The limited role and risky profile of Rituximab in nephritis associated with Henoch-Schönlein purpura
Adult-onset IgA vasculitis or Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA1-dominant deposits. The symptoms include cutaneous purpura, ankle arthritis, enteritis and nephritis. HSP nephritis (HSPN) can be severe and refractory to corticosteroids with/without immunosuppressive agents. The concept of depletion of antibody producing B cell with Rituximab is appealing despite the uncertainity of HSP pathogenesis. In the present case report; we describe our experience with Rituximab treatment in a patient with this disease. Our patient had different triggering factors for her relapses and lately Rituximab itself. Review of the literature indicates that autoantibodies to Gd-IgA1 did not decrease with Rituximab therapy and others indicated an inherited predisposition for higher levels in patients with progressive disease. Our findings confirm the limited role and risky profile of Rituximab in treatment of HSP.
Keywords: HSP, IgA, Rituximab, vasculitis.
2- Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for classification of childhood vasculitidies. Ann Rheum Dis 2006; 65: 936–941.
3- Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. "Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome". Arthritis and Rheum 1997; 40: 859–864
4- Hetland LE, Susrud KS, Lindahl KH, Baygum A. Henoch-Schonlen Purpura: A Literature Review. Acta Dermato-venereologica 2017; 97: 1160-1166.
5- Donnithorne KJ, Atkinson TP, Hinze CH, Nogueira JB, Saeed SA, et al. Rituximab therapy for severe refractory chronic Henoch-Schönlein purpura. J Pediatr 2009; 155: 136–139.
6- Maritati F, Fenoglio R, Pillebout E, Emmi G, Urban ML, et al. Brief report: Rituximab for the treatment of adult-onset IgA vasculitis (Henoch-Schonlen). Arthritis Rheumatol 2018; 70: 109-114.
7- Rai A, Nast C, Adler S. Henoch-Schonlen purpura nephritis. J Am Soc Nephrol 1999; 10: 2637-1644.
8- Sais G, Vidaller A, Jucgla A, Servitje O, Concom E, Peyri J. Prognostic factors in Leukocytoclastic vasculitis: a clinicopathologic study of 160 patients. Dermatol 1998; 134: 309-315.
9- Shresstha S, Sumingan N, Tan J, et al. Henoch Schonelen purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM 2006; 99: 253-265.
10- Jicheng Lv, Yihe Yang, Hong Zhang, Wenfang Chen, Xiaoxia Pan, et al. Prediction of Outcomes in Crescentic IgA Nephropathy in a Multicenter Cohort Study. JASN 2013; 24: 2118-2125.
11- Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, et al. Working Group of the International IgA Nephropathy Network and the Renal Pathology Society: The Oxford classification of IgA nephropathy: Rationale, clinicopathological correlations, and classification. Kidney Int 2009; 76: 534–545.
12- Zaza G, Bernich P, Lupo A. “Triveneto” Register of Renal Biopsies (TVRRB): Incidence of primary glomerulonephritis in a large North-Eastren Italian area: a 13-year renal biopsy study. Nephrol Dial Transplant 2013; 28: 367-372.
13- Barbour SJ, Reich HN. Risk stratification of patients with IgA nephropathy. Am J Kidney Dis 2012; 59: 865-873.
14- Conley ME, Cooper MD, Michael AF. Selective deposition of immunoglobulin A1 in immunoglobulin A nephropathy, anaphylactoid purpura nephritis, and systemic lupus erythematosus. J Clin Invest 1980; 66: 1432–1436.
15- Berthoux F, Suzuki H, Thibaudin L, Yanagawa H, Maillard N, et al. Autoantibodies targeting galactose-deficient IgA1 associate with progression of IgA nephropathy. J Am Soc Nephrol 2012; 23: 1579–1587.
16- Lomax-Browne HJ, Visconti A, Pusey CD, Cook HT, Spector TD, et al.
IgA1 glycosylation is heritable in healthy twins. JASN 2017; 28: 64-68.
17- Tanaka S, Ninomiya T, Katafuchi R, Masutani K, Nagata M, et al. The effect of renin-angiotensin system blockade on the incidence of end-stage renal disease in IgA nephropathy. Clin Exp Nephrol 2016; 20: 689-698.
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