Sirolimus as a possible treatment in Cleavage-resistant RIPK1-induced autoinflammatory (CRIA) syndrome.

IF 8.2 1区医学 Q1 ALLERGY

Journal of Allergy and Clinical Immunology-In Practice Pub Date : 2024-11-16 DOI:10.1016/j.jaip.2024.11.003

Marco Cattalini, Manuela Cortesi, Manuela Baronio, Antonella Insalaco, Fabrizio de Benedetti, Vassilios Lougaris, Raffaele Badolato

引用次数: 0

Abstract

Clinical implications: We present the case of a girl with CRIA syndrome treated with sirolimus with significant reduction in the number of days with fever, the total dose of glucocorticoids used and the inflammatory markers levels, together with a reduction in the double negative T-cells and control of PI3K/mTor Activity. We propose sirolimus as a valid therapeutic choice in patients with CRIA syndrome.

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西罗莫司可用于治疗抗裂解 RIPK1 诱导的自身炎症（CRIA）综合征。

临床意义：我们介绍了一例西罗莫司治疗 CRIA 综合征女孩的病例，该治疗显著减少了发热天数、糖皮质激素总剂量和炎症指标水平，同时减少了双阴性 T 细胞，控制了 PI3K/mTor 活性。我们建议西罗莫司作为CRIA综合征患者的有效治疗选择。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Allergy and Clinical Immunology-In Practice ALLERGYIMMUNOLOGY-IMMUNOLOGY

CiteScore

11.10

自引率

9.60%

发文量

683

审稿时长

50 days

期刊介绍： JACI: In Practice is an official publication of the American Academy of Allergy, Asthma & Immunology (AAAAI). It is a companion title to The Journal of Allergy and Clinical Immunology, and it aims to provide timely clinical papers, case reports, and management recommendations to clinical allergists and other physicians dealing with allergic and immunologic diseases in their practice. The mission of JACI: In Practice is to offer valid and impactful information that supports evidence-based clinical decisions in the diagnosis and management of asthma, allergies, immunologic conditions, and related diseases. This journal publishes articles on various conditions treated by allergist-immunologists, including food allergy, respiratory disorders (such as asthma, rhinitis, nasal polyps, sinusitis, cough, ABPA, and hypersensitivity pneumonitis), drug allergy, insect sting allergy, anaphylaxis, dermatologic disorders (such as atopic dermatitis, contact dermatitis, urticaria, angioedema, and HAE), immunodeficiency, autoinflammatory syndromes, eosinophilic disorders, and mast cell disorders. The focus of the journal is on providing cutting-edge clinical information that practitioners can use in their everyday practice or to acquire new knowledge and skills for the benefit of their patients. However, mechanistic or translational studies without immediate or near future clinical relevance, as well as animal studies, are not within the scope of the journal.