Analyze of clinical characteristics of interstitial lung disease secondary to idiopathic inflammatory myositis with anti-melanoma differentiation-associated gene 5 antibody positive_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

WANG Pengfei ¹ , HAO Binwei ¹ , JIANG Xiaopeng ² ,  DUAN Wei ¹

1. Department of Respiratory and Critical Care Medicine, Shanxi Bethune Hospital (Shanxi Academy of Medical Sciences), Taiyuan, Shanxi 030032, P. R. China;
2. Department of Radiology, Shanxi Bethune Hospital (Shanxi Academy of Medical Sciences), Taiyuan, Shanxi 030032, P. R. China;

Corresponding author：

DUAN Wei, Email: xiaoduan19851105@sina.com

Keywords：

Interstitial lung disease; idiopathic inflammatory myositis; anti-melanoma differentiation associated gene 5; clinical characteristics; therapy

DOI：

10.7507/1671-6205.202206004

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Objective This study aims to analyze the clinical characteristics of idiopathic inflammatory myopathy (IIM) associated interstitial lung disease (ILD) with positive anti melanoma differentiation-associated gene 5 (MDA5) antibody. Methods The clinical data of 91 patients with IIM in Shanxi Bethune Hospital from Jan 2019 to Mar 2022 were collected. Then various comparisons and analyzed retrospectively in the clinical symptoms, laboratory indicators and imaging characteristics between the negative MDA5-IIM and the positive anti-MDA5-IIM. Results Compared with the negative anti-MDA5-IIM patients, the significantly higher incidences of Gottron sign (71.0% vs. 38.3%), ulcerative rash (45.2% vs.16.7%), dyspnea (64.5% vs. 36.7%), and ILD (71.0% vs. 43.3%), in the positive anti-MDA5-IIM patients (P<0.05). Compared with the negative anti-MDA5-IIM patients, the more ground-glass opacification (GGO) in the positive anti-MDA5-IIM patients on high resolution computed tomography (81.8% vs.53.8%, P<0.05). Compared with the positive anti-MDA5-IIM patients without ILD, the positive anti-MDA5-IIM patients with ILD had significantly higher incidence of Gottron sign (77.3% vs. 33.3%), the increased levels of lactate dehydrogenase [(525.20±203.09) IU/L vs. (321.73±188.88) IU/L], Krebs von den Lungen-6 [(1399.55±869.64) U/mL vs. (371.56±128.92) U/mL], and D-dimer [(1226.65±902.31) U/L vs. (703.73±160.11) U/L], as well as higher positive rate of anti-Ro-52 antibody (68.2% vs. 22.2%) and the higher case fatality rate (54.5% vs. 0.0%), with all P<0.05. The triple treatment of high-dose glucocorticoid (GC) combined with intravenous cyclophosphamide (IVCY) and another immunosuppressive agent (tacrolimus or tofacitinib) was found to be effective in the MDA5-IIM associated ILD group, with a lower case fatality rate (28.6%) than high-dose GC combined with IVCY group (47.1%). Conclusions The positive anti-MDA5-IIM with ILD of patients have the higher incidence of rash and dyspnea, with GGO as the most common imaging manifestation. When rapidly progressive interstitial lung disease (RPILD) is combined, the case fatality rate higher. Meanwhile, early intensive immunosuppressive therapy may improve the survival rate of RPILD patients with positive anti-MDA5 antibody.

Citation： WANG Pengfei, HAO Binwei, JIANG Xiaopeng, DUAN Wei. Analyze of clinical characteristics of interstitial lung disease secondary to idiopathic inflammatory myositis with anti-melanoma differentiation-associated gene 5 antibody positive. Chinese Journal of Respiratory and Critical Care Medicine, 2023, 22(4): 245-250. doi: 10.7507/1671-6205.202206004 Copy

