布劳综合征是一种影响儿童的罕见遗传性肉芽肿疾病。它可能导致威胁视力的并发症和关节变形。由于这种疾病的罕见性，目前还没有标准化的治疗指南。本研究旨在提供关于布劳综合征不同治疗选择的最新概述。我们在PubMed数据库中调查了布劳综合征患者使用的不同治疗方法，并提出了一种疾病管理的治疗算法。高剂量的皮质类固醇被认为是布劳综合征的过渡治疗。如果患者有关节或眼部受累，应开启甲氨蝶呤治疗。对于患有葡萄膜炎或残留性关节炎的患者，应加用抗肿瘤坏死因子α药物。如果患者仍有症状，切换到另一种抗肿瘤坏死因子α药物是最佳选择。对于对第一线和第二线生物治疗无效的患者，切换到抗白细胞介素 1、抗白细胞介素 6 或 tofacitinib是必要的。本文提出了一种布劳综合征治疗算法。还需要其他研究来确认这些治疗的有效性。•布劳综合征是一种罕见但严重的肉芽肿病，可能导致威胁视力的并发症和关节变形。•布劳综合征似乎对治疗具有耐药性。•高剂量皮质类固醇通常是不足够的，只能作为过渡治疗考虑。•布劳综合征可能被视为葡萄膜炎的不良因素，因此，对于患有葡萄膜炎或残留性关节炎的患者，应开启抗肿瘤坏死因子α治疗。© 2023. 作者（在专属许可下）授权给施普林格出版社德国 GmbH，施普林格自然出版集团的一部分。

Blau syndrome is a rare genetic granulomatosis affecting children. It could be responsible for vision-threatening complications and articular deformation. Due to the rarity of this disease, there are no standardized guidelines for its management. This work aimed to provide an updated overview of the different therapeutic options for Blau syndrome. We conducted research in the PubMed database for the different treatments used in Blau syndrome patients, and we proposed a therapeutic algorithm for disease management. High doses of corticosteroids are considered as a bridging therapy in Blau syndrome. Methotrexate should be initiated if the patient has articular or ocular involvement. An anti-tumor necrosis factor α should be added for patients with uveitis or residual arthritis. If the patient remains symptomatic, a switch to another anti-tumor necrosis factor α is the best option. In non-responders to the first- and second-line biotherapies, a switch to an anti-interleukin 1, an anti-interleukin 6, or tofacitinib is necessary.This article suggested an algorithm for the treatment of Blau syndrome. Other studies are necessary to confirm the efficacy of these treatments.• Blau syndrome is a rare but severe granulomatosis that could be responsible for vision-threatening complications and articular deformation. • Blau syndrome seems to be refractory to treatments.• High doses of corticosteroids are usually insufficient and should be considered only as a bridging therapy. • Blau syndrome could be considered as a poor factor for uveitis, thus, an anti-tumor necrosis factor α should be initiated for patients with uveitis or with residual arthritis.© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.