Department of Respiratory Medicine and Allergy, K85, Karolinska University Hospital Huddinge, Stockholm, SE-14186, Sweden.
Division of Immunology and Allergy, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden.
Curr Allergy Asthma Rep. 2024 Feb;24(2):39-51. doi: 10.1007/s11882-024-01126-0. Epub 2024 Jan 20.
The current article aims to provide a comprehensive update on diagnostic criteria for mast cell activation syndrome (MCAS), addressing challenges in diagnosing and classifying MCAS and its variants.
In recent years, there has been a significant increase in our knowledge regarding the underlying mechanisms responsible for the activation of mast cells (MCs) in various pathological conditions. Furthermore, a set of criteria and a classification for MCASs have been established. MCAS is characterized by the presence of typical clinical symptoms, a substantial elevation in serum tryptase levels during an attack compared to the patient's baseline tryptase levels, and a response to MC mediator-targeting therapy. In this report, a thorough examination was conducted on the contemporary literature relating to MCAS, with a focus on comparing the specificity, sensitivity, and robustness of MCAS-related parameters within proposals for diagnosing and classifying MCAS and its variants. Moreover, the significance of employing specific consensus criteria in the assessment and categorization of MCAS in individual patients was underscored, due to the escalating occurrence of patients receiving a misdiagnosis of MCAS based on nonspecific criteria.
本文旨在提供对肥大细胞活化综合征(MCAS)诊断标准的全面更新,讨论 MCAS 及其变异型的诊断和分类中的挑战。
近年来,我们对导致肥大细胞(MC)在各种病理条件下活化的潜在机制有了更深入的了解。此外,还建立了一套 MCAS 的标准和分类。MCAS 的特征是存在典型的临床症状、在发作期间与患者基础血清胰蛋白酶水平相比显著升高,以及对 MC 介质靶向治疗有反应。本报告对与 MCAS 相关的当代文献进行了全面检查,重点比较了 MCAS 相关参数在诊断和分类 MCAS 及其变异型中的特异性、敏感性和稳健性。此外,强调了在评估和分类个别患者的 MCAS 时使用特定共识标准的重要性,因为根据非特异性标准,越来越多的患者被误诊为 MCAS。