1.	赵江峰, 叶霜. 抗mda5抗体相关皮肌炎的诊疗进展. 中华检验医学杂志, 2021, 44(11): 1070-1075.
2.	McPherson M, Economidou S, Liampas A, et al. Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: a systematic review. Semin Arthritis Rheum, 2022, 53: 151959.
3.	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. Ann Rheum Dis, 2017, 76(12): 1955-1964.
4.	Travis WD, Costabel U, Hansell IIM, et al. An official American Thoracic Society European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med, 2013, 188(6): 733-748.
5.	Xu Y, Yang CS, Li YJ, et al. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis. Clin Rheumatol, 2016, 35(1): 113-116.
6.	武雅楠, 王颖嫒, 祁福敏, 等. 抗黑色素瘤分化相关蛋白-5抗体阳性炎性肌病临床特征分析. 中华风湿病学杂志, 2022, 26(2): 105-114.
7.	马文兰, 白灵, 朱嘉睿, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎合并间质性肺病的发病机制研究进展. 中华风湿病学杂志, 2021, 25(12): 841-845.
8.	Kurtzman D, Vleugels RA. Anti-melanoma differentiation associated gene5 (MDA5) dermatomyositis aconcise review with an emphasis on distinctive clinical features. Am Acad Dermatol, 2018, 78 (4): 776-785.
9.	Allenbach Y, Uzunhan Y, Toquet S, et al. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: study of 121 cases. Neurology, 2020, 95(1): e70-e78.
10.	Gono T, Kawaguchi Y, Satoh T, et al. Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford), 2010, 49(9): 1713-1719.
11.	Moghadam-Kia S, Oddis CV, S ato S, et al. Anti-me1anoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res, 2016, 68(5): 689-694.
12.	Tsuji H, Nakashima R, Hosono Y, et al. Multicenter prospective study of the efficacy and safety of combined immunosuppressive therapy with high-dose glucocorticoid, tacrolimus, and cyclophosphamide in interstitial lung diseases accompanied by anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Arthritis Rheumatol, 2020, 72(3): 488-498.
13.	Chen ZW, Wang XD, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med, 2019, 381(3): 291-293.
14.	康宁, 陈鸿志, 周永慧, 等. 联用托法替布成功治疗MDA5阳性无肌炎型皮肌炎相关间质性肺病一例. 中国呼吸与危重监护杂志, 2021, 20(6): 440-443.
15.	Ge YP, Li SS, Tian XL, et al. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy. Clin Rheumatol, 2021, 40(6): 2311–2317.
16.	Shirakashi M, Nakashima R, Tsuji H, et al. Efﬁcacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment. Rheumatology, 2020, 59(11): 3284–3292.
17.	鞠伟威, 王培绒, 杨国欢, 等. 抗MDA-5抗体阳性炎性肌病合并间质性肺病的临床特点和预后分析. 国际呼吸杂志, 2022, 42(4): 267-274.
18.	Lian XY, Zou J, Guo Q, et al. Mortality risk prediction in amyopathic dermatomyositis associated with interstitial lung disease: the FLAIR model. Chest, 2020, 158(4): 1535-1545.
19.	Habe K, Wada H, Higashiyama A, et al. Elevated plasma D-dimer levels in dermatomyositis patients with cutaneous manifestations. Sci Rep, 2019, 9(1): 1410.
20.	徐婷, 张光峰, 林浩博, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎合并快速进展性间质性肺病的临床及预后分析. 中华风湿病学杂志, 2021, 25(6): 361-367.
21.	Sabbagh S, Pinal-Fernandez I, Kishi T, et al. Anti-Ro52 auto-antibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis. Ann Rheum Dis, 2019, 78(7): 988-995.
22.	Temmoku J, Sato S, Fujita Y, et al. Clinical significance of myositis-specific auto antibody profiles in Japanese patients with polymyositis/dermatomyositis. Medicine, 2019, 98(20): e15578.
23.	Ye Y, Fu Q, Wang R, et al. Serum KL-6 level is a prognostic marker in patients with anti-MDA5 antibody-positive dermatomyositis associated with interstitial lung disease. J Clin Lab Anal, 2019, 33(8): e22978.
24.	Li YH, Li YM, Wu J, et al. Predictors of poor outcome of anti-MDA5-associated rapidly progressive interstitial lung disease in a Chinese cohort with dermatomyositis. J Immunol Res, 2020, 2020: 2024869.
25.	陈望, 高万勤, 史晓飞, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎的临床和胸部CT表现. 中华放射学杂志, 2021, 55(2): 143-147.
26.	Egashira R. High-resolution CT findings of myositis-related interstitial lung disease. Medicina (Kaunas), 2021, 57(7): 692.

