Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany.
Urticaria Center of Reference and Excellence (UCARE), Institute of Allergology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology (ITMP), Allergology and Immunology, Berlin, Germany.
J Allergy Clin Immunol Pract. 2023 Aug;11(8):2417-2425.e1. doi: 10.1016/j.jaip.2023.02.006. Epub 2023 Feb 16.
Two endotypes of chronic spontaneous urticaria (CSU) associated with mast cell-activating autoantibodies are described, namely autoallergic chronic spontaneous urticaria (aaCSU; with immunoglobulin E [IgE]-anti-autoallergens) and autoimmune chronic spontaneous urticaria (aiCSU; with IgG-anti-high-affinity receptor for the Fc region of immunoglobulin E [FcεRI]/IgE).
To investigate the rates of CSU patients with aaCSU and aiCSU.
We analyzed 111 CSU patients for aaCSU (ie, IgE to thyroid peroxidase, interleukin 24) and for aiCSU (ie, a positive autologous serum skin and Basophil Activation Test plus immunoglobulin G [IgG]-anti-FcεRI/IgE). Clinical and laboratory parameters were compared in patients with aaCSU, aiCSU, and both.
Across 111 patients with CSU, 64 (58%) had aaCSU and 9 (8%) had aiCSU. Eight of the 9 aiCSU patients had aaCSU, but only 8 of 64 patients with aaCSU had aiCSU. In total, 7% (8 of 111) of patients had both aiCSU and aaCSU, 41% (46 of 111) had neither, and 16% (18 of 111) tested negative for all markers of aaCSU and aiCSU assessed. Patients with aaCSU or aiCSU are different from those without: patients with stand-alone aaCSU tend to be younger than non-aaCSU patients, aiCSU, and aaCSU/aiCSU overlapping subpopulations. In contrast, patients with aiCSU, with or without aaCSU coexistence, are more often female, have higher levels of thyroid peroxidase autoantibodies (both IgG and IgE), and show more severe quality of life impairment.
Our novel finding that aiCSU coexisting with aaCSU needs to be confirmed in bigger cohorts and multicenter studies. Autoimmunity driven by autoreactive IgE and/or IgG in CSU needs further investigation for better understanding of the pathophysiology.
描述了两种与肥大细胞激活自身抗体相关的慢性自发性荨麻疹(CSU)表型,即自身过敏型慢性自发性荨麻疹(aaCSU;伴免疫球蛋白 E [IgE]-抗自身过敏原)和自身免疫型慢性自发性荨麻疹(aiCSU;伴 IgG-抗高亲和力免疫球蛋白 E(FcεRI)/IgE 受体)。
调查 aaCSU 和 aiCSU 的 CSU 患者比例。
我们分析了 111 例 CSU 患者的 aaCSU(即甲状腺过氧化物酶 IgE、白细胞介素 24)和 aiCSU(即自身血清皮肤阳性和嗜碱性粒细胞激活试验加 IgG-抗 FcεRI/IgE)。比较了 aaCSU、aiCSU 和两者均有的患者的临床和实验室参数。
在 111 例 CSU 患者中,64 例(58%)有 aaCSU,9 例(8%)有 aiCSU。9 例 aiCSU 患者中有 8 例同时有 aaCSU,但 64 例 aaCSU 患者中仅有 8 例有 aiCSU。总的来说,7%(8/111)的患者同时有 aiCSU 和 aaCSU,41%(46/111)的患者两者均无,16%(18/111)的患者评估的 aaCSU 和 aiCSU 的所有标志物均为阴性。有 aaCSU 或 aiCSU 的患者与无 aaCSU 的患者不同:有单纯 aaCSU 的患者往往比非 aaCSU 患者、aiCSU 和 aaCSU/aiCSU 重叠亚群年轻。相比之下,有 aiCSU 的患者,无论是否共存 aaCSU,更多为女性,甲状腺过氧化物酶自身抗体(IgG 和 IgE 均)水平更高,生活质量受损更严重。
我们的新发现,aiCSU 与 aaCSU 共存需要在更大的队列和多中心研究中进一步证实。CSU 中由自身反应性 IgE 和/或 IgG 驱动的自身免疫需要进一步研究,以更好地了解其病理生理学。