Crescentic glomerulonephritis in children: a single centre experience (original) (raw)
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Journal of Pediatric Nephrology, 2023
Background and Aim: This study aims to evaluate the clinical and histopathological profile in children with crescentic glomerulonephritis (CGN) and determine the predictors of renal outcome. Methods: In this retrospective study, we reviewed all native kidney biopsies performed in patients <18 years over 9 years (2011-2019). Individuals with ≥20% crescents with follow-up for at least 1 year were enrolled. Results: This study included 34 patients. The most common variety was immune-complex glomerulonephritis (GN) (type II CGN) (n=21; 62%), including patients with Henoch-Schonlein purpura (n=6), lupus nephritis (n=6), post-infectious GN (n=3), C3GN (n=3), and dense deposit disease (n=3). The second most common was pauci-immune GN (type III CGN; n=12; 35%) followed by anti-glomerular basement membrane disease (type I CGN; n=1; 3%). Hypertension (88%), hematuria (84.2%), and oliguria (64%) were the most common presenting features. The outcome predictors for poor renal survival were the presence of oliguria (HR-5.11, P=0.035), severe hypertension (HR-11.51, P=0.019), estimated glomerular filtration rate <15 mL/min/1.73 m 2 at presentation (HR-5.05, P=0.007), percentage of crescents (HR-10.66, P=0.001), presence of fibrous crescents (HR-6.34, P=0.001), and interstitial fibrosis and tubular atrophy (HR-8.88, P=0.0046). The overall outcome of the study revealed complete recovery (n=12), partial recovery (n=6), chronic kidney disease (n=3), and end-stage renal disease (n=13). The renal survival in patients with ≥50% crescents was poor (P=0.037) as compared to subjects with <50% crescents. Conclusion: Renal survival can be predicted by the severity of presenting features and histopathological markers. Two-thirds of patients had type II CGN with renal survival outcomes similar to type III CGN. The percentage of crescents is the most important predictor of renal survival.
Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries
Pediatric Nephrology, 2008
Crescentic glomerulonephritis (CsGN) is an uncommon entity in children. This prospective study was conducted to evaluate the aetiology, clinical spectrum and outcome in children with crescentic glomerulonephritis. The single-centre prospective study comprised of 22 children with biopsy proven CsGN who had been referred to our institute over the period January 2000 to December 2005. These patients were subjected to detailed clinical and biochemical examinations. The diagnosis of underlying renal disease was based on various criteria, including the clinical picture, serology and histopathology. The patients received intravenous methyl prednisolone, oral steroid treatment, and oral cyclophosphamide with or without plasmapheresis. All patients received supportive care, including control of hypertension and oedema and supportive management of renal insufficiency. During this 5-year period, CsGN accounted for 5.1% of all biopsies done in children. The mean age was 12.27 years (range 4 years to 18 years). There were eight girls and 14 boys. The mean duration of symptoms prior to referral was 2.47 months (range 5 days to 21 months). Aetiology was immune complex in 19 cases, anti-glomerular basement membrane (anti-GBM) antibody disease in two cases and pauciimmune (Wegener's granulomatosis) in one case. The percentage of crescents ranged from 50% to 100% (mean 70.6%). Twenty-one out of 22 (95.5%) children in our series had hypertension at presentation that required treatment with antihypertensive medications. The serum creatinine level at presentation ranged from 1.5 mg/dl to 11.4 mg/dl (mean 5.5 mg/dl). Of the 22 children, two were lost to follow-up, while the mean follow-up period of the rest of the 20 children was 8.13 months (range 1 month to 43 months). At the last follow-up of the 22 children, ten had stage 5 chronic kidney disease (CKD) and three had stage 4 CKD, while seven children had a calculated glomerular filtration rate (GFR) of >60 ml/min per 1.73 m 2 body surface area. Persistent proteinuria was seen on follow-up in the majority [13/20 (65%)] of patients. The outcome of CsGN in children continues to be poor, in our experience, due to delayed referral and delayed diagnosis. This was correlated histologically by the presence of fibrocellular crescents in the majority of our patient. Thus CsGN should be treated as a renal emergency. A greater awareness of this disease needs to be created amongst the referring paediatricians in developing countries to facilitate early diagnosis and prompt treatment.
