Doroudchi Mohammad-Ali, Stephens Alexis V, Wang Ziwei, Dhami Jaspreet, Butte Manish J
Division of Immunology, Allergy, and Rheumatology, Department of Pediatrics, University of California Los Angeles, Los Angeles, CA, USA.
Division of Pulmonary, Critical Care & Sleep Medicine, Department of Medicine, University of California Los Angeles, Los Angeles, CA, USA.
Clin Immunol. 2024 May;262:110176. doi: 10.1016/j.clim.2024.110176. Epub 2024 Mar 8.
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.
活化磷脂酰肌醇3-激酶δ综合征（APDS）是一种免疫缺陷病，临床表现多样，包括感染、免疫失调、自身免疫、淋巴细胞增殖和恶性肿瘤。免疫复合物介导的血管炎在APDS患者中尚未见报道。本文报告了3例APDS患者的病例系列，他们患有难治性IgA血管炎（也称为过敏性紫癜），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，导致局部组织损伤。来尼利西布是一种PI3K p110δ抑制剂，是美国食品药品监督管理局（FDA）批准用于治疗APDS的药物。来尼利西布治疗后，IgA血管炎得到缓解。对于包括IgA血管炎在内的免疫失调患者，应筛查是否患有APDS。
