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英国首次关于IgG4相关疾病诊断与管理的跨地区专家多学科会议的经验。

Experience from the first UK inter-regional specialist multidisciplinary meeting in the diagnosis and management of IgG4-related disease.

作者信息

Goodchild George, Peters Rory Jr, Cargill Tamsin N, Martin Harry, Fadipe Adetokunbo, Leandro Maria, Bailey Adam, Collier Jane, Firmin Louisa, Chouhan Manil, Rodriguez-Justo Manuel, Sadler Ross, Chapman Roger W, Bungay Helen, Fryer Eve, David Joel, Luqmani Raashid, Barnes Eleanor, Webster George J, Culver Emma L

机构信息

University College London Hospitals NHS Foundation Trust, London, UK.

Oxford University Hospitals NHS Foundation Trust, Oxford, UK and University of Oxford, Oxford, UK.

出版信息

Clin Med (Lond). 2020 May;20(3):e32-e39. doi: 10.7861/clinmed.2019-0457.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a complex multisystem fibro-inflammatory disorder, requiring diagnostic differentiation from malignancy and other immune-mediated conditions, and careful management to minimise glucocorticoid-induced toxicity and prevent progressive organ dysfunction. We describe the experience of the first inter-regional specialist IgG4-RD multidisciplinary team meeting (MDM) incorporating a broad range of generalists and specialists, held 6-weekly via web-link between Oxford University Hospitals NHS Foundation Trust and University College London Hospitals NHS Foundation Trust. Over 3 years, there were 206 discussions on 156 patients. Of these, 97 (62%) were considered to have definite or possible IgG4-RD; 67% had multi-organ involvement and 23% had a normal serum IgG4. The average number of specialist opinions sought prior to MDM was four per patient. Management was changed in the majority of patients (74%) with the treatment escalation recommended in 61 cases, including 19 for rituximab. Challenges arose from delays and misdiagnosis, cross-specialty presentation and the management of sub-clinical disease. Our cross-discipline IgG4-RD MDM enabled important diagnostic and management decisions in this complex multisystem disorder, and can be used as a model for other centres in the UK.

摘要

免疫球蛋白G4相关性疾病(IgG4-RD)是一种复杂的多系统纤维炎症性疾病,需要与恶性肿瘤及其他免疫介导性疾病进行鉴别诊断,并需谨慎管理以尽量减少糖皮质激素诱导的毒性反应并预防进行性器官功能障碍。我们介绍了首次跨地区专科IgG4-RD多学科团队会议(MDM)的经验,该会议由广泛的通科医生和专科医生参与,通过牛津大学医院国民保健服务信托基金和伦敦大学学院医院国民保健服务信托基金之间的网络链接每6周举行一次。在3年多的时间里,针对156例患者进行了206次讨论。其中,97例(62%)被认为患有确诊或可能的IgG4-RD;67%的患者有多器官受累,23%的患者血清IgG4水平正常。在MDM之前,每位患者平均寻求四种专科意见。大多数患者(74%)的管理方案发生了改变,61例建议加强治疗,其中19例使用利妥昔单抗。延迟诊断和误诊、跨专科表现以及亚临床疾病的管理带来了挑战。我们的跨学科IgG4-RD MDM为这种复杂的多系统疾病做出了重要的诊断和管理决策,可作为英国其他中心的范例。

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