Background Crescentic glomerulonephritis (CrGN) is a pathological description of rapidly progressive glomerulonephritis (RPGN). It is characterized by renal failure and is associated with a grave prognosis. This study aimed to investigate the clinical outcomes of patients diagnosed with crescentic glomerulonephritis at the King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. Method This retrospective study included patients with CrGN who underwent treatment at the nephrology department at KAUH from June 2021 to August 2022. We collected and analyzed data from 56 patients diagnosed with CrGN on the basis of renal biopsies between 2002 and 2015. Result The study included 17 cases of CrGN. The mean age of patients at the time of diagnosis was 18.06 ± 13.49 years. The distribution of histological findings showed that cellular crescents (94.1%) and interstitial fibrosis and tubular atrophy (IFTA) (76.5%) were the most commonly observed histological findings. The most common underlying etiology was lupus nephritis (41.2%). Regarding the lab results, the mean serum creatinine level at admission was 378.88 ± 273.27 μmol/L, proteinuria was 1.53 ± 1.23 and glomerular filtration rate (GFR) level was 36.94 ± 45.08 mL/min. The factors associated with poor renal outcome were IFTA (P=0.01), phosphate level before discharge, serum creatinine level before and after discharge (P=0.032), and GFR level after discharge (P=0.001). Conclusion Crescentic glomerulonephritis is an important cause of acute kidney injury due to its potential to result in severe glomerular injury. In our study, 12 out of 17 patients experienced poor renal outcomes, which were associated with a high risk of morbidity and mortality. Therefore, early detection and treatment of CrGN is crucial in order to manage the disease.

Effective prognostic markers are needed for antineutrophil cytoplasmic antibody-associated vasculitis (AAV). This study evaluated the clinical associations of serum vascular endothelial growth factor-A (sVEGF-A) and sVEGF-A165b (an antiangiogenic isoform of VEGF-A) concentrations with time to remission of AAV in a nationwide Japanese prospective follow-up cohort.
We collected samples from patients with AAV who were enrolled in the nationwide Japanese cohort study (RemIT-JAV-RPGN). We measured sVEGF-A and sVEGF-A165b concentrations using enzyme-linked immunosorbent assays in 57 serum samples collected 6 months before and after initiation of AAV treatment. Patients were classified based on AAV disease subtypes: microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA).
Results revealed significant reductions in sVEGF-A and sVEGF-A165b concentrations in patients with microscopic polyangiitis and EGPA, respectively. However, despite the comparable concentrations of sVEGF-A and sVEGF-A165b during the 6 months of treatment in granulomatosis with polyangiitis patients, correlation analysis revealed that the differences in log2-transformed concentrations of sVEGF-A and sVEGF-A165b were inversely correlated with time to remission in granulomatosis with polyangiitis patients.
These results suggest that sVEGF-A and -A165b can serve as potential markers of time to remission in patients with granulomatosis with polyangiitis.

Background: The spectrum and outcomes of crescentic glomerulonephritis (Cr.GN) in South Asia is vastly different from that reported worldwide and there is a paucity of information. The aim of the study was to study the demography, clinical presentation, histology and predictors of longitudinal outcomes of Cr.GN in this population. Methods: An observational cohort study of renal biopsies was performed in the largest tertiary center in South India over a period of 10 years (January 2006 to December 2015) with ≥50% crescents on renal histology indicating Cr.GN. Results: A total of 8645 kidney biopsies were done; 200 (2.31%) were Cr.GN. Patients were categorized into three etiological groups: anti-glomerular basement membrane (type I), immune complex (type II), and pauci-immune (type III). Type II was the most common (96, 46.5%), followed by type III (73, 38%) and type I (31, 15.5%). Female preponderance was seen across all types. About half of all patients presented with recent onset hypertension. Type II had the highest median proteinuria (4.2 (2.1-6) g/day, p=0.06) and the median estimated glomerular filtration rate was lowest in type I (5 (4-8) ml/min/1.73m 2, p<0.001). Among type III, anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis was seen only in ~50% of patients. Nearly one third of patients with type I were also positive for ANCA making them \'double positive\'. Acute glomerular insults like tuft necrosis and chronic changes as evidenced by moderate to severe interstitial fibrosis, was a predominant feature of type I. Conclusions: ANCA-negative pauci-immune vasculitis, as well as double positive Cr.GN, are reported for the first time in South-Asia. Renal survival was significantly worse in type I/III compared to type II. Types I/III, moderate to severe interstitial fibrosis and tubular atrophy, presence of oliguria/anuria and increasing percentage of crescents in renal biopsy were significant predictors of end stage kidney disease in our cohort.

