北京大学学报(医学版) ›› 2021, Vol. 53 ›› Issue (5): 1012-1016. doi: 10.19723/j.issn.1671-167X.2021.05.034

• 短篇论著 • 上一篇    

托法替布联合治疗抗MDA5抗体阳性的无肌病皮肌炎并发快速进展型间质性肺病5例临床分析

吴燕芳,高飞,林滇恬,陈志涵(),林禾   

  1. 福建医科大学省立临床医学院,福建省立医院风湿免疫科,福州 350001
  • 收稿日期:2020-07-22 出版日期:2021-10-18 发布日期:2021-10-11
  • 通讯作者: 陈志涵 E-mail:han213@163.com
  • 基金资助:
    福建省自然科学基金(2017J01170);福建省自然科学基金(2019J01184)

  • Received:2020-07-22 Online:2021-10-18 Published:2021-10-11
  • Supported by:
    Natural Science Foundation of Fujian Province(2017J01170);Natural Science Foundation of Fujian Province(2019J01184)

RICH HTML

  

关键词: 皮肌炎, 肺疾病, 间质性, 托法替布, 抗MDA5抗体

中图分类号: 

  • R593.26

图1

病例1典型皮疹"

表1

TOF治疗前患者的观察指标"

Items Case 1 Case 2 Case 3 Case 4 Case 5
Gender Female Female Female Female Male
Age/years 44 47 37 36 56
Clinical characteristics
Rash +++ ++ +++ ++ +++
Muscle weakness + - + - +
Arthritis - - + - +
Fever + - + - +
Course of dyspnea/weeks 2 4 4 2 2
Course of RP-ILD/weeks 2 4 4 2 2
Classification of ILD NSIP+DAD NSIP+UIP NSIP+OP NSIP NSIP+DAD
Quantitative score of HRCT 22 13 19 10 25
Laboratory results
Lymphocyte/(×109/L) 0.8 0.7 0.8 1.4 0.8
CK/(IU/L) 52 19 42 84 195
LDH/(IU/L) 668 217 218 360 444
AST/(IU/L) 61 42 33 54 57
Anti-MDA5 antibody + + + + +
Anti-Ro-52 antibody + + + - +
EMG Normal Normal Not done Normal Not done
BALF examination
Cytological examination Neu, 6%;
Lym, 81%;
Mac, 12%
Neu, 8%;
Lym, 31%;
Mac, 42%
Not done Neu, 5%;
Lym, 21%;
Mac, 74%
Neu, 22%;
Lym, 10%;
Mac, 68%
mNGS Pneumocystis jiroveci 603 - - - -
Pre-hospital medication MP+CTX +CSA MP+CTX MP+CTX None None

图2

病例1肺部HRCT"

表2

存活患者治疗前后各观察指标结果(n=4)"

