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

Mazdak Khalighi, Shihtien Wang, Kammi J. Henriksen, Margret Bock, Mahima Keswani, Anthony Chang, Shane M. Meehan

Research output: Contribution to journalArticle

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Abstract

Background: Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Methods: Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 % among pediatric patients). Results: There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 % of patients, and 80 % had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m2. Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 % had developed chronic kidney disease at follow-up (eGFR <90 ml/min/1.73 m2), of whom 85 % of those had crescentic, mixed or sclerotic GN. Conclusion: Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function.

Original languageEnglish (US)
Pages (from-to)953-959
Number of pages7
JournalPediatric Nephrology
Volume30
Issue number6
DOIs
StatePublished - Jun 1 2015
Externally publishedYes

Fingerprint

Glomerulonephritis
Pediatrics
Antineutrophil Cytoplasmic Antibodies
Glomerular Filtration Rate
Biopsy
Systemic Vasculitis
Polyarteritis Nodosa
Wounds and Injuries
Chronic Renal Insufficiency
Cyclophosphamide
Adrenal Cortex Hormones
Pathology
Kidney
Incidence

Keywords

  • Acute kidney injury
  • Anti-neutrophil cytoplasmic antibody
  • Crescentic glomerulonephritis
  • Pauci-immune glomerulonephritis
  • Small vessel vasculitis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

Cite this

Khalighi, M., Wang, S., Henriksen, K. J., Bock, M., Keswani, M., Chang, A., & Meehan, S. M. (2015). Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients. Pediatric Nephrology, 30(6), 953-959. https://doi.org/10.1007/s00467-014-2970-9

Pauci-immune glomerulonephritis in children : A clinicopathologic study of 21 patients. / Khalighi, Mazdak; Wang, Shihtien; Henriksen, Kammi J.; Bock, Margret; Keswani, Mahima; Chang, Anthony; Meehan, Shane M.

In: Pediatric Nephrology, Vol. 30, No. 6, 01.06.2015, p. 953-959.

Research output: Contribution to journalArticle

Khalighi, M, Wang, S, Henriksen, KJ, Bock, M, Keswani, M, Chang, A & Meehan, SM 2015, 'Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients', Pediatric Nephrology, vol. 30, no. 6, pp. 953-959. https://doi.org/10.1007/s00467-014-2970-9
Khalighi, Mazdak ; Wang, Shihtien ; Henriksen, Kammi J. ; Bock, Margret ; Keswani, Mahima ; Chang, Anthony ; Meehan, Shane M. / Pauci-immune glomerulonephritis in children : A clinicopathologic study of 21 patients. In: Pediatric Nephrology. 2015 ; Vol. 30, No. 6. pp. 953-959.
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abstract = "Background: Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Methods: Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 {\%} among pediatric patients). Results: There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 {\%} of patients, and 80 {\%} had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m2. Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 {\%} had developed chronic kidney disease at follow-up (eGFR <90 ml/min/1.73 m2), of whom 85 {\%} of those had crescentic, mixed or sclerotic GN. Conclusion: Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function.",
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AU - Bock, Margret

AU - Keswani, Mahima

AU - Chang, Anthony

AU - Meehan, Shane M.

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N2 - Background: Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Methods: Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 % among pediatric patients). Results: There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 % of patients, and 80 % had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m2. Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 % had developed chronic kidney disease at follow-up (eGFR <90 ml/min/1.73 m2), of whom 85 % of those had crescentic, mixed or sclerotic GN. Conclusion: Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function.

AB - Background: Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Methods: Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 % among pediatric patients). Results: There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 % of patients, and 80 % had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m2. Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 % had developed chronic kidney disease at follow-up (eGFR <90 ml/min/1.73 m2), of whom 85 % of those had crescentic, mixed or sclerotic GN. Conclusion: Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function.

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KW - Anti-neutrophil cytoplasmic antibody

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KW - Pauci-immune glomerulonephritis

KW - Small vessel vasculitis

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