#2777 RISK FACTORS ASSOCIATED WITH OUTCOMES IN PATIENTS WITH HENOCH - SCHӧNLEIN PURPURA NEPHRITIS

Abstract

Abstract Background and Aims Henoch - Schӧnlein purpura (HSP) is an immuno-mediated small-vessel vasculitis that manifests as non—thrombocytopenic purpura, arthritis or arthralgia, abdominal pain and/or kidney involvement. HSP nephritis can presented is micro- or macroscopic hematuria, proteinuria, nephrotic or nephritic syndrome, as well as acute renal failure. The aim of the study is to identify risk factors associated with unfavorable outcomes in patients with HSP nephritis. Method This retrospective study enrolled 68 patients with HSP nephritis. Renal and extra-renal symptoms were analyzed. A diagnosis of HSP nephritis was made when hematuria and/or proteinuria, and/or renal failure was associated with a palpable purpuric eruption or abdominal or joint pains (at least two of these three clinical signs) and predominant mesangial IgA immune deposits, confirmed by renal biopsy. The patients were subdivided into five classes according to the renal manifestation at onset of disease: 1) micro- or macroscopic hematuria; 2) mild proteinuria (<1 g/L) ± hematuria; 3) acute nephritic syndrome, defined as moderate proteinuria, hematuria, increased serum creatinine and/or hypertension; 4) nephrotic syndrome; 5) mixed nephritic-nephrotic syndrome. Results Of 68 patients with HSP nephritis, diagnosed by kidney biopsy, 41 (60,29%) were male and 27 (39,71%) - female. Age of onset was between 18 and 66 years (mean 37,28 ± 9,34). Duration of follow-up was between 2 and 28 years. 29 patients had histories of infection preceding presentation. At onset all patients had palpable purpura and urinary abnormalities (only hematuria - in 16,18%; mild proteinuria ± hematuria – in 44,12%; moderate or severe proteinuria and hematuria – in 39,70%). Arthralgias were present in 49 patients (72,06%), gastrointestinal involvement – in 32 patients (47,05%). Renal function was impaired in 26,47% of patients, and 51,47% were hypertensive. Mesangial hypercellularity lesions were found in most patients (97,06%), endocapillary proliferation – in 20,58%, segmental sclerosis – in 32,35%, tubular atrophy/interstitial fibrosis – in 38,23%. Corticosteroids and cyclophosphamide were prescribed in patients who presented with severe clinical and histological features and/or rapidly progressing renal disease. During follow-up classical extra-renal organ diseases were seen in 55,88% of patients, and hematuria and/or proteinuria – in 77,94%. At final review 26,47% had progression of renal failure. Risk factors for renal failure were moderate or severe proteinuria during follow-up (p<0,001), renal impairment at presentation (p<0,001), hypertension at presentation and during follow up (p<0,05), crescents, interstitial fibrosis and tubular atrophy (p<0,001). No significant difference in renal outcome was observed between patients who had relapses in extra-renal organs versus in those who did not. Conclusion Our results indicated that lower GFR, nephrotic syndrome, nephritic-nephrotic syndrome and crescentic nephritis were risk factors for unfavorable outcomes.