Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients

Khalighi, Mazdak A.; Wang, Shih Tien; Henriksen, Kammi J.; Bock, Margret; Keswani, Mahima; Chang, Anthony; Meehan, Shane M.

doi:10.1007/s00467-014-2970-9

Cited by 13 publications

(10 citation statements)

References 35 publications

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“…These results can predict the progression of renal function to ESRD in children with AAV. However, the results of this study are not entirely consistent with previous studies (33)(34)(35)(36)(37), primarily because of the small sample size, which did not meet the requirements of events per variable (EPV). Although the results might not be stable enough, they were reported considering the rare incidence and absent clinical evidences in children.…”

Section: Discussioncontrasting

confidence: 99%

“…Previous studies have reported that baseline eGFR < 60 ml/min/1.73 m 2 , hypertension, baseline Scr, oliguria/anuria, histopathological classification, and renal tubular atrophy are risk factors for poor prognosis in AAV (33)(34)(35)(36)(37)(38)(39)(40)(41). In this study, the univariate analysis showed that baseline eGFR < 60 ml/min/1.73 m 2 , hypertension, glomerular sclerosis >50% of total glomerular count, mesangial cell, and matrix hyperplasia were risk factors for progression to ESRD in AAV children (P < 0.05).…”

Section: Discussionmentioning

confidence: 99%

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Clinicopathological Analysis of 34 Cases of Primary Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Chinese Children

Pei

Rong

et al. 2021

Front. Pediatr.

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Background: This study aimed to summarize the clinicopathological features and prognostic risk factors of primary antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in children.Methods: Clinical and prognostic data for children admitted to our center with AAV between September 2003 and September 2020 were studied retrospectively. The incidence and risk factors of end-stage renal disease (ESRD) were calculated and analyzed.Results: Thirty-four children were enrolled; 28 were female, with a median onset age of 10 years. Except for one case negative for ANCA, the other 33 patients were diagnosed with microscopic polyangiitis (MPA). The most frequently involved organ was the kidney (100.0%), followed by the lungs (58.8%) and heart (50.0%). Twenty children (58.8%) progressed to ESRD with a median course of 3 months, and they were more likely to present respiratory and cardiovascular system involvement than were the non-ESRD group (P < 0.05). Patients in the ESRD group also had a higher serum creatinine level, 24-h protein excretion, Pediatric Vasculitis Activity Score (PVAS), and a lower level of estimated glomerular filtration rate (eGFR), hemoglobin, and complement C3 than had those in the non-ESRD group (P < 0.05). The main pathological manifestations were crescentic and sclerotic classes in the ESRD group and focal class in the non-ESRD group. After 6 months of induction therapy, 90.0% of cases achieved complete or partial remission. The multivariate logistic regression model showed that baseline eGFR < 60 ml/min/1.73 m2 was an independent risk factor for progressing to ESRD (OR = 0.016, 95% CI = 0.001~0.412, P = 0.012).Conclusions: AAV in children usually occurs in teenage girls, and the most commonly involved organ is the kidney, of which hematuria is the most common symptom, followed by proteinuria, abnormal renal function (eGFR < 90 ml/min/1.73 m2), etc. The primary type of AAV is MPA. Nearly 60% of patients progressed to ESRD with a median course of 3 months. Baseline eGFR < 60 ml/min/1.73 m2 is an independent risk factor for ESRD progression in AAV children.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Clinicopathological Analysis of 34 Cases of Primary Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Chinese Children

Pei

Rong

et al. 2021

Front. Pediatr.

