Validation of the absolute renal risk of dialysis/death in adults with IgA nephropathy secondary to Henoch-Schönlein purpura: a monocentric cohort study

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. CONCLUSION: 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.