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老年患者常见可变免疫缺陷:长期临床经验

Common Variable Immunodeficiency in Elderly Patients: A Long-Term Clinical Experience.

作者信息

Danieli Maria Giovanna, Mezzanotte Cristina, Verga Jacopo Umberto, Menghini Denise, Pedini Veronica, Bilò Maria Beatrice, Moroncini Gianluca

机构信息

Department of Clinical and Molecular Sciences, Marche Polytechnic University, 60126 Ancona, Italy.

Department of Internal Medicine, Clinica Medica, Ospedali Riuniti, 60126 Ancona, Italy.

出版信息

Biomedicines. 2022 Mar 9;10(3):635. doi: 10.3390/biomedicines10030635.

Abstract

Background: Common variable immunodeficiency (CVID) is a complex, predominantly antibody deficiency usually diagnosed between 20−40 years. Few data about elderly patients are reported in the literature. Our aim was to evaluate the clinical phenotypes of elderly patients with CVID. Method: A retrospective analysis of adult patients with CVID was performed in our Referral Centre, focusing on the main differences between “older” patients (≥65 years at the diagnosis) and “younger” patients (<65 years). Results: The data from 65 younger and 13 older patients followed up for a median period of 8.5 years were available. At diagnosis, recurrent infections represented the only clinical manifestation in 61% and 69% of younger and older patients, respectively. The incidence of autoimmune diseases was higher in elderly patients compared with younger ones (30 vs. 18%, respectively). During the follow-up, the incidence of autoimmune disorders and enteropathy increased in the younger patients whereas neoplasia became the most prevalent complication in the elderly (38%). All patients received a replacement therapy with immunoglobulin, with good compliance. Conclusion: CVID occurrence in elderly patients is rarely described; therefore, the clinical characteristics are not completely known. In our series, neoplasia became the most prevalent complication in the elderly during the follow-up. In elderly patients, 20% SCIg was as safe as in the younger ones, with good compliance. A genetic analysis is important to confirm the diagnosis, identify specific presentations in the different ages, clarify the prognosis and guide the treatment. Future clinical research in this field may potentially help to guide their care.

摘要

背景

普通可变免疫缺陷(CVID)是一种复杂的、主要为抗体缺陷的疾病,通常在20至40岁之间被诊断出来。文献中报道的老年患者数据很少。我们的目的是评估老年CVID患者的临床表型。方法:在我们的转诊中心对成年CVID患者进行回顾性分析,重点关注“老年”患者(诊断时≥65岁)和“年轻”患者(<65岁)之间的主要差异。结果:有65名年轻患者和13名老年患者的数据可供分析,中位随访时间为8.5年。在诊断时,分别有61%的年轻患者和69%的老年患者仅表现为反复感染。老年患者自身免疫性疾病的发生率高于年轻患者(分别为30%和18%)。在随访期间,年轻患者自身免疫性疾病和肠病的发生率增加,而肿瘤形成成为老年患者最常见的并发症(38%)。所有患者均接受免疫球蛋白替代治疗,依从性良好。结论:老年患者中CVID的发生情况很少被描述;因此,其临床特征尚不完全清楚。在我们的系列研究中,肿瘤形成在随访期间成为老年患者最常见的并发症。在老年患者中,20%的皮下注射免疫球蛋白与年轻患者一样安全,依从性良好。基因分析对于确诊、识别不同年龄段的特定表现、明确预后和指导治疗很重要。该领域未来的临床研究可能有助于指导对他们的护理。

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