title: Pauci-immune glomerulonephritis in children: a clinicopathologic study of 21 patients.
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 m(2). 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 m(2)), of whom 85 % of those had crescentic, mixed or sclerotic GN.
  
  
  CONCLUSIONS
  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.
authors: Department of Pathology, University of Chicago Medical Center, 5841 S. Maryland Ave, MC 6101, Chicago, IL, 60637, USA, Mazdak.khalighi@uchospitals.edu.
authors: Mazdak A
authors: MA
authors: Khalighi
authors: Shihtien
authors: S
authors: Wang
authors: Kammi J
authors: KJ
authors: Henriksen
authors: Margret
authors: M
authors: Bock
authors: Mahima
authors: M
authors: Keswani
authors: Anthony
authors: A
authors: Chang
authors: Shane M
authors: SM
authors: Meehan
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002641:Chicago; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000187:drug effects / Q000276:immunology / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D011237:Predictive Value of Tests; D051436:Renal Insufficiency, Chronic / Q000276:immunology / Q000473:pathology / Q000517:prevention & control; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide