Henoch-Sch？nlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Sch？nlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000？mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials. 1. Background Henoch-Sch？nlein purpura (HSP) is characterized by a leukocytoclastic vasculitis involving small vessels with deposition of immune IgA complexes [1]. HSP mainly affects children with an incidence around 15/100,000 per year [2] and is less frequent in adults [3]. Clinical symptoms include purpura, arthralgia, glomerulonephritis, and gastrointestinal and peripheral nerve involvement. Compared with children, clinical features and prognosis are different in adults: firstly the renal damage seems more frequent and serious, and then the frequency of associated cancers is higher [3, 4]. Cancers involved are bronchopulmonary, digestive, renal, and prostate. HSP prognosis is usually good, except in gastrointestinal and nephritis involvements. The treatment of HSP remains a matter of debate, both in adult and pediatric patients. Guidelines from pediatric experts do not support a systematic corticosteroid treatment [2, 5]. In adults the treatment usually consists in corticosteroids, eventually associated with an immunosuppressive treatment in cases of severe or relapsed forms [3, 4]. However, recent randomized studies failed to demonstrate a benefit to cyclophosphamide in addition to corticosteroids in severe glomerulonephritis forms in adults [6]. The B lymphocytes seem to be implicated in the pathogenesis of IgA nephropathy, a form of HSP limited to kidney [7, 8]. Consequently rituximab is a potential interesting targeted treatment and was already tested in three children [9] and two adult cases [10, 11] with severe HSP. We
References
[1]
R. J. Levinsky and T. M. Barratt, “IgA immune complexes in Henoch-Sch？nlein purpura,” The Lancet, vol. 2, no. 8152, pp. 1100–1103, 1979.
[2]
F. T. Saulsbury, “Clinical update: Henoch-Sch？nlein purpura,” The Lancet, vol. 369, no. 9566, pp. 976–978, 2007.
[3]
E. Pillebout, E. Thervet, G. Hill, C. Alberti, P. Vanhille, and D. Nochy, “Henoch-Sch？nlein Purpura in adults: outcome and prognostic factors,” Journal of the American Society of Nephrology, vol. 13, no. 5, pp. 1271–1278, 2002.
[4]
R. Blanco, V. M. Martínez-Taboada, V. Rodríguez-Valverde, M. García-Fuentes, and M. A. González-Gay, “Henoch-Sch？nlein purpura in adulthood and childhood: two different expressions of the same syndrome,” Arthritis & Rheumatology, vol. 40, pp. 859–864, 1997.
[5]
K. L. Gibson, M. A. Amamoo, and W. A. Primack, “Corticosteroid therapy for Henoch Sch？nlein purpura,” Pediatrics, vol. 121, no. 4, pp. 870–872, 2008.
[6]
E. Pillebout, C. Alberti, L. Guillevin, A. Ouslimani, and E. Thervet, “Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Sch？nlein Purpura,” Kidney International, vol. 78, no. 5, pp. 495–502, 2010.
[7]
A. C. Allen, P. S. Topham, S. J. Harper, and J. Feehally, “Leucocyte β1,3 galactosyltransferase activity in IgA nephropathy,” Nephrology Dialysis Transplantation, vol. 12, no. 4, pp. 701–706, 1997.
[8]
K. Yamada, N. Kobayashi, T. Ikeda et al., “Down-regulation of core 1 β1,3-galactosyltransferase and Cosmc by Th2 cytokine alters O-glycosylation of IgA1,” Nephrology Dialysis Transplantation, vol. 25, no. 12, pp. 3890–3897, 2010.
[9]
K. J. Donnithorne, T. P. Atkinson, C. H. Hinze et al., “Rituximab therapy for severe refractory chronic Henoch-Sch？nlein purpura,” Journal of Pediatrics, vol. 155, no. 1, pp. 136–139, 2009.
[10]
E. Pillebout, F. Rocha, L. Fardet, M. Rybojad, J. Verine, and D. Glotz, “Successful outcome using rituximab as the only immunomodulation in Henoch-Sch？nlein purpura: case report,” Nephrology Dialysis Transplantation, vol. 26, no. 6, pp. 2044–2046, 2011.
[11]
A. El-Husseini, A. Ahmed, A. Sabucedo, and E. Fabulo, “Refractory Henoch-Sch？nlein purpura: atypical aetiology and management,” Journal of Renal Care, vol. 39, pp. 77–81, 2013.
[12]
B. J. Foster, C. Bernard, K. N. Drummond, and A. K. Sharma, “Effective therapy for severe Henoch-Sch？nlein purpura nephritis with prednisone and azathioprine: a clinical and histophatologic study,” Journal of Pediatrics, vol. 136, no. 3, pp. 370–375, 2000.
[13]
J. I. Shin, J. M. Park, J. S. Lee, J. H. Kim, P. K. Kim, and H. J. Jeong, “Successful use of cyclosporin A in severe Sch？nlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine,” Clinical Rheumatology, vol. 25, no. 5, pp. 759–760, 2006.
[14]
M. Zaffanello and V. Fanos, “Treatment-based literature of Henoch-Sch？nlein purpura nephritis in childhood,” Pediatric Nephrology, vol. 24, no. 10, pp. 1901–1911, 2009.
[15]
Z. Tang, S.-M. Ji, D.-R. Chen et al., “Recurrent or de novo IgA nephropathy with crescent formation after renal transplantation,” Renal Failure, vol. 30, no. 6, pp. 611–616, 2008.
[16]
J. Gough, A. Yilmaz, S. Yilmaz, and H. Benediktsson, “Recurrent and de novo glomerular immune-complex deposits in renal transplant biopsies,” Archives of Pathology and Laboratory Medicine, vol. 129, no. 2, pp. 231–233, 2005.
[17]
K. K. Lau, H. Suzuki, J. Novak, and R. J. Wyatt, “Pathogenesis of Henoch-Sch？nlein purpura nephritis,” Pediatric Nephrology, vol. 25, no. 1, pp. 19–26, 2010.
[18]
F. T. Saulsbury, “Alterations in the O-linked glycosylation of IgA1 in children with Henoch-Sch？nlein purpura,” Journal of Rheumatology, vol. 24, no. 11, pp. 2246–2249, 1997.
