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

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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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Authors and Affiliations

  1. Department of Pathology, University of Chicago Medical Center, 5841 S. Maryland Ave, MC 6101, Chicago, IL, 60637, USA

    Mazdak A. Khalighi, Kammi J. Henriksen, Anthony Chang & Shane M. Meehan

  2. Division of Pediatric Kidney Diseases, Northwestern University Feinberg School of Medicine, Chicago, IL, 60611, USA

    Shihtien Wang, Margret Bock & Mahima Keswani

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  1. Mazdak A. Khalighi
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  2. Shihtien Wang
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Correspondence to Mazdak A. Khalighi.

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Khalighi, M.A., Wang, S., Henriksen, K.J. et al. Pauci-immune glomerulonephritis in children: A clinicopathologic study of 21 patients. Pediatr Nephrol 30, 953–959 (2015). https://doi.org/10.1007/s00467-014-2970-9

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  • DOI: https://doi.org/10.1007/s00467-014-2970-9

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