Development of an algorithm for IgG4-related disease management

医学 IgG4相关疾病 算法 疾病 病理 计算机科学
作者
Olimpia Orozco-Gálvez,Andreu Fernández‐Codina,Marco Lanzillotta,Mikaël Ebbo,N. Schleinitz,Emma Culver,Vinciane Rebours,David D’Cruz,Emanuel Della‐Torre,Fernando Martínez‐Valle
出处
期刊:Autoimmunity Reviews [Elsevier]
卷期号:22 (3): 103273-103273 被引量:16
标识
DOI:10.1016/j.autrev.2023.103273
摘要

IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition affecting multiple organs lacking standardized management. In this article, we review the evidence available to provide European expert-based statements on the management of IgG4-RD which were integrated in a final algorithm. A panel of nine European experts in IgG4-RD from different specialties was asked to elaborate a set of consensus statements through a Delphi exercise. Three rounds of survey were taken. Consensus was reached when ≥75% of the responders agreed with a statement. Thirty-one statements on induction treatment, maintenance treatment, non-pharmacological treatment, and general considerations were assessed. Patients should be treated promptly in situations when there is an immediate organ threatened, or when organ damage is anticipated. Glucocorticoids (GC) are considered the first line of treatment and should be progressively tapered. Maintenance treatment is recommended for patients with high disease activity or with risk factors for relapse. Rituximab is effective for induction and maintenance of remission, but its use can be limited by economics. Low dose GC with or without GC-sparing agents can be used for maintenance therapy. Stenting or surgery should be ancillary to pharmacological treatment. Follow up should be based on physical examination, blood works, and imaging studies. Furthermore, it should be tailored on individual patient clinical history. 18-fluorodeoxyglucose positron emission tomography/computerized tomography may provide additional information over other imaging modalities. These new statements and algorithm reached a high degree of agreement and may help guiding the clinical management of IgG4-RD.
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