Fenoglio Roberta, Naretto Carla, Basolo Bruno, Quattrocchio Giacomo, Ferro Michela, Mesiano Paola, Beltrame Giulietta, Roccatello Dario
Nephrology and Dialysis Unit, Center of Research of Immunopathology and Rare Diseases, Department of Rare, Immunologic, Hematologic and Immunohematologic Diseases, Giovanni Bosco Hospital and University of Turin, Turin, Italy.
Immunol Res. 2017 Feb;65(1):186-192. doi: 10.1007/s12026-016-8827-5.
Henoch-Schonlein purpura, also called IgA-vasculitis, is a systemic small vessels vasculitis with immunoglobulin A1-dominant immune deposits. The optimal treatment remains controversial. Because IgA-vasculitis is characterized by leukocyte infiltration of the blood vessel walls along with immunoglobulin A deposition, and because glucocorticosteroids inhibit inflammatory processes, early administration of glucocorticosteroids has been postulated to be effective, but this indication remains controversial. Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate) have been used in combination with glucocorticosteroids without definitive evidence of effectiveness. The efficacy of rituximab in adult IgA-vasculitis has been reported in few cases. We described a monocentric experience on the use of rituximab in adult IgA-vasculitis with biopsy-proven nephritis. The patients achieved a complete remission of nephritis and syndromic manifestations, and no patients experienced adverse reactions. These data have been compared with the limited literature nowadays available.
过敏性紫癜,又称IgA血管炎,是一种以免疫球蛋白A1为主的免疫沉积物的系统性小血管炎。最佳治疗方案仍存在争议。由于IgA血管炎的特征是血管壁白细胞浸润以及免疫球蛋白A沉积,且糖皮质激素可抑制炎症过程,因此有人推测早期使用糖皮质激素可能有效,但这一适应证仍存在争议。免疫抑制剂(硫唑嘌呤、环磷酰胺、环孢素、霉酚酸酯)已与糖皮质激素联合使用,但尚无明确有效的证据。利妥昔单抗治疗成人IgA血管炎的疗效仅在少数病例中有报道。我们描述了一项单中心使用利妥昔单抗治疗经活检证实为肾炎的成人IgA血管炎的经验。患者的肾炎和综合征表现完全缓解,且无患者出现不良反应。这些数据已与目前有限的文献进行了比较。