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Abstract：
This review aims to describe clinical and histological features, treatment, and prognosis in patients with anti-signal recognition particle (SRP) autoantibodies positive immune-mediated necrotizing myopathy (SRP-IMNM) based on previous findings.
Anti-SRP autoantibodies are specific in IMNM. Humoral autoimmune and inflammatory responses are the main autoimmune characteristics of SRP-IMNM. SRP-IMNM is clinically characterized by acute or subacute, moderately severe, symmetrical proximal weakness. Younger patients with SRP-IMNM tend to have more severe clinical symptoms. Patients with SRP-IMNM may be vulnerable to cardiac involvement, which ought to be regularly monitored and cardiac magnetic resonance imaging is the recommended detection method. The pathological features of SRP-IMNM are patchy or diffuse myonecrosis and myoregeneration accompanied by a paucity of inflammatory infiltrates. Endoplasmic reticulum stress-induced autophagy pathway and necroptosis are activated in skeletal muscle of SRP-IMNM. Treatment of refractory SRP-IMNM encounters resistance and warrants further investigation.
Anti-SRP autoantibodies define a unique population of IMNM patients. The immune and non-immune pathophysiological mechanisms are involved in SRP-IMNM.
Anti-SRP autoantibodies are specific in IMNM. Humoral autoimmune and inflammatory responses are the main autoimmune characteristics of SRP-IMNM. SRP-IMNM is clinically characterized by acute or subacute, moderately severe, symmetrical proximal weakness. Younger patients with SRP-IMNM tend to have more severe clinical symptoms. Patients with SRP-IMNM may be vulnerable to cardiac involvement, which ought to be regularly monitored and cardiac magnetic resonance imaging is the recommended detection method. The pathological features of SRP-IMNM are patchy or diffuse myonecrosis and myoregeneration accompanied by a paucity of inflammatory infiltrates. Endoplasmic reticulum stress-induced autophagy pathway and necroptosis are activated in skeletal muscle of SRP-IMNM. Treatment of refractory SRP-IMNM encounters resistance and warrants further investigation.
Anti-SRP autoantibodies define a unique population of IMNM patients. The immune and non-immune pathophysiological mechanisms are involved in SRP-IMNM.
摘要：
这篇综述旨在描述临床和组织学特征，治疗,根据以前的发现，抗信号识别颗粒（SRP）自身抗体阳性的免疫介导的坏死性肌病（SRP-IMNM）患者的预后。
抗SRP自身抗体在IMNM中是特异性的。体液自身免疫和炎症反应是SRP-IMNM的主要自身免疫特征。SRP-IMNM的临床特征为急性或亚急性，中度严重,对称近端弱点。患有SRP-IMNM的年轻患者倾向于具有更严重的临床症状。SRP-IMNM患者可能易受心脏受累，应定期监测和心脏磁共振成像是推荐的检测方法。SRP-IMNM的病理特征是斑片状或弥漫性肌坏死和肌肾再生，伴有少量炎性浸润。内质网应激诱导的自噬途径和坏死在SRP-IMNM骨骼肌中被激活。难治性SRP-IMNM的治疗遇到阻力，值得进一步研究。
抗SRP自身抗体定义了IMNM患者的独特群体。SRP-IMNM涉及免疫和非免疫病理生理机制。
抗SRP自身抗体在IMNM中是特异性的。体液自身免疫和炎症反应是SRP-IMNM的主要自身免疫特征。SRP-IMNM的临床特征为急性或亚急性，中度严重,对称近端弱点。患有SRP-IMNM的年轻患者倾向于具有更严重的临床症状。SRP-IMNM患者可能易受心脏受累，应定期监测和心脏磁共振成像是推荐的检测方法。SRP-IMNM的病理特征是斑片状或弥漫性肌坏死和肌肾再生，伴有少量炎性浸润。内质网应激诱导的自噬途径和坏死在SRP-IMNM骨骼肌中被激活。难治性SRP-IMNM的治疗遇到阻力，值得进一步研究。
抗SRP自身抗体定义了IMNM患者的独特群体。SRP-IMNM涉及免疫和非免疫病理生理机制。
