Longitudinal Course of Disease in a Large Cohort of Myositis Patients With Autoantibodies Recognizing the Signal Recognition Particle.
Longitudinal Course of Disease in a Large Cohort of Myositis Patients With Autoantibodies Recognizing the Signal Recognition Particle.
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大量肌炎自身抗体识别信号识别颗粒的肌炎患者的纵向病程。
DOI：
10.1002/acr.22920
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发表时间：
2017-02
影响因子：
4.7
通讯作者：
Mammen, Andrew L.
中科院分区：
文献类型：
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作者：
Pinal-Fernandez, Iago;Parks, Cassie;Werner, Jessie L.;Albayda, Jemima;Paik, Julie J.;Danoff, Sonye K.;Casciola-Rosen, Livia;Christopher-Stine, Lisa;Mammen, Andrew L.
免疫介导性坏死性肌病(IMNM)患者常出现识别信号识别颗粒(SRP)或HMG-CoA还原酶(HMGCR)的自身抗体。在这里，我们研究了一组抗SRP患者，以确定与疾病严重程度和临床改善相关的因素；我们还比较了抗SRP自身抗体和抗HMGCR自身抗体患者虚弱的严重程度。纳入2002年至2015年约翰霍普金斯肌炎队列中的所有抗SRP患者。在每次访问中记录有关近端肌力、肌酸激酶(CK)水平和免疫抑制治疗的纵向信息。使用单变量和多变量多水平回归模型来评估影响康复的预后因素。将抗SRP患者的强度与之前描述的49名抗HMGCR受试者的强度进行比较。对37例抗SRP患者和380例门诊就诊资料进行分析。初诊年龄越小，初次就诊(p=0.02)和以后所有就诊(p=0.002)时的虚弱程度越严重。只有50%的患者在治疗4年后达到接近完全或完全强壮的程度，其中大多数人的CK水平持续升高。利妥昔单抗对17例抗SRP患者中的13例有效。抗SRP抗体阳性患者显著弱于抗HMGCR自身抗体阳性患者(−为1.3分，p=0.001)。发病年龄越小，抗SRP肌炎的症状越严重。此外，即使在强度随着免疫抑制而改善的抗SRP患者中，大多数患者仍有持续的疾病活动，CK水平升高证明了这一点。最后，抗SRP患者明显弱于抗HMGCR患者，这提供了这些自身抗体与不同形式的IMNM相关的证据。
Patients with immune-mediated necrotizing myopathy (IMNM) often have autoantibodies recognizing the signal recognition particle (SRP) or HMG-CoA reductase (HMGCR). Here, we studied a cohort of anti-SRP patients to identify factors associated with disease severity and clinical improvement; we also compared the severity of weakness in those with anti-SRP versus anti-HMGCR autoantibodies. All anti-SRP patients in the Johns Hopkins Myositis Cohort from 2002 to 2015 were included. Longitudinal information regarding proximal muscle strength, creatine kinase (CK) levels, and immunosuppressive therapy were recorded at each visit. Univariate and multivariate multilevel regression models were used to assess prognostic factors influencing recovery. Strength in the anti-SRP patients was compared to strength in 49 previously described anti-HMGCR subjects. Data from 37 anti-SRP patients and 380 total clinic visits was analyzed. Younger age at onset was associated with more severe weakness at the first visit (p=0.02) and all subsequent visits (p=0.002). Only 50% of patients reached near-full or full strength after 4 years of treatment and most of these continued to have elevated CK levels. Rituximab appeared to be effective in 13 of 17 anti-SRP patients. Anti-SRP patients were significantly weaker than those with anti-HMGCR autoantibodies (−1.3 strength points, p=0.001). Younger age at onset is associated with more severe weakness in anti-SRP myositis. Furthermore, even among anti-SRP patients whose strength improved with immunosuppression, most had ongoing disease activity as demonstrated by elevated CK levels. Finally, anti-SRP patients were significantly weaker than anti-HMGCR patients, providing evidence that these autoantibodies are associated with distinct forms of IMNM.
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影响因子：
--
作者：
Werner, Jessie L.;Christopher-Stine, Lisa;Ghazarian, Sharon R.;Pak, Katherine S.;Kus, Jordan E.;Daya, Natalie R.;Lloyd, Thomas E.;Mammen, Andrew L.
通讯作者：
Mammen, Andrew L.
影响因子：
27.4
作者：
Hengstman, G. J. D.;ter Laak, H. J.;van Engelen, B. G. M.
通讯作者：
van Engelen, B. G. M.
影响因子：
4.7
作者：
Valiyil, Ritu;Casciola-Rosen, Livia;Hong, Grace;Mammen, Andrew;Christopher-Stine, Lisa
通讯作者：
Christopher-Stine, Lisa
影响因子：
10.9
作者：
Valenzuela, Antonia;Chung, Lorinda;Casciola-Rosen, Livia;Fiorentino, David
通讯作者：
Fiorentino, David
影响因子：
3.3
作者：
Gallagher, D;Visser, M;Heymsfield, SB
通讯作者：
Heymsfield, SB