused by the combined regimen, including MRAs.

Histone H3K27 methyltransferase EZH2 regulates apoptotic and inflammatory responses in sepsis-induced AKI.

Rationale: The role of histone methylation modifications in renal disease, particularly in sepsis-induced acute kidney injury (AKI), remains unclear. This study aims to investigate the potential involvement of the histone methyltransferase zeste homolog 2 (EZH2) in sepsis-induced AKI and its impact on apoptosis and inflammation. Methods: We first examined the expression of EZH2 in the kidney of sepsis-induced AKI (LPS injection) mice and LPS-stimulated tubular epithelial cells. We next constructed the EZH2 knockout mice to further confirm the effects of EZH2 on apoptosis and inflammatory response in AKI. And the inflammatory level of epithelial cells can be reflected by detecting chemokines and the chemotaxis of macrophages. Subsequently, we constructed the EZH2 knocked-down cells again and performed Chromatin Immunoprecipitation sequencing to screen out the target genes regulated by EZH2 and the enrichment pathway. Then we confirmed the EZH2 target gene and its regulatory pathway in vivo and in vitro experiments. Experimental results were finally confirmed using another in vivo model of sepsis-induced AKI (cecal perforation ligation). Results: The study found that EZH2 was upregulated in sepsis-induced AKI and that silencing EZH2 could reduce renal tubular injury by decreasing apoptosis and inflammatory response of tubular epithelial cells. EZH2 knockout mice showed significantly reduced renal inflammation and macrophage infiltration. Chromatin immunoprecipitation sequencing and polymerase chain reaction identified Sox9 as a target of EZH2. EZH2 was found to be enriched on the promoter of Sox9. Silencing EZH2 resulted in a significant increase in the transcriptional level of Sox9 and activation of the Wnt/ß-catenin signaling pathway. The study further reversed the effects of EZH2 silencing by silencing Sox9 or administering the Wnt/ß-catenin inhibitor icg001. It was also found that Sox9 positively regulated the expression of ß-catenin and its downstream pathway-related genes. Finally, the study showed that the EZH2 inhibitor 3-deazaneplanocin A significantly alleviated sepsis-induced AKI. Conclusion: Our results indicate that silencing EZH2 can protect renal function by relieving transcriptional inhibition of Sox9, activating the Wnt/ß-catenin pathway, and attenuating tubular epithelial apoptosis and inflammatory response of the renal interstitium. These results highlight the potential therapeutic value of targeting EZH2 in sepsis-induced AKI.

Thrombotic thrombocytopenic purpura developed after pegylated interferon treatment for hepatitis B infection.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia. It is related to severe deficiency in ADAMTS13, which is usually acquired via ADAMTS13 autoantibodies or inherited via mutations of the ADAMTS13 gene. The etiology of acquired TTP including HIV infection, pregnancy, autoimmune disease, organ transplantation, drugs, malignancy and so on. Here, we firstly reported a patient diagnosed as acquired TTP after pegylated interferon therapy for hepatitis B and COVID-19 vaccination. CASE PRESENTATION: A 36-year-old male attended to our unit with a five-day history of intermittent hematuria and progressive fatigue on January 5th, 2022. He had a 13 years history of hepatitis B infection and undergone pegylated interferon treatment (which was paused for two months because of COVID-19 vaccination) for nearly 3 years. Laboratory evaluation revealed a haemoglobin level of 61 g/L, platelet count of 11 × 109/L, lactate dehydrogenase 2133 U/L. The direct and indirect Coombs test were both negative. On a peripheral blood smear, there were about 18.8% schistocytes. Meanwhile, the results of ADAMTS 13 activity and antibody were < 5% and 181.34 ng/ml (131.25-646.5), respectively CONCLUSION: This case firstly reported the rare complication of TTP after pegylated interferon treatment for hepatitis B and COVID-19 vaccine injection. This unique sign warrants more attention as an early cue of diagnosis of TTP and be aware of the rarity adverse effect of interferon therapy and COVID-19 vaccination.

