抗信号识别颗粒阳性坏死性肌病-干燥综合征重叠综合征：临床和肌病学特征的描述性研究。
Anti-signal recognition particle positive necrotizing myopathy-sjogren's syndrome overlap syndrome: a descriptive study on clinical and myopathology features.
机构信息
Department of Neurology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology, Jiefang Street 1095#, Wuhan, 430000, China.
Department of Geriatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
出版信息
BMC Musculoskelet Disord. 2023 Mar 23;24(1):219. doi: 10.1186/s12891-023-06354-5.
BACKGROUND AND OBJECTIVE
The aim of this study was to elucidate the clinical and myopathological characteristics of patients with anti-signal recognition particle (SRP) positive immune-mediated necrotizing myopathy (IMNM) overlap Sjogren's syndrome (SS).
MATERIALS AND METHODS
We retrospectively analyzed the data of anti-SRP positive IMNM patients admitted in the Neurology Department of Tongji Hospital between January 2011 to December 2020. Patients were divided into two groups: anti-SRP IMNM overlap SS group and anti-SRP IMNM control group. The clinical features, laboratory results, histological features, treatment, and prognosis were compared between the two groups.
RESULTS
A total of 30 patients with anti-SRP IMNM were included, including six anti-SRP IMNM overlap SS patients (two males, four females), with a median age of 39 years, and 24 anti-SRP IMNM patients (ten males, fourteen females), with a median age of 46 years. The anti-SRP IMNM overlap SS group had a lower prevalence of muscle atrophy (0 vs 50%, p = 0.019), and a higher prevalence of extramuscular manifestations, including cardiac abnormalities and ILD (Interstitial lung disease). CD4 + and CD68 + inflammatory infiltrations were significantly increased in anti-SRP IMNM overlap SS patients, with an increased presence of CD4 + cells in both necrotic(p = 0.023) and endomysial areas (p = 0.013), and more CD68 + cells (p = 0.016) infiltrated the endomysial area. Deposition of membrane attack complex (MAC) on sarcolemma (p = 0.013) was more commonly seen in the anti-SRP IMNM overlap SS group.
CONCLUSION
Our data revealed that anti-SRP IMNM-SS overlap patients may present with milder muscular manifestation, but worse extramuscular manifestations compared to anti-SRP IMNM patients without SS. CD4 + and CD68 + inflammatory infiltrations and MAC deposition were remarkably increased in anti-SRP IMNM-SS overlap patients.
背景与目的
本研究旨在阐明抗信号识别颗粒（SRP）阳性免疫介导性坏死性肌病（IMNM）合并干燥综合征（SS）患者的临床和肌病理特征。
材料与方法
我们回顾性分析了 2011 年 1 月至 2020 年 12 月期间在同济医院神经内科住院的抗-SRP 阳性 IMNM 患者的数据。患者分为两组：抗-SRP IMNM 合并 SS 组和抗-SRP IMNM 对照组。比较两组患者的临床特征、实验室结果、组织学特征、治疗和预后。
结果
共纳入 30 例抗-SRP IMNM 患者，其中抗-SRP IMNM 合并 SS 患者 6 例（男 2 例，女 4 例），中位年龄 39 岁，抗-SRP IMNM 患者 24 例（男 10 例，女 14 例），中位年龄 46 岁。抗-SRP IMNM 合并 SS 组肌肉萎缩的发生率较低（0%比 50%，p=0.019），而肌肉外表现，包括心脏异常和间质性肺病（ILD）的发生率较高。抗-SRP IMNM 合并 SS 患者的 CD4+和 CD68+炎症浸润明显增加，坏死区（p=0.023）和肌内膜区（p=0.013）CD4+细胞增多，且更多的 CD68+细胞（p=0.016）浸润肌内膜区。抗-SRP IMNM 合并 SS 组肌膜膜攻击复合物（MAC）沉积更为常见（p=0.013）。
结论
我们的数据表明，与无 SS 的抗-SRP IMNM 患者相比，抗-SRP IMNM-SS 重叠患者可能表现为更轻微的肌肉表现，但更严重的肌肉外表现。抗-SRP IMNM-SS 重叠患者的 CD4+和 CD68+炎症浸润和 MAC 沉积明显增加。
Zotero 插件