Fibrillary glomerulonephritis (FGN) is a rare disorder accounting for up to 1% of all glomerulonephritis (GN). FGN usually manifests as nephrotic or subnephrotic proteinuria, hematuria, and hypertension in patients after the sixth decade. The overall prognosis of FGN is very poor. Crescentic presentation of FGN is uncommon which may be diagnosed as rapidly progressive glomerulonephritis (RPGN) unless electron microscopy and/or special stains are done. We report a case of a young female who presented as RPGN but diagnosis was revised to crescentic FGN after electron microscopy and immunohistochemical staining with DNAJB9 stain. Patient remained dialysis-dependent after treatment with steroid and cyclophosphamide for 2 months and progressed to end-stage renal disease (ESRD). Crescentic FGN usually does not respond to treatment and invariably progresses to ESRD over few months. This case emphasizes the defining role of electron microscopy and special stains in diagnosing uncommon glomerular diseases.

UNASSIGNED: Antiglomerular basement membrane disease manifests as rapidly progressive glomerulonephritis and alveolar hemorrhage. It encompasses 10-15% of crescentic glomerulonephritis and is associated with poor outcome. In this study, we have elaborated on the clinical details, morphological features, and outcome of anti-GBM glomerulonephritis.
UNASSIGNED: All the consecutive biopsy-proven cases of anti-GBM glomerulonephritis over a period of 4½ years were analyzed, retrospectively.
UNASSIGNED: Sixteen cases were diagnosed as anti-GBM glomerulonephritis during the study period. Twelve patients presented with rapidly progressive renal failure of which four patients required hemodialysis at the time of presentation. Goodpasture\'s syndrome was noted in two patients. Thirteen cases were positive for circulating anti-GBM antibodies and two patients showed double positivity for both anti-GBM antibodies and ANCA. Fifteen biopsies revealed crescentic glomerulonephritis with linear deposition of IgG along the glomerular basement membrane in all the 16 cases.
UNASSIGNED: Renal biopsy analysis is important in the diagnosis of Anti GBM nephritis. Morphology is an important predictor of disease progression.

BACKGROUND: The prognostic value of the EUVAS-proposed histopathological classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has been evaluated throughout the world. Here, we performed a Japanese nationwide biopsy survey to assess the association between this histopathological classification and renal prognosis after 2-year follow-up in ANCA-associated glomerulonephritis.
METHODS: We collected 67 renal biopsy materials of the 321 entries in the RemIT-JAV-RPGN cohort study, and assessed their histologies. Based on the EUVAS-proposed histopathological classification and some histological parameters, we statistically evaluated renal survival and the comparison of renal function for 2 years.
RESULTS: Based on the histopathological classification, the largest number of biopsy samples belonged to the Focal class, followed by the Mixed, Crescentic, and Sclerotic classes (n = 30, 19, 10, 8, respectively). Although the number of events might be too low (four patients with renal death) to make this conclusion, the Focal and Mixed classes had higher renal-survival rates compared to the others in the renal-survival curve. Comparing renal function among all classes, the estimated glomerular filtration rate (eGFR) throughout 2-year follow-up period was significantly higher in the Focal class compared to the other 3 classes. The eGFR-values in the Crescentic, Mixed, and Sclerotic classes increased with time. Based on both combined results, the Focal class could be the best prognosis.
CONCLUSIONS: This histopathological classification was valuable for both the stratification of renal function and the estimation of partial renal survival during 2-year follow-up in ANCA-associated glomerulonephritis.