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北京大学学报（医学版） ›› 2021, Vol. 53 ›› Issue (6): 1088-1093. doi: 10.19723/j.issn.1671-167X.2021.06.014

• 论著 • 上一篇    下一篇

88例重叠肌炎的临床及免疫学特征

肖云抒1,朱冯赟智2,罗澜2,邢晓燕2,李玉慧2,△(),张学武2,沈丹华1   

  1. 1.北京大学人民医院病理科,北京 100044
    2.北京大学人民医院风湿免疫科,北京 100044
  • 收稿日期:2021-08-08 出版日期:2021-12-18 发布日期:2021-12-13
  • 通讯作者: 李玉慧 E-mail:liyuhui84@163.com
  • 基金资助:
    国家自然科学基金(81801617);国家自然科学基金(81771678);国家自然科学基金(81971520);北京大学人民医院研究与发展基金(RDX2020-03)

Clinical and immunological characteristics of 88 cases of overlap myositis

XIAO Yun-shu1,ZHU Feng-yun-zhi2,LUO Lan2,XING Xiao-yan2,LI Yu-hui2,△(),ZHANG Xue-wu2,SHEN Dan-hua1   

  1. 1. Department of Pathology, Peking University People’s Hospital, Beijing 100044, China
    2. Department of Rheumatology and Immunology, Peking University People’s Hospital, Beijing 100044, China
  • Received:2021-08-08 Online:2021-12-18 Published:2021-12-13
  • Contact: Yu-hui LI E-mail:liyuhui84@163.com
  • Supported by:
    National Natural Science Foundation of China(81801617);National Natural Science Foundation of China(81771678);National Natural Science Foundation of China(81971520);Peking University People’s Hospital Research and Development Funds(RDX2020-03)

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摘要：

目的:探讨重叠肌炎 (overlap myositis, OM) 患者的临床及免疫学特征。方法:对2004年1月至2020年8月于北京大学人民医院住院的特发性炎性肌病 (idiopathic inflammatory myopathies,IIMs) 患者资料进行回顾性分析,包括人口学特征、临床症状、实验室指标、免疫学指标(抗核抗体、类风湿因子、肌炎相关性抗体、肌炎特异性抗体)、生存情况,并比较OM和其他炎性肌病患者的临床和实验室特点及预后差别。结果:共纳入368例IIMs患者,其中OM患者占23.9% (88/368)。OM患者女性占85.2% (75/88),中位病程13.5个月,结缔组织病(connective tissue disease, CTD)合并皮肌炎(dermatomyositis, DM)、多肌炎(polymyositis, PM)、免疫介导坏死性肌病(immune-mediated necrotizing myopathy, IMNM)、抗合成酶综合征(anti-synthetase syndrome, ASS)分别为 60.2%、3.4%、2.3%、 34.1%。OM患者和非OM患者相比,女性比例高(85.2% vs. 72.1%, P=0.016),病程长[13.5(4.5, 48.0)月 vs. 4.0(2.0,12.0)月,P<0.001)]。在临床特点方面,OM患者比非OM患者V型疹(25.0% vs. 44.6%, P=0.001)、甲周红斑(8.0% vs. 19.6%, P=0.013)发生率低,雷诺现象(14.8% vs. 1.8%, P<0.001)、间质性肺炎(88.6% vs. 72.1%, P=0.001)、肺动脉高压(22.7% vs. 7.5%, P<0.001)、心脏受累(18.2% vs. 9.3%, P=0.033)发生率高。在免疫学特点方面,天门冬氨酸氨基转移酶升高比例低(31.8% vs. 45.0%, P=0.035), C反应蛋白升高比例高(58.0% vs. 44.6%, P=0.037),抗核抗体(85.1% vs. 63.4%, P=0.001)、类风湿因子(40.2% vs. 17.8%, P<0.001)、抗Ro-52抗体(71.6% vs. 56.1%,P=0.038)阳性率高。OM患者和非OM患者的预后无明显差异。结论:OM出现脏器受累多见,易合并肺动脉高压、心脏损害。

关键词: 重叠肌炎, 特发性炎性肌病, 肺动脉高压, 自身抗体

Abstract:

Objective: To investigate the clinical and immunological characteristics of overlap myositis (OM) patients. Methods: The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People’s Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron’s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic’s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud’s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival. Results: A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5,48.0) months vs. 4.0(2.0,12.0) months, P<0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud’s phenomenon (14.8% vs. 1.8%, P<0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P<0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P<0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients. Conclusion: Pulmonary hypertension and myocardial involvement were frequently observed in OM.

