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老年与年轻显微镜下多血管炎(MPA)患者:单中心回顾性研究。

Elderly versus younger patients with microscopic polyangiitis vasculitis (MPA): a single-center retrospective study.

机构信息

Cumming School of Medicine, University of Calgary, Calgary, T2N 4N1, Canada.

Section of Rheumatology, Department of Internal Medicine, University of Calgary, Calgary, AB, T2N 4N1, Canada.

出版信息

Clin Rheumatol. 2024 Nov;43(11):3431-3438. doi: 10.1007/s10067-024-07122-z. Epub 2024 Sep 5.

Abstract

Microscopic polyangiitis (MPA) is a form of necrotizing vasculitis affecting the small vessels. Our study is the first study with the objective of describing the difference in clinical presentation of MPA and response to treatment at 6 months based on the age of disease onset. All patients seen at a tertiary vasculitis clinic between 2015 and 2023 with a diagnosis of MPA were included. Patients were divided into an elderly group (age >  = 65 years) and a younger group (age < 65). Comparative analysis was conducted to characterize differences amongst the elderly and younger patients, including differences in organ involvement and clinical presentation, Birmingham Vasculitis Activity Score (BVAS) scores, Vasculitis Damage Index( VDI) scores, and response to treatment at 6 months. Thirty-one patients were included in our study. Younger MPA patients (n = 18) with mean age at diagnosis of 53.17 years were compared with older MPA patients(n = 13) with mean age at diagnosis of 76.08 years. The younger patients had statistically significant higher BVAS scores (p = 0.009), along with higher incidence of renal (p = 0.028), pulmonary (p = 0.0069), and cutaneous (p = 0.026) manifestations at disease onset. Furthermore, amongst the younger population, there was noted statistically significant clinical improvement at 6 months following treatment induction, particularly in the domains of general symptoms (p = 0.011), MSK (p = 0.019), cutaneous (p = 0.011), and pulmonary symptoms (p = 0.04). In contrast, the elderly population presented with a predominant of non-specific constitutional symptoms, with statistically significant improvement in the domain of non-specific general symptoms at 6 months (p = 0.00008). All MPA patients responded well to treatment, with statistically significant improvement amongst younger patients (p = 0.0032), but no statistically significant improvement amongst elderly patients (p = 0.67). Our study findings concluded that MPA's clinical presentation differed by age group. Younger patients had more aggressive vasculitis disease presentation with better response to treatments, whereas, elderly patients had less severe disease presentation with predominant of general symptoms at disease onset. Key Points • MPA clinical presentation differed by age at disease onset. Younger patients presented with more aggressive and classic vasculitis-like presentations, with multi-system organ involvement and statistically significant higher incidence of renal, pulmonary, and cutaneous manifestations. In contrast, elderly patients had a predominant of constitutional and non-specific symptoms with often delayed diagnosis. • All MPA patients responded well to treatment. Amongst the younger population, there was statistically significant improvement in BVAS scores after treatment induction at 6 months; however, there was no statistically significant improvement of BVAS scores in the elderly population. • Future studies are required to further understand the difference in the clinical presentation of MPA based on the age at disease onset, and the implication on disease diagnosis and management.

摘要

显微镜下多血管炎(MPA)是一种影响小血管的坏死性血管炎。我们的研究首次旨在根据发病年龄描述 MPA 的临床表型差异和 6 个月时治疗反应。纳入了 2015 年至 2023 年间在三级血管炎诊所就诊的所有诊断为 MPA 的患者。将患者分为老年组(年龄≥65 岁)和年轻组(年龄<65 岁)。进行了对比分析,以描述老年和年轻患者之间的差异,包括器官受累和临床表现、Birmingham 血管炎活动评分(BVAS)评分、血管炎损伤指数(VDI)评分以及 6 个月时的治疗反应。我们的研究纳入了 31 名患者。年轻 MPA 患者(n=18)的平均发病年龄为 53.17 岁,与老年 MPA 患者(n=13)的平均发病年龄 76.08 岁进行了比较。年轻患者的 BVAS 评分显著更高(p=0.009),并且在发病时更常出现肾脏(p=0.028)、肺部(p=0.0069)和皮肤(p=0.026)表现。此外,在年轻人群中,在诱导治疗后 6 个月时,临床症状显著改善,尤其是在全身症状(p=0.011)、肌肉骨骼(p=0.019)、皮肤(p=0.011)和肺部症状(p=0.04)方面。相比之下,老年人群表现出以非特异性全身症状为主,在 6 个月时非特异性全身症状域的改善具有统计学意义(p=0.00008)。所有 MPA 患者对治疗反应良好,年轻患者的改善具有统计学意义(p=0.0032),但老年患者无统计学意义(p=0.67)。我们的研究结果表明,MPA 的临床表现因年龄组而异。年轻患者的血管炎表现更具侵袭性,治疗反应更好,而老年患者的疾病表现较轻,以全身症状为主。

关键点

  1. MPA 的临床表现因发病年龄而异。年轻患者表现出更具侵袭性和经典血管炎样表现,多系统器官受累,且肾脏、肺部和皮肤表现的发生率更高。相比之下,老年患者主要表现为全身性和非特异性症状,常常导致延迟诊断。

  2. 所有 MPA 患者对治疗反应良好。在年轻人群中,治疗诱导后 6 个月时 BVAS 评分有统计学意义的改善;然而,老年人群的 BVAS 评分无统计学意义的改善。

  3. 需要进一步研究以更好地了解基于发病年龄的 MPA 的临床表型差异,以及对疾病诊断和管理的影响。

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