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

详细信息    查看全文

• 作者：Mazdak A. Khalighi ; Shihtien Wang ; Kammi J. Henriksen ; Margret Bock…
• 关键词：Pauci ; immune glomerulonephritis ; Crescentic glomerulonephritis ; Anti ; neutrophil cytoplasmic antibody ; Small vessel vasculitis ; Acute kidney injury
• 刊名：Pediatric Nephrology
• 出版年：2015
• 出版时间：June 2015
• 年：2015
• 卷：30
• 期：6
• 页码：953-959
• 全文大小：165 KB
• 参考文献：1.Jennette JC, Falk RJ, Gasim AH (2011) Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis. Curr Opin Nephrol Hypertens 20:263-70View Article PubMed Central PubMed
  2.Dillon MJ, Ozen S (2006) A new international classification of childhood vasculitis. Pediatr Nephrol 21:1219-222View Article PubMed
  3.Bomback AS, Appel GB, Radhakrishnan J, Shirazian S, Herlitz LC, Stokes B, D'Agati VD, Markowitz GS (2011) ANCA-associated glomerulonephritis in the very elderly. Kidney Int 79:757-64View Article PubMed
  4.Kambham N (2012) Crescentic Glomerulonephritis: an update on Pauci-immune and Anti-GBM diseases. Adv Anat Pathol 19:111-24View Article PubMed
  5.Zand L, Specks U, Sethi S, Fervenza FC (2014) Treatment of ANCA-associated vasculitis: new therapies and a look at old entities. Adv Chronic Kidney Dis 21:182-93View Article PubMed
  6.Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, Ekstrand A, Gaskin G, Gregorini G, de Groot K, Gross W, Hagen EC, Mirapeix E, Pettersson E, Siegert C, Sinico A, Tesar V, Westman K, Pusey C (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349:36-4View Article PubMed
  7.Pepper RJ, Chanouzas D, Tarzi R, Little MA, Casian A, Walsh M, Pusey CD, Harper L, Salama AD (2013) Intravenous cyclophosphamide and plasmapheresis in dialysis-dependent ANCA-associated vasculitis. Clin J Am Soc Nephrol 8:219-24View Article PubMed Central PubMed
  8.Schwartz GJ, Work DF (2009) Measurement and estimation of GFR in children and adolescents. Clin J Am Soc Nephrol 4:1832-843View Article PubMed
  9.Wong H, Mylrea K, Feber J, Drukker A, Filler G (2006) Prevalence of complications in children with chronic kidney disease according to KDOQI. Kidney Int 70:585-90PubMed
  10.Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noel LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM (2010) Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol 21:1628-636View Article PubMed
  11.Haas M, Jafri J, Bartosh SM, Karp SL, Adler SG, Meehan SM (2000) ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits. Am J Kidney Dis 36:709-18View Article PubMed
  12.Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Fessler BJ, Ding L, Viviano L, Tchao NK, Phippard DJ, Asare AL, Lim N, Ikle D, Jepson B, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh K, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Mueller M, Sejismundo LP, Mieras K, Stone JH (2013) Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 369:417-27View Article PubMed
  13.Yin XL, Zou MS, Zhang Y, Wang J, Liu TL, Tang JH, Qiu LR, Chen Y, Yuan HQ, Zhou JH (2013) Twenty-three-year review of disease patterns from renal biopsies: an experience from a pediatric renal center. J Nephrol 26:699-07View Article PubMed
  14.Hattori M, Kurayama H, Koitabashi Y (2001) Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 12:1493-500PubMed
  15.Siomou E, Tramma D, Bowen C, Milford DV (2012) ANCA-associated glomerulonephritis/systemic vasculitis in childhood: clinical features-outcome. Pediatr Nephrol 27:1911-920View Article PubMed
  16.Krmar RT, Kagebrand M, Hansson ME, Halling SE, Asling-Monemi K, Herthelius M, Holtback U, Christensson M, Wernerson A, Bruchfeld A (2013) Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis. Clin Nephrol 80:388-94View Article PubMed
  17.Hauer HA, Bajema IM, Van Houwelingen HC, Ferrario F, Noel LH, Waldherr R, Jayne DR, Rasmussen N, Bruijn JA, Hagen EC (2002) Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 62:1732-742View Article PubMed
  18.Ellis CL, Manno RL, Havill JP, Racusen LC, Geetha D (2013) Validation of the new classification of pauci-immune glomerulonephritis in a United States cohort and its correlation with renal outcome. BMC Nephrol 14:210View Article PubMed Central PubMed
  19.Ford SL, Polkinghorne KR, Longano A, Dowling J, Dayan S, Kerr PG, Holdsworth SR, Kitching AR, Summers SA (2014) Histopathologic and clinical predictors of kidney outcomes in ANCA-associated vasculitis. Am J Kidney Dis 63:227-35View Article PubMed
  20.Schlieben DJ, Korbet SM, Kimura RE, Schwartz MM, Lewis EJ (2005) Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis 45:758-61View Article PubMed
  21.Tanaka H, Waga S, Suzuki K, Nakahata T, Kawachi H, Shimizu F, Ito E (2002) Rapidly progressive, pauci-immune diffuse crescentic glomerulonephritis in an infant. Pediatr Nephrol 17:730-32View Article PubMed
  22.Naidu GS, Sharma A, Nada R, Kohli HS, Jha V, Gupta KL, Sakhuja V, Rathi M (2014) Histopathological classif
• 作者单位：Mazdak A. Khalighi (1)
  Shihtien Wang (2)
  Kammi J. Henriksen (1)
  Margret Bock (2)
  Mahima Keswani (2)
  Anthony Chang (1)
  Shane M. Meehan (1)
  
  1. Department of Pathology, University of Chicago Medical Center, 5841 S. Maryland Ave, MC 6101, Chicago, IL, 60637, USA
  2. Division of Pediatric Kidney Diseases, Northwestern University Feinberg School of Medicine, Chicago, IL, 60611, USA
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pediatrics
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1432-198X

文摘

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--), focal (n--), mixed (n--) and sclerotic GN (n--). Necrotizing arteritis was identified in a minority of patients (n--). 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.