A Nomogram to Identify Hyperkalemia Risk in Patients with Advanced CKD.

Background: Hyperkalemia is a common and life-threatening complication of CKD. We aimed to develop and validate a nomogram that could identify the risk of hyperkalemia (≥5.5 mmol/L) in patients with CKD. Methods: A retrospective cohort study was performed in adult patients with predialysis advanced CKD (stages ≥3) in 2020-2021 for the outcome of hyperkalemia within 6 months. The training set was used to identify risk factors of hyperkalemia. Then a nomogram was developed by multivariable logistic regression analysis. C-statistics, calibration curves, and decision curve analysis (DCA) were used, and the model was validated in the internal and two external validation sets. Results: In total, 847 patients with advanced CKD were included. In 6 months, 28% of patients had hyperkalemia (234 out of 847). Independent risk factors were: age ≥75 years, higher CKD stages, previous event of serum potassium ≥5.0 mmol/L within 3 months, and comorbidities with heart failure, diabetes, or metabolic acidosis. Then the nomogram on the basis of the risk factors adding the use of renin-angiotensin-aldosterone system inhibitors was constructed. The C-statistic of the model was 0.76 (95% CI, 0.70 to 0.78), and was stable in both the internal validation set (0.73; 95% CI, 0.63 to 0.82) and external validation sets (0.88; 95% CI, 0.84 to 0.95 and 0.82; 95% CI, 0.72 to 0.92). Calibration curves and DCA analysis both found good performances of the nomogram. Conclusion: A feasible nomogram and online calculator were developed and validated to evaluate the risk of hyperkalemia within 6 months in patients with advanced CKD. Patients with CKD and a high risk of hyperkalemia may benefit from intensive monitoring and early triage.

p-Cresyl sulfate predicts clinical outcomes in sustained peritoneal dialysis: a 5-year follow-up cohort study and meta-analysis.

BACKGROUND: The impact of p-cresyl sulfate (PCS) and indoxyl sulfate (IS) on the prognosis of patients with uremia remains controversial. We performed a prospective study on peritoneal dialysis (PD) to investigate the relationship between PCS or IS levels with clinical outcomes. METHODS: This prospective cohort study investigated the association of serum PCS and IS with clinical outcomes in patients undertaking PD. We performed a correlations analysis to explore the influencing factors of PCS an IS. Meta-analysis was conducted to objectively evaluate the prognostic effects of PCS and IS on different stages of CKD patients. RESULTS: A total of 127 patients were enrolled consecutively and followed with an average period of 51.3 months. Multivariate Cox regression showed that serum total PCS not only contributed to the occurrence of PD failure event (HR: 1.05, 95% CI = 1.02 to 1.07, p < 0.001), but also increased the risk of cardiovascular event (HR: 1.08, 95% CI = 1.04 to 1.13, p < 0.001) and PD-associated peritonitis (HR: 1.04, 95% CI = 1.02 to 1.08, p = 0.001). Dividing the total PCS level by 18.99 mg/L, which was calculated from the best cutoff value of the ROC curve, patients with total PCS higher than 18.99 mg/L had worse prognosis. Meta-analysis confirmed its value in cardiovascular event in PD. CONCLUSION: The serum total PCS concentration was a detrimental factor for higher PD failure event, cardiovascular event, and PD-associated peritonitis. It could be used as an innovative marker in predicting poor clinical outcome in PD.

Chromatin Methylation Abnormalities in Autosomal Dominant Polycystic Kidney Disease.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide and is one of the major causes of end-stage renal disease. PKD1 and PKD2 are two genes that mainly contribute to the development and progression of ADPKD. The precise mechanism is not fully understood. In recent years, epigenetic modification has drawn increasing attention. Chromatin methylation is a very important category of PKD epigenetic changes and mostly involves DNA, histone, and RNA methylation. Genome hypomethylation and regional gene hypermethylation coexist in ADPKD. We found that the genomic DNA of ADPKD kidney tissues showed extensive demethylation by whole-genome bisulphite sequencing, while some regional DNA methylation from body fluids, such as blood and urine, can be used as diagnostic or prognostic biomarkers to predict PKD progression. Histone modifications construct the histone code mediated by histone methyltransferases and contribute to aberrant methylation changes in PKD. Considering the complexity of methylation abnormalities occurring in different regions and genes on the PKD epigenome, more specific therapy aiming to restore to the normal genome should lead to the development of epigenetic treatment.

Susceptibility of renal fibrosis in diabetes: Role of hypoxia inducible factor-1.

Diabetes may prevent kidney repair and sensitize the kidney to fibrosis or scar formation. To test this possibility, we examined renal fibrosis induced by unilateral ureteral obstruction (UUO) in diabetic mouse models. Indeed, UUO induced significantly more renal fibrosis in both Akita and STZ-induced diabetic mice than in nondiabetic mice. The diabetic mice also had more apoptosis and interstitial macrophage infiltration during UUO. In vitro, hypoxia induced higher expression of the fibrosis marker protein fibronectin in high glucose-conditioned renal tubular cells than in normal glucose cells. Mechanistically, hypoxia induced significantly more hypoxia-inducible factor-1 α (HIF-1 α) in high glucose cells than in normal glucose cells. Inhibition of HIF-1 attenuated the expression of fibronectin induced by hypoxia in high-glucose cells. Consistently, UUO induced significantly higher HIF-1α expression along with fibrosis in diabetic mice kidneys than in nondiabetic kidneys. The increased expression of fibrosis induced by UUO in diabetic mice was diminished in proximal tubule-HIF-1α-knockout mice. Together, these results indicate that diabetes sensitizes kidney tissues and cells to fibrogenesis probably by enhancing HIF-1 activation.

In-center Nocturnal Hemodialysis Reduced the Circulating FGF23, Left Ventricular Hypertrophy, and All-Cause Mortality: A Retrospective Cohort Study.

Fibroblast growth factor 23(FGF23) is the most important biomarker and pathogenic factor in Chronic Kidney Disease-Mineral and Bone Disorder (CKD-MBD). In the moderate and severe stages of chronic renal failure, abnormally elevated circulating FGF23 can lead to some complications, including myocardial hypertrophy, which is positively correlated with all-cause mortality. However, the circulating FGF23 level of different hemodialysis modalities, the underlying essential regulatory factors, and potential clinical benefits remain to be elucidated. In this retrospective cohort study, 90 in-center nocturnal hemodialysis (INHD) and 90 matched conventional hemodialysis (CHD) patients were enrolled. The complete blood count, intact FGF23(iFGF23), calcium, phosphorus, PTH, and other biochemical and echocardiographic parameters of INHD and CHD patients were collected and analyzed at 1-year follow-up. The all-cause mortality was recorded during the 7-year follow-up. Furthermore, the regulatory factors of iFGF23 and its association with echocardiographic parameters and mortality were investigated by multivariate regression. The levels of iFGF23 and serum phosphate in patients undergoing INHD were significantly lower than those in patients undergoing CHD. The left ventricular volume index (LVMI) in patients with INHD was significantly attenuated and positively correlated with the drop of serum iFGF23. The INHD group had reduced all-cause mortality compared to the CHD group. Multivariate analysis showed that iFGF23 was positively correlated with serum calcium, serum phosphorus, and calcium-phosphate product. The calcium-phosphate product is an independent determining factor of serum iFGF23. Compared with the CHD group, the INHD group presented with a significantly reduced circulating iFGF23 level, which was closely associated with attenuation of left ventricular hypertrophy, but INHD reduced all-cause mortality in an FGF23 independent manner.

Ligustilide ameliorates hippocampal neuronal injury after cerebral ischemia reperfusion through activating PINK1/Parkin-dependent mitophagy.

