
Journal of Diagnostics Concepts & Practice››2022,Vol. 21››Issue (03): 408-414.doi:10.16150/j.1671-2870.2022.03.021
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ZHANG Hao, CHI Huihui, SU Yutong, YANG Chengde(
)
Received:2022-01-20Online:2022-06-25Published:2022-08-17Contact:YANG Chengde E-mail:yangchengde@sina.comCLC Number:
ZHANG Hao, CHI Huihui, SU Yutong, YANG Chengde. Study on advances in the association between autoantibodies and clinical phenotypes in idiopathic inflammatory myopathy[J]. Journal of Diagnostics Concepts & Practice, 2022, 21(03): 408-414.
| 肌炎特异性 自身抗体 |
肌炎患者中 检测阳性率 |
相关肌 炎类型 |
病理 | 肺 | 皮肤 | 肌肉 | 肿瘤相关性 | 其他 | 预后 |
|---|---|---|---|---|---|---|---|---|---|
| 抗ARS |
成人:21%~30%,儿童:5%~8% |
抗合成酶综合征 |
抗Jo-1阳性以束周坏死多见 |
多数起病时没有肺部累及的患者,随后会进展为间质性肺病 | 皮肌炎典型皮疹常见 |
肌肉累及程度因抗体类型不同 | 暂无报道 |
常有雷诺现象、技工手、关节炎、发热等症状 | 多数预后良好,但合并严重间质性肺病者预后较差 |
| 抗MDA5 |
成人:6%~36%,儿童:8%~28% |
皮肌炎,临床无肌病性皮肌炎 |
血管纤维蛋白沉积为特征的血管病变,伴血管周围炎症 | 肺部累及多见 |
皮肌炎典型皮疹常见,可伴皮肤溃疡 |
肌肉症状常较轻或不累及肌肉 | 暂无报道 |
无 |
合并严重间质性肺病者预后差,死亡率高 |
| 抗SAE |
成人:2%~8%,儿童:1% |
皮肌炎 |
/ |
较少累及 |
皮肌炎典型皮疹常见 |
起病时较少累及肌肉,随病情进展可逐渐累及 | 有报道与肺癌、消化系统肿瘤及妇科肿瘤相关 | 无 |
预后良好 |
| 抗Mi-2 |
成人:5%~10%,儿童:2%~4% |
皮肌炎 |
可有严重的肌肉纤维化及炎细胞浸润 | 较少累及 |
皮肌炎典型皮疹常见 |
肌肉症状常较严重 |
有报道 |
无 |
治疗反应较好,复发率低 |
| 抗NXP2 |
成人:4%~12%,儿童:16%~23% |
皮肌炎,肿瘤相关性肌炎 |
可见CD4+T细胞浸润 |
较少累及 |
皮肌炎典型皮疹常见 |
起病时肌肉症状较重,可伴吞咽困难 | 有报道 |
与钙质沉着相关 |
复发率较高 |
| 抗TIF1-γ |
成人:4%~13%,儿童:16%~35% |
皮肌炎,肿瘤相关性肌炎 |
/ |
较少累及 |
严重皮疹包括向阳疹,红白斑块等 | 肌肉症状常较轻 |
与成人(>50岁)恶性肿瘤密切相关 | 与钙质沉着相关 |
合并肿瘤的患者预后较差,生存率较低 |
| 抗SRP |
成人:4%~15%,儿童:1%~4% |
免疫介导的坏死性肌病 |
肌纤维坏死及较少的淋巴细胞或炎症浸润 | 较少累及 |
较少出现皮疹且通常不典型 |
严重且快速进展的对称性近端肌无力症状 | 暂无报道 |
可累及心脏 |
治疗反应常较差,病死率较高 |
| 抗HMGCR |
成人:6%~10%,儿童:1% |
免疫介导的坏死性肌病 |
肌纤维坏死及较少的淋巴细胞或炎症浸润 | 较少累及 |
较少出现皮疹且通常不典型 |
严重且快速进展的对称性近端肌无力症状 | 有报道与罹患肿瘤风险增加相关 |
与他汀暴露关系密切 |
年轻患者较年长患者预后差 |
| 抗cN1A |
包涵体肌炎中阳性率35%~50% | 包涵体肌炎 | 可见嗜酸性胞质包涵体 | 较少累及 |
皮疹不常见 |
肌酸激酶水平轻度升高 | 暂无报道 |
可见于其他自身免疫疾病 | 死亡风险增高 |
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