Abstract
BackgroundWe 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.MethodsOur 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.ResultsThe 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.ConclusionThis 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.
Highlights
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
IgA nephropathies, defined as at least 1+ mesangial IgA deposits by immunofluorescence, are clinically divided in two groups:-primary IgAN, called Berger’s disease which represent 90% of the cases, and-secondary IgAN observed in different clinical conditions: Henoch-Schönlein Purpura, Henoch-Schönlein purpura (HSP), alcoholic liver cirrhosis, some cases of Systemic Lupus Erythematosus, Systemic lupus erythematosus (SLE), and few other rare conditions. The goal of this retrospective observational study was to review all our cases of secondary IgAN and to apply our ARR score for an additional validation in another group of patients, and we focused on IGAN 2ary to HSP, which represented the majority of the cases
Baseline data at diagnosis All 74 patients presented with cutaneous purpura at least on one occasion with the classical characteristics; the initial presentation was associated with arthralgia in 57 (77.0%) together with abdominal pain in 33 (44.6%) leading to the diagnosis of Henoch-Schönlein purpura
Summary
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. In primary IgAN, many risk factors predictive of progression have been described [1,2] and in a previous work [3], we focused on the following three consensual and major risk factors: occurrence of arterial hypertension (HT), amount of daily proteinuria, and severe renal lesions on optical microscopy (appreciated by local pathological scoring [4,5] or by the new Oxford classification [6]) These risk factors were simplified and dichotomized, before integration in an Absolute Renal Risk (ARR) score, which proved to be an overall accurate predictor of ultimate prognosis. These findings were validated in a retrospective historical cohort including 250 patients [3]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