1. 赵江峰, 叶霜. 抗mda5抗体相关皮肌炎的诊疗进展. 中华检验医学杂志, 2021, 44(11): 1070-1075.
2. McPherson M, Economidou S, Liampas A, et al. Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: a systematic review. Semin Arthritis Rheum, 2022, 53: 151959.
3. 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. Ann Rheum Dis, 2017, 76(12): 1955-1964.
4. Travis WD, Costabel U, Hansell IIM, et al. An official American Thoracic Society European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med, 2013, 188(6): 733-748.
5. Xu Y, Yang CS, Li YJ, et al. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis. Clin Rheumatol, 2016, 35(1): 113-116.
6. 武雅楠, 王颖嫒, 祁福敏, 等. 抗黑色素瘤分化相关蛋白-5抗体阳性炎性肌病临床特征分析. 中华风湿病学杂志, 2022, 26(2): 105-114.
7. 马文兰, 白灵, 朱嘉睿, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎合并间质性肺病的发病机制研究进展. 中华风湿病学杂志, 2021, 25(12): 841-845.
8. Kurtzman D, Vleugels RA. Anti-melanoma differentiation associated gene5 (MDA5) dermatomyositis aconcise review with an emphasis on distinctive clinical features. Am Acad Dermatol, 2018, 78 (4): 776-785.
9. Allenbach Y, Uzunhan Y, Toquet S, et al. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: study of 121 cases. Neurology, 2020, 95(1): e70-e78.
10. Gono T, Kawaguchi Y, Satoh T, et al. Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis. Rheumatology (Oxford), 2010, 49(9): 1713-1719.
11. Moghadam-Kia S, Oddis CV, S ato S, et al. Anti-me1anoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res, 2016, 68(5): 689-694.
12. Tsuji H, Nakashima R, Hosono Y, et al. Multicenter prospective study of the efficacy and safety of combined immunosuppressive therapy with high-dose glucocorticoid, tacrolimus, and cyclophosphamide in interstitial lung diseases accompanied by anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Arthritis Rheumatol, 2020, 72(3): 488-498.
13. Chen ZW, Wang XD, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med, 2019, 381(3): 291-293.
14. 康宁, 陈鸿志, 周永慧, 等. 联用托法替布成功治疗MDA5阳性无肌炎型皮肌炎相关间质性肺病一例. 中国呼吸与危重监护杂志, 2021, 20(6): 440-443.
15. Ge YP, Li SS, Tian XL, et al. Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy. Clin Rheumatol, 2021, 40(6): 2311–2317.
16. Shirakashi M, Nakashima R, Tsuji H, et al. Efﬁcacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment. Rheumatology, 2020, 59(11): 3284–3292.
17. 鞠伟威, 王培绒, 杨国欢, 等. 抗MDA-5抗体阳性炎性肌病合并间质性肺病的临床特点和预后分析. 国际呼吸杂志, 2022, 42(4): 267-274.
18. Lian XY, Zou J, Guo Q, et al. Mortality risk prediction in amyopathic dermatomyositis associated with interstitial lung disease: the FLAIR model. Chest, 2020, 158(4): 1535-1545.
19. Habe K, Wada H, Higashiyama A, et al. Elevated plasma D-dimer levels in dermatomyositis patients with cutaneous manifestations. Sci Rep, 2019, 9(1): 1410.
20. 徐婷, 张光峰, 林浩博, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎合并快速进展性间质性肺病的临床及预后分析. 中华风湿病学杂志, 2021, 25(6): 361-367.
21. Sabbagh S, Pinal-Fernandez I, Kishi T, et al. Anti-Ro52 auto-antibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis. Ann Rheum Dis, 2019, 78(7): 988-995.
22. Temmoku J, Sato S, Fujita Y, et al. Clinical significance of myositis-specific auto antibody profiles in Japanese patients with polymyositis/dermatomyositis. Medicine, 2019, 98(20): e15578.
23. Ye Y, Fu Q, Wang R, et al. Serum KL-6 level is a prognostic marker in patients with anti-MDA5 antibody-positive dermatomyositis associated with interstitial lung disease. J Clin Lab Anal, 2019, 33(8): e22978.
24. Li YH, Li YM, Wu J, et al. Predictors of poor outcome of anti-MDA5-associated rapidly progressive interstitial lung disease in a Chinese cohort with dermatomyositis. J Immunol Res, 2020, 2020: 2024869.
25. 陈望, 高万勤, 史晓飞, 等. 抗黑色素瘤分化相关基因5抗体阳性皮肌炎的临床和胸部CT表现. 中华放射学杂志, 2021, 55(2): 143-147.
26. Egashira R. High-resolution CT findings of myositis-related interstitial lung disease. Medicina (Kaunas), 2021, 57(7): 692.

Chinese Journal of Respiratory and Critical Care Medicine

Analyze of clinical characteristics of interstitial lung disease secondary to idiopathic inflammatory myositis with anti-melanoma differentiation-associated gene 5 antibody positive

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