Journal of Clinical Medicine, 2020
There is no evidence-based definition for diagnosing crescentic glomerulonephritis. The prognostic implications of crescentic lesions on kidney biopsy have not been quantified. Our objective was to determine risk factors for end-stage kidney disease (ESKD) in patients with glomerulonephritis and crescents on kidney biopsy. A query of the Pediatric Nephrology Research Consortium’s Pediatric Glomerulonephritis with Crescents registry identified 305 patients from 15 centers. A retrospective cohort study was performed with ESKD as the primary outcome. Median age at biopsy was 11 years (range 1–21). The percentage of crescents was 3–100% (median 20%). Etiologies included IgA nephropathy (23%), lupus (21%), IgA vasculitis (19%) and ANCA-associated GN (13%), post-infectious GN (5%), and anti-glomerular basement membrane disease (3%). The prevalence of ESKD was 12% at one year and 16% at last follow-up (median = 3 years, range 1–11). Median time to ESKD was 100 days. Risk factors for ESKD i...
Clinicopathological Spectrum and Outcome of Crescentic Glomerulonephritis
Journal of Renal and Hepatic Disorders, 2022
Crescentic glomerulonephritis (CrGN) is characterized by the presence of crescents in more than 50% of glomeruli. This study aims to identify the etiology and clinicopathological features and outcomes of CrGN. In this observational study, 80 biopsy-proven CrGN were included. Patients’ demographic profile, clinical parameters, treatments, and outcomes were collected and analyzed. The mean age in our study population was 40.86 ± 16.5 years. Type II CrGN was the most common type of CrGN. Female predominance was observed in type I and type II CrGN. The highest percentage of glomeruli with crescents was seen in type I (87 ± 15.2%, P = 0.04), followed by type III and type II. At the last follow-up, mean estimated glomerular filtration rate was 25.8 ± 11.41 mL/min/1.73 m2 and was significantly lower in type I CrGN (11.6 ± 4.8 mL/min/1.73 m2 P = 0.001). The overall 5-year renal survival rate was 55% and was highest in type II (69.4%), followed by type III and type I (27.3%) CrGN (P = 0.0299...
Nephro-Urology Monthly, 2017
Background: Crescentic extra-capillary glomerulonephritis (ECGN) is a diagnostic and therapeutic emergency. In black Africans, little data are available on this lesion. This study aimed at determining the clinical/biological and evolutionary patterns of ECGN and identifying factors of poor prognosis. Methods: This was a retrospective study conducted over a period of 5 years. All patients with extra-capillary proliferation of ≥ 50% in Bowman's space upon kidney biopsy were involved. The researchers compared 2 groups of patients: group A (patients, who fully recovered from renal function after 3 months of treatment) and group B (patients developing signs of chronic renal insufficiency) in order to identify factors responsible for a poor renal prognosis. Results: Forty cases of crescentic glomerulonephritis were found in 750 kidney biopsies, i.e. a prevalence of 5.33%. The mean age of the patients was 33.9 ± 16.65 years, with a gender ratio of 0.53. Upon admission, 85% of cases had renal insufficiency, with oligoanuria present in 62.5%. By employing light microscopy, the crescents were found to be cellular in 60%, fibro-cellular in 32.5%, and fibrous in 7.5%. The etiologies were mainly lupus in 32.5% of cases, followed by ANCA-related vasculitis in 27.5% of cases, and infectious causes in 17.5% of cases. Outside of the infectious context, basic treatment relied on immune suppression combining corticosteroids and cytotoxic drugs. The evolution was marked by transition to a chronic state in 72.7% of cases; 18.2% of patients recovered normal renal function. The factors responsible for a poor renal prognosis were oligoanuria (P = 0.0008), high creatinine level upon admission (P = 0.0001), use of hemodialysis (P = 0.0001), and evidence of fibro-cellular and fibrous crescents (P = 0.013). Conclusions: Crescentic glomerulonephritis was relatively common in the current patients. The etiology was dominated by lupus in the study's context. This rapidly causes chronic renal failure, hence explaining the interest in early management.