Items Baseline After treatment
1 month 3 months 6 months
Quantitative score of HRCT 16.00±2.74 12.50±1.66 7.75±1.93 6.75±3.20
Oxygenation index 257.30±48.71 344.30±31.93 442.50±14.36 448.00±13.86
6-minute walk test/m 150.00±88.98 290.00±42.03 390.00±23.80 435.00±22.40
Pulmonary function index
FVC% 54.50%±4.56% 74.41%±1.16% 74.90%±1.76%
TLC% 52.88%±4.04% 76.45%±4.24% 80.10%±6.67%
DLCO% 52.58%±2.20% 64.33%±1.73% 66.53%±2.67%
ESR/(mm/h) 42.50±19.65 23.50±4.84 11.25±1.49 10.25±2.50
CRP/(mg/L) 9.70±5.52 1.50 (1.32-3.44) 1.00±0.24 1.24±0.69
Serum ferritin/(μg/L) 957.30±168.20 407.50±86.83 153.30±62.65 72.50±32.35
[1] Nakashima R, Hosono Y, Mimori T. Clinical significance and new detection system of autoantibodies in myositis with interstitial lung disease [J]. Lupus, 2016, 25(8):925-933.
doi: 10.1177/0961203316651748 pmid: 27252271
[2] 陈芳, 王冬雪, 舒晓明, 等. 血清抗黑色素瘤分化相关基因抗体检测在多发性肌炎/皮肌炎患者中的意义 [J]. 中华风湿病学杂志, 2012, 16(1):13-18.
[3] Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyo-sitis-associated interstitial lung disease [J]. N Engl J Med, 2019, 381(3):291-293.
doi: 10.1056/NEJMc1900045
[4] Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups [J]. Ann Rheum Dis, 2017, 76(12):1955-1964.
doi: 10.1136/annrheumdis-2017-211468 pmid: 29079590
[5] Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias [J]. Am J Respir Crit Care Med, 2013, 188(6):733-748.
doi: 10.1164/rccm.201308-1483ST
[6] Goldin JG, Lynch DA, Strollo DC, et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease [J]. Chest, 2008, 134(2):358-367.
doi: 10.1378/chest.07-2444
[7] 卢昕, 王国春. 重视新型肌炎特异性自身抗体的临床应用 [J]. 中华风湿病学杂志, 2015, 19(3):145-147.
[8] Cao H, Pan M, Kang Y, et al. Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti-melanoma differentiation-associated gene 5 antibody [J]. Arthritis Care Res (Hoboken), 2012, 64(10):1602-1610.
doi: 10.1002/acr.21728
[9] Ye S, Chen XX, Lu XY, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: A retrospective cohort study [J]. Clin Rheumatol, 2007, 26(10):1647-1654.
doi: 10.1007/s10067-007-0562-9
[10] Kurasawa K, Arai S, Namiki Y, et al. Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis [J]. Rheumatology (Oxford), 2018, 57(12):2114-2119.
doi: 10.1093/rheumatology/key188
[11] 许文英, 梅焕平. 生物制剂在多肌炎皮肌炎并发间质性肺病的治疗进展 [J]. 中华风湿病学杂志, 2016, 20(7):489-492.
[12] 黄慧, 邵池, 徐作军. 美国胸科协会官方指南: 支气管肺泡灌洗液的细胞学分析在间质性肺疾病中的临床应用(摘译本) [J]. 中华结核和呼吸杂志, 2012, 35(9):650-654.
[13] Kono M, Miyashita K, Hirama R, et al. Prognostic significance of bronchoalveolar lavage cellular analysis in patients with acute exa-cerbation of interstitial lung disease [J]. Respir Med, 2021, 186:106534.
doi: 10.1016/j.rmed.2021.106534
[14] Salonen J, Lampela H, Keskitalo E, et al. Bronchoalveolar lavage differential cell count on prognostic assessment of patients with stable or acute interstitial lung disease: A retrospective real-life study [J]. Clin Immunol, 2020, 220:108594.
doi: 10.1016/j.clim.2020.108594
[15] Xu Y, Yang CS, Li YJ, et al. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis [J]. Clin Rheumatol, 2016, 35(1):113-116.
doi: 10.1007/s10067-015-3139-z pmid: 26660480
[1] 柯涵炜, 王起, 许克新. 优化环磷酰胺剂量在间质性膀胱炎/膀胱疼痛综合征啮齿动物模型中的应用[J]. 北京大学学报(医学版), 2024, 56(5): 908-912.
[2] 周庆欣,杨晴晴,石舒原,李沛,孙凤. 健康体检人群血尿酸与气流阻塞的相关性[J]. 北京大学学报(医学版), 2024, 56(4): 693-699.
[3] 何珊,陈炘,程琦,朱灵江,张培玉,童淑婷,薛静,杜燕. 托法替布通过JAK/STAT3通路抑制肺成纤维细胞向肌成纤维细胞转化[J]. 北京大学学报(医学版), 2024, 56(3): 505-511.
[4] 赖展鸿,李嘉辰,贠泽霖,张永刚,张昊,邢晓燕,邵苗,金月波,王乃迪,李依敏,李玉慧,栗占国. 特发性炎性肌病完全临床应答相关因素的单中心真实世界研究[J]. 北京大学学报(医学版), 2024, 56(2): 284-292.
[5] 邹雪,白小娟,张丽卿. 艾拉莫德联合托法替布治疗难治性中重度类风湿关节炎的疗效[J]. 北京大学学报(医学版), 2023, 55(6): 1013-1021.
[6] 李文根,古晓东,翁锐强,刘苏东,陈超. 血浆外泌体miR-34-5p和miR-142-3p在系统性硬化症中的表达及临床意义[J]. 北京大学学报(医学版), 2023, 55(6): 1022-1027.
[7] 王子恺,莫佳丽,张蒙,廖纪萍. 2013—2020年北京市慢性阻塞性肺疾病急性加重女性住院患者的流行病学和住院费用分析[J]. 北京大学学报(医学版), 2023, 55(6): 1074-1081.
[8] 辛鹏,张昊,姜振明. 膀胱内灌注电灼联合水扩张法治疗女性间质性膀胱炎[J]. 北京大学学报(医学版), 2023, 55(5): 865-870.
[9] 哈雪梅,姚永正,孙莉华,辛春杨,熊焰. 实性肺胎盘样变形1例及文献复习[J]. 北京大学学报(医学版), 2023, 55(2): 357-361.
[10] 邢晓燕,张筠肖,朱冯赟智,王一帆,周新尧,李玉慧. 皮肌炎合并巨噬细胞活化综合征5例[J]. 北京大学学报(医学版), 2022, 54(6): 1214-1218.
[11] 朱琳,张维宇,许克新. 环磷酰胺诱导SD大鼠膀胱疼痛综合征模型的有效性[J]. 北京大学学报(医学版), 2022, 54(4): 735-740.
[12] 张朴丽,杨红霞,张立宁,葛勇鹏,彭清林,王国春,卢昕. 血清YKL-40在诊断抗黑色素瘤分化相关基因5阳性皮肌炎合并严重肺损伤中的价值[J]. 北京大学学报(医学版), 2021, 53(6): 1055-1060.
[13] 王飞,朱翔,贺蓓,朱红,沈宁. 自发缓解的滤泡性细支气管炎伴非特异性间质性肺炎1例报道并文献复习[J]. 北京大学学报(医学版), 2021, 53(6): 1196-1200.
[14] 王佳文,刘敬超,孟令峰,张威,刘晓东,张耀光. 间质性膀胱炎/膀胱疼痛综合征患者生活质量及相关因素分析[J]. 北京大学学报(医学版), 2021, 53(4): 653-658.
[15] 夏芳芳,鲁芙爱,吕慧敏,杨国安,刘媛. 系统性红斑狼疮伴间质性肺炎的临床特点及相关因素分析[J]. 北京大学学报(医学版), 2021, 53(2): 266-272.
Viewed
Full text


Abstract

Cited

  Shared   
  Discussed   
No Suggested Reading articles found!