Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneous clinical manifestations of infections, immune dysregulation, autoimmunity; lymphoproliferation; and malignancy. Immune complex-mediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of immune complex-mediated vasculitis that activates complement and attracts neutrophils, macrophages and eosinophils to cause local tissue injury. Leniolisib is an inhibitor of PI3K p110δ and an FDA-approved treatment for APDS. IgA vasculitis resolved upon treatment with leniolisib. Patients with immune dysregulation including IgA vasculitis should be screened for APDS.

中文翻译：

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患有免疫复合物血管炎的 APDS 患者通过 leniolisib 得到缓解

激活的磷酸肌醇3激酶δ综合征（APDS）是一种先天性免疫缺陷，具有感染、免疫失调、自身免疫等异质性临床表现；淋巴增殖；和恶性肿瘤。 APDS 患者中尚未描述免疫复合物介导的血管炎。在这里，我们提供了三名患有难治性 IgA 血管炎（也称为过敏性紫癜）的 APDS 患者的病例系列，这是一种免疫复合物介导的血管炎，可激活补体并吸引中性粒细胞、巨噬细胞和嗜酸性粒细胞，从而导致局部组织损伤。 Leniolisib 是一种 PI3K p110δ 抑制剂，也是 FDA 批准的 APDS 治疗药物。 IgA 血管炎经 leniolisib 治疗后得到缓解。患有免疫失调（包括 IgA 血管炎）的患者应筛查 APDS。