伴有抗信号识别颗粒抗体的炎性肌病：100例患者的病例系列
Inflammatory myopathy with anti-signal recognition particle antibodies: case series of 100 patients.
作者信息
Suzuki Shigeaki, Nishikawa Atsuko, Kuwana Masataka, Nishimura Hiroaki, Watanabe Yurika, Nakahara Jin, Hayashi Yukiko K, Suzuki Norihiro, Nishino Ichizo
机构信息
Department of Neurology, Keio University School of Medicine, Tokyo, Japan.
Department of Neuromuscular Research, National Institute of Neuroscience, and Department of Clinical Development, Translational Medical Center, National Center of Neurology and Psychiatry, Tokyo, Japan.
出版信息
Orphanet J Rare Dis. 2015 May 13;10:61. doi: 10.1186/s13023-015-0277-y.
BACKGROUND
Anti-signal recognition particle (SRP) antibodies are used as serological markers of necrotizing myopathy, which is characterized by many necrotic and regenerative muscle fibers without or with minimal inflammatory cell infiltration. The clinical spectrum associated with anti-SRP antibodies seems to be broad.
OBJECTIVE
To describe the clinical characteristics, autoantibodies status, and neurological outcome associated with anti-SRP antibody.
METHODS
We studied clinical and laboratory findings of 100 patients with inflammatory myopathy and anti-SRP antibodies. Anti-SRP antibodies in serum were detected by the presence of 7S RNA using RNA immunoprecipitation. In addition, enzyme-linked immunosorbent assays (ELISAs) using a 54-kD protein of SRP (SRP54) and 3-hydroxyl-3-methylglutatyl-coenzyme A reductase (HMGCR) were also conducted.
RESULTS
The mean onset age of the 61 female and 39 male patients was 51 years (range 4-82 years); duration ≥ 12 months before diagnosis was seen in 23 cases. All patients presented limbs weakness; 63 had severe weakness, 70 neck weakness, 41 dysphagia, and 66 muscle atrophy. Extramuscular symptoms and associated disorders were infrequent. Creatine kinase levels were mostly more than 1000 IU/L. Histological diagnosis showed 84 patients had necrotizing myopathy, and apparent cell infiltration was observed in 16 patients. Anti-SRP54 antibodies were undetectable in 18 serum samples with autoantibodies to 7S RNA. Anti-HMGCR antibodies were positive in 3 patients without the statin treatment, however, were negative in 5 patients with statin-exposure at disease onset. All but 3 patients were treated by corticosteroids and 62 (77 %) of these 81 patients required additional immunotherapy. After 2-years treatment, 22 (27 %) of these 81 patients had poor neurological outcomes with modified Rankin scale scores of 3-5. Multivariate analysis revealed that pediatric disease onset was associated with the poor outcomes.
CONCLUSION
Anti-SRP antibodies are associated with different clinical courses and histological presentations.
背景
抗信号识别颗粒（SRP）抗体用作坏死性肌病的血清学标志物，其特征为存在许多坏死和再生的肌纤维，无炎症细胞浸润或炎症细胞浸润极少。与抗SRP抗体相关的临床谱似乎很广。
目的
描述与抗SRP抗体相关的临床特征、自身抗体状态及神经学转归。
方法
我们研究了100例炎性肌病伴抗SRP抗体患者的临床和实验室检查结果。采用RNA免疫沉淀法通过检测7S RNA来检测血清中的抗SRP抗体。此外，还进行了使用SRP的54-kD蛋白（SRP54）和3-羟基-3-甲基戊二酰辅酶A还原酶（HMGCR）的酶联免疫吸附测定（ELISA）。
结果
61例女性和39例男性患者的平均发病年龄为51岁（范围4 - 82岁）；23例患者在诊断前病程≥12个月。所有患者均出现肢体无力；63例有严重无力，70例有颈部无力，41例有吞咽困难，66例有肌肉萎缩。肌外症状及相关疾病不常见。肌酸激酶水平大多超过1000 IU/L。组织学诊断显示84例患者有坏死性肌病，16例患者观察到明显的细胞浸润。在18份对7S RNA有自身抗体的血清样本中未检测到抗SRP54抗体。3例未接受他汀类药物治疗的患者抗HMGCR抗体呈阳性，然而，5例疾病发作时暴露于他汀类药物的患者抗HMGCR抗体呈阴性。除3例患者外，所有患者均接受了糖皮质激素治疗，这81例患者中有62例（77%）需要额外的免疫治疗。经过2年治疗，这81例患者中有22例（27%）神经学转归较差，改良Rankin量表评分为3 - 5分。多因素分析显示儿童期发病与不良转归相关。
结论
抗SRP抗体与不同的临床病程和组织学表现相关。
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