Prognostic factors in crescentic glomerulonephritis: a single-center experience
Iranian journal of kidney diseases, 2015
Crescentic glomerulonephritis (CGN) is a fatal disease, rapidly leading to end-stage renal disease. Diagnosis should be accurate and treatment should be started immediately. We investigated the factors associated with the renal prognosis in CGN patients. Forty-one patients with CGN who were followed up at the Nephrology Clinic of Ankara Numune Education and Research Hospital were divided into 2 arms of the dialysis-dependent group after treatment and the group that was followed up without dialysis. Demographic and clinical features along with biopsy findings during time of diagnosis were evaluated for both groups. The mean age was 41.3 ± 17.2 years old and 26 were men. Twenty patients developed end-stage renal disease, requiring long-term dialysis. The dialysis-dependent group had higher serum creatinine levels (8.2 ± 3.6 mg/dL versus 2.6 ± 2.5 mg/dL) and percentages of glomeruli with crescent (83.1 ± 19.1% versus 56.4 ± 11.9%), were more likely to have oligoruia-anuria (90.5% versu...
Rapidly progressive glomerulonephritis in children
Pakistan Journal of Medical Sciences, 2021
Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders. Clinical manifestations include gross hematuria, proteinuria, oliguria, hypertension and edema. Diagnostic evaluation, including renal function tests, electrolytes, urinalysis/microscopy and serology including (anti GBM antibody, antineutrophil cytoplasmic antibody (ANCA)) starts simultaneously with management. An urgent renal biopsy is required to allow specific pathologic diagnosis as well as to a...
Etiology and Frequency Distribution of Crescentic Glomerulonephritis in Renal Biopsy
Background: Crescentic glomerulonephritis results from proliferating cells in Bowman's capsule and formation of crescents. It is classified in three major groups: Anti-Glomerular basement membrane (anti GBM) antibody mediated, Immune-complex mediated, and antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. Methods: This is a cross-sectional study of 87 biopsy-proven cases of crescentic glomerulonephritis during 2011-2015 in a single center. They were classified into six groups: Lupus nephritis, Immunoglobulin A (IgA) nephropathy, ANCA-associated glomerulonephritis (containing of cANCA and pANCA vasculitis), Goodpasture syndrome, Cryoglobulinemia, and Poststreptococcal glomerulonephritis (PSGN). Findings: Of 87 renal biopsy, 62% were males. The mean age in all patients groups was 36 ± 17 years. Frequency distribution of the groups in our study were as follow: IgA nephropathy (41.3%), Lupus nephritis (36.7%), ANCA-associated glomerulonephritis (16%), Goodpasture syndrome (2.2%), Cryoglobulinemia (2.2%), and PSGN (1.1%). Mean and median of serum creatinine and proteinuria were 2 ± 1 mg/dl (Median = 1mg/dl) and 1930 ± 830 mg/day (Median = 2000mg/day). In this study, we found a significant positive correlation between the number of crescents and age (P < 0.001), additionally we found a positive association between the proportion of crescents and serum creatinine (P = 0.007), however, there were no significant correlation between the proportion of crescents and proteinuria (P > 0.050). Conclusion: This study reveals the importance of relationship between the proportion of crescents and serum creatinine .Therefore the proportion of crescents may influence the prognosis of the disease
International Urology and Nephrology, 2003
This study included 128 patients withcrescentic glomerulonephritis (CGN) havingsufficient clinical and histopathological dataand were followed up in our institute for amean period of 34 ± 28 months. There were 49males and 79 females with mean age 22.7 ± 14years. We studied the effect of clinical,laboratory and histopathological parameters onkidney function and patient survival at the endpoint of the study. The multivariate analysisrevealed that serum creatinine at presentation,nephrotic range proteinuria during the followup period, percentage of glomeruli affected bycrescents, percentage of fibrous crescents andabsence of cellular infiltration weresignificant risk factors affecting the kidneyfunction at termination of the study. The onlyrisk factor which correlated significantly withthe patient mortality was the serum creatinineat last follows up.