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“…Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan. Table 4 shows a summary of previous literature in which pediatric rapidly progressive or crescentic glomerulonephritis has been included [7][8][9][10][11][12][13][14][15][16][17][18]. The patient survival rate was extremely high in these reported cases, consistent with the present study.…”

Section: Plos Onesupporting

confidence: 84%

Age-dependent survival in rapidly progressive glomerulonephritis: A nationwide questionnaire survey from children to the elderly

et al. 2020

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Background Rapidly progressive glomerulonephritis (RPGN) has been known to have a poor prognosis. Although evidence across adult RPGN cases has accumulated over many years, the number of case series in adolescents and young adults has been limited, requiring further studies. Methods A total of 1,766 cases from 1989 to 2007 were included in this nationwide questionnaire survey, led by Intractable (former name, Progressive) Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan. To elucidate age-related differences in 2-year patient and renal survival rates, the cases were divided into the following four groups: children (0–18 years), young adults (19–39 years), the middle-aged (40–64 years), and the elderly (over 65 years). Results Of the 1,766 total RPGN cases, antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis comprised 1,128 cases (63.9% of all RPGN cases), showing a tendency to increase with age. Two-year patient survival for RPGN was 93.9% among children, 92.6% in young adults, 83.2% in the middle-aged, and 68.8% in the elderly. The younger group (children plus young adults) showed a clearly higher survival rate compared to the older group (middle-aged plus elderly) (p<0.05). ANCA-associated glomerulonephritis also showed similar age-related results with all RPGN cases. The comparison of renal prognosis showed no statistically significant differences both in RPGN and in ANCA-associated GN. Conclusion The present study described the age-dependent characteristics of the classification of RPGN, especially focusing on a better prognosis of the younger group in patient survival both in RPGN and in ANCA-associated GN.

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“…Yin et al6 reported only 10 pediatric cases out of 1579 renal biopsy series of 23 years. In different pediatric studies, mean age at presentation has been reported to range from 11 to 12.27 years while the youngest patient was 3 years old 1 , 3 , 4. Overall prognosis of the condition is poor.…”

Section: Discussionmentioning

confidence: 99%

“…Pauci-immune glomerulonephritis (GN) is more common in adults than in children and it is associated with ANCA positivity in 80% of the patients. ANCA positivity also commonly accompanies small vessel vasculitis such as granulomatosis with poliangiitis, microscopic poliarteritis nodosa (PAN), and Churg-Strauss syndrome 1. Pauci-immune GN is one of the usual patterns of renal involvement in these vasculitic syndromes.…”

Section: Introductionmentioning

confidence: 99%

Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

et al. 2019

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Pauci-immune glomerulonephritis (GN) is more common in elderly people compared to children and the etiology is not completely understood yet. Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in 80% of the patients. We report a case of a 7-year-old girl who presented with malaise and mildly elevated creatinine diagnosed as ANCA-associated pauci-immune crescentic glomerulonephritis with crescents in 20 of 25 glomeruli (80%). Of these 20 crescents, 12 were cellular, 4 fibrocellular, and 4 globally sclerotic. She did not have purpura, arthritis, or systemic symptoms and she responded well to initial immunosuppressive treatment despite relatively severe histopathology. The patient was given three pulses of intravenous methylprednisolone (30 mg/kg on alternate days) initially and continued with cyclophosphamide (CYC; 2 mg/kg per day) orally for 3 months with prednisone (1 mg/kg per day). In one month, remission was achieved with normal serum creatinine and prednisone was gradually tapered. The case of this child with a relatively rare pediatric disease emphasizes the importance of early and aggressive immunosuppressive treatment in patients with renal-limited ANCA-associated pauci-immune crescentic GN even if with a mild clinical presentation. As in our patient, clinical and laboratory findings might not always exactly reflect the severity of renal histopathology and thus kidney biopsy is mandatory in such children to guide the clinical management and predict prognosis.

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Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients

Cited by 13 publications

References 35 publications

Clinicopathological Analysis of 34 Cases of Primary Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Chinese Children

Clinicopathological Analysis of 34 Cases of Primary Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Chinese Children

Age-dependent survival in rapidly progressive glomerulonephritis: A nationwide questionnaire survey from children to the elderly

Anca-associated crescentic glomerulonephritis in a child with isolated renal involvement

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