BACKGROUND: Mitophagy plays a critical role in cerebral ischemia/reperfusion by timely removal of dysfunctional mitochondria. In mammals, PINK1/Parkin is the most classic pathway mediating mitophagy. And the activation of PINK1/Parkin mediated mitophagy exerts neuroprotective effects during cerebral ischemia reperfusion injury (CIRI). Ligustilide (LIG) is a natural compound extracted from ligusticum chuanxiong hort and angelica sinensis (Oliv.) diels that exerts neuroprotective activity after cerebral ischemia reperfusion injury (CIRI). However, it still remains unclear whether LIG could attenuates cerebral ischemia reperfusion injury (CIRI) through regulating mitophagy mediated by PINK1/Parkin. PURPOSE: To explore the underlying mechanism of LIG on PINK1/Parkin mediated mitophagy in the hippocampus induced by ischemia reperfusion. METHODS: This research used the middle cerebral artery occlusion and reperfusion (MCAO/R) animal model and oxygen-glucose deprivation and reperfusion (OGD/R) as in vitro model. Neurological behavior score, 2, 3, 5-triphenyl tetrazolium chloride (TTC) staining and Hematoxylin and Eosin (HE) Staining were used to detect the neuroprotection of LIG in MCAO/R rats. Also, the levels of ROS, mitochondrial membrane potential (MMP) and activities of Na+-K+-ATPase were detected to reflect mitochondrial function. Moreover, transmission electron microscope (TEM) and fluorescence microscope were used to observe mitophagy and the western blot was performed to explore the changes in protein expression in PINK1/Parkin mediated mitophagy. Finally, exact mechanism between neuroprotection of LIG and mitophagy mediated by PINK1/Parkin was explored by cell transfection. RESULTS: The results show that LIG improved mitochondrial functions by mitophagy enhancement in vivo and vitro to alleviate CIRI. Whereas, mitophagy enhanced by LIG under CIRI is abolished by PINK1 deficiency and midivi-1, a mitochondrial division inhibitor which has been reported to have the function of mitophagy, which could further aggravate the ischemia-induced brain damage, mitochondrial dysfunction and neuronal injury. CONCLUSION: LIG could ameliorate the neuronal injury against ischemia stroke by promoting mitophagy via PINK1/Parkin. Targeting PINK1/Parkin mediated mitophagy with LIG treatment might be a promising therapeutic strategy for ischemia stroke.

Prognostic analysis of crescentic glomerulonephritis with acute kidney injury: a single-center cohort with 5-year follow-up.

BACKGROUND: Crescentic glomerulonephritis (CrGN) represents a severe form of glomerular injury that results in high rates of renal failure. This study aimed to investigate the influence of potential clinical and pathological factors on renal outcomes of CrGN; and the relationship between 3-month and 5-year follow-up as well. METHODS: The cohort enrolled patients diagnosed of biopsy proven CrGN with acute kidney injury(AKI) from January 1, 2010, to January 1, 2018 in Shanghai Changzheng Hospital and followed up for 5 years. RESULTS: A total of 56 patients were included, among whom 11 patients (19.6%) had type I, 12 (21.4%) had type II, and 33 (58.9%) had type III CrGN. Patients with type II CrGN tended to have a lower crescent score, less renal tubular damage, and lower serum creatinine than the other two types. Three-month cumulative renal survival rates of types I, II, and III CrGN were 45.5%, 66.7%, and 48.5%, respectively. Five-year cumulative renal survival rates of types I, II, and III CrGN were 27.2%, 83.3%, and 36.4%, respectively. The Kappa Consistency Test between 3-month and 5-year outcomes was 0.573(P < 0.001). Cox regression model showed that in-hospital dialysis was an indicative renal survival factor in 3 months (HR 15.670, 95% CI 2.987-82.210, P = 0.001). It also showed that the crescent score had an unfavorable effect for renal survival in 5 years (HR 1.750 95% CI 1.018-3.009, P = 0.043). CONCLUSIONS: Clinical manifestations and outcomes varied by different CrGN types. Three-month outcomes could partially reflect the 5-year outcomes. Severity of crescents was an independent risk factor for renal survival in CrGN.