Key words: Overlap myositis, Idiopathic inflammatory myopathy, Pulmonary hypertension, Autoantibodies

中图分类号: 

  • R593.26

表1

OM患者临床特征"

Variables OM (n=88) Non-OM (n=280) P (OM vs. non-OM)
Female,n (%) 75 (85.2) 202 (72.1) 0.016
Age on set/years,$\overline{x}$±s 50.3±14.4 49.2±14.0 0.525
Duration/months,M (P25,P75) 13.5 (4.5, 48.0) 4.0 (2.0, 12.0) <0.001
Fever,n (%) 40 (45.5) 107 (38.2) 0.262
Gottron’s sign /papules,n (%) 62 (70.5) 186 (66.4) 0.517
Heliotrope rash,n (%) 29 (33.0) 124 (44.3) 0.064
V-sign,n (%) 22 (25.0) 125 (44.6) 0.001
Shawl sign,n (%) 14 (15.9) 72 (25.7) 0.062
Mechanic’s hands,n (%) 35 (39.8) 88 (31.4) 0.156
Skin ulceration,n (%) 6 (6.8) 16 (5.7) 0.796
Periungual erythema,n (%) 7 (8.0) 55 (19.6) 0.013
Subcutaneous calcinosis,n (%) 1 (1.1) 7 (2.5) 0.686
Dysphagia,n (%) 4 (4.5) 5 (1.8) 0.226
Myalgia,n (%) 18 (20.5) 35 (12.5) 0.081
Myasthenia,n (%) 45 (51.1) 156 (55.7) 0.464
Arthritis,n (%) 48 (54.6) 125 (44.6) 0.113
Raynaud’s phenomenon,n (%) 13 (14.8) 5 (1.8) <0.001
Interstitial lung disease,n (%) 78 (88.6) 202 (72.1) 0.001
Pulmonary hypertension,n (%) 20 (22.7) 21 (7.5) <0.001
Myocardial involvement,n (%) 16 (18.2) 26 (9.3) 0.033

表2

OM患者的免疫学特征"

Variables OM (n=88) Non-OM (n=280) P (OM vs. non-OM)
Elevated ALT,n (%) 32 (36.4) 131 (46.8) 0.109
Elevated AST,n (%) 28 (31.8) 126 (45.0) 0.035
Elevated LDH,n (%) 68 (77.3) 240 (85.7) 0.070
Elevated CK,n (%) 27 (30.7) 112 (40.0) 0.131
Elevated ESR,n (%) 54 (61.4) 145 (51.8) 0.141
Elevated CRP,n (%) 51 (58.0) 125 (44.6) 0.037
ANA positivity,n (%) 57/67 (85.1) 137/216 (63.4) 0.001
RF positivity,n (%) 33/82 (40.2) 43/241 (17.8) <0.001
Anti-Mi-2 positivity,n (%) 2/52 (3.9) 6/100 (6.0) 0.716
Anti-TIF-1γ positivity,n (%) 3/37 (8.1) 6/79 (7.6) 1.000
Anti-MDA5 positivity,n (%) 14/44 (31.8) 33/91 (36.3) 0.701
Anti-NXP2 positivity,n (%) 3/36 (8.3) 9/80 (11.3) 0.752
Anti-SAE1 positivity,n (%) 3/38 (7.9) 6/78 (7.7) 1.000
Anti-synthetase positivity,n (%) 34/60 (56.7) 66/118 (55.9) 1.000
Anti-SRP positivity,n (%) 8/54 (14.8) 7/99 (7.1) 0.156
Anti-Ku positivity,n (%) 6/55 (10.9) 4/100 (4.0) 0.168
Anti-PM-Scl100 positivity,n (%) 6/56 (10.7) 8/102 (7.8) 0.567
Anti-PM-Scl75 positivity,n (%) 4/50 (8.0) 9/96 (9.4) 1.000
Anti-Ro-52 positivity,n (%) 53/74 (71.6) 78/139 (56.1) 0.038

图1

OM患者及非OM患者的Kaplan-Meier生存曲线分析"

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