title: Validation of the absolute renal risk of dialysis/death in adults with IgA nephropathy secondary to Henoch-Schönlein purpura: a monocentric cohort study.
abstract: BACKGROUND
  We established earlier the absolute renal risk (ARR) of dialysis/death (D/D) in primary IgA nephropathy (IgAN) which permitted accurate prospective prediction of final prognosis. This ARR was based on the potential presence at initial diagnosis of three major, independent, and equipotent risk factors such as hypertension, quantitative proteinuria≥1 g per day, and severe pathological lesions appreciated by our local classification scoring≥8 (range 0-20). We studied the validity of this ARR concept in secondary IgAN to predict future outcome and focused on Henoch-Schönlein purpura (HSP) nephritis.
  
  
  METHODS
  Our cohort of adults IgAN concerned 1064 patients with 101 secondary IgAN and was focused on 74 HSP (59 men) with a mean age of 38.6 at initial diagnosis and a mean follow-up of 11.8 years. Three major risk factors: hypertension, proteinuria≥1 g/d, and severe pathological lesions appreciated by our global optical score≥8 (GOS integrated all elementary histological lesions), were studied at biopsy-proven diagnosis and their presence defined the ARR scoring: 0 for none present, 3 for all present, 1 or 2 for the presence of any 1 or 2 risk factors. The primary end-point was composite with occurrence of dialysis or death before (D/D). We used classical statistics and both time-dependent Cox regression and Kaplan-Meier survival curve methods.
  
  
  RESULTS
  The cumulative rate of D/D at 10 and 20 years post-onset was respectively 0 and 14% for ARR=0 (23 patients); 10 and 23% for ARR=1 (N=19); 27 and 33% for ARR=2 (N=24); and 81 and 100% (before 20 y) in the 8 patients with ARR=3 (P=0.0007). Prediction at time of diagnosis (time zero) of 10y cumulative rate of D/D event was 0% for ARR=0, 10% for ARR=1, 33% for ARR=2, and 100% by 8.5y for ARR=3 (P=0.0003) in this adequately treated cohort.
  
  
  CONCLUSIONS
  This study clearly validates the Absolute Renal Risk of Dialysis/Death concept in a new cohort of HSP-IgAN with utility to individual management and in future clinical trials.
authors.affiliation: University Hospital of Saint-Etienne; Nephrology, Dialysis and Renal Transplantation Department, North Hospital, Saint-Etienne, France.
authors.forename: Hesham
authors.initials: H
authors.lastname: Mohey
authors.forename: Blandine
authors.initials: B
authors.lastname: Laurent
authors.forename: Christophe
authors.initials: C
authors.lastname: Mariat
authors.forename: Francois
authors.initials: F
authors.lastname: Berthoux
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D005922:Glomerulonephritis, IGA / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications* / Q000175:diagnosis / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis / Q000401:mortality*; D012189:Retrospective Studies; D012307:Risk Factors
references.citation: Kidney Int. 2011 Jul;80(1):79-87
references.pmid: 21326171
references.citation: J Am Soc Nephrol. 2011 Apr;22(4):752-61
references.pmid: 21258035
references.citation: Q J Med. 1978 Oct;47(188):495-516
references.pmid: 375278
references.citation: Clin Exp Rheumatol. 2006 Mar-Apr;24(2 Suppl 41):S26-30
references.pmid: 16859592
references.citation: J Am Soc Nephrol. 2005 Jul;16(7):2088-97
references.pmid: 15930092
references.citation: Pediatr Neonatol. 2009 Aug;50(4):162-8
references.pmid: 19750891
references.citation: Arch Dermatol. 1997 Apr;133(4):438-42
references.pmid: 9126006
references.citation: Pediatr Nephrol. 2007 Dec;22(12):2067-72
references.pmid: 17943324
references.citation: N Engl J Med. 2002 Sep 5;347(10):738-48
references.pmid: 12213946
references.citation: QJM. 2006 Apr;99(4):253-65
references.pmid: 16565522
references.citation: Mod Pathol. 2001 Jul;14(7):635-40
references.pmid: 11454994
references.citation: Nephrol Dial Transplant. 1997 Nov;12(11):2277-83
references.pmid: 9394311
references.citation: J Am Soc Nephrol. 2002 May;13(5):1271-8
references.pmid: 11961015
references.citation: Am J Kidney Dis. 2006 Dec;48(6):1009-16
references.pmid: 17162160
references.citation: Clin J Am Soc Nephrol. 2011 Dec;6(12):2806-13
references.pmid: 22157710
references.citation: Pediatr Nephrol. 2005 Sep;20(9):1269-72
references.pmid: 15947991
references.citation: Clin Nephrol. 1989 Feb;31(2):60-6
references.pmid: 2646052
references.citation: Clin J Am Soc Nephrol. 2011 Oct;6(10):2384-8
references.pmid: 21885791
references.citation: Kidney Int. 2012 Dec;82(12):1304-12
references.pmid: 22895518
references.citation: Am J Kidney Dis. 2006 Jun;47(6):993-1003
references.pmid: 16731294
references.citation: Pediatr Nephrol. 2010 Jan;25(1):19-26
references.pmid: 19526254
references.citation: Clin Nephrol. 1990 Aug;34(2):45-51
references.pmid: 2225552
references.citation: Q J Med. 1985 May;55(217):145-52
references.pmid: 3889976
references.citation: Am J Kidney Dis. 1991 Jul;18(1):12-9
references.pmid: 2063844
references.citation: Clin Nephrol. 1986 Sep;26(3):125-30
references.pmid: 3769227
references.citation: Kidney Int. 2009 Sep;76(5):534-45
references.pmid: 19571791
references.citation: Clin J Am Soc Nephrol. 2011 Mar;6(3):679-89
references.pmid: 21393485
references.citation: Clin Nephrol. 2002 Jul;58(1):1-8
references.pmid: 12141401