Clinical features and prognosis of anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease: a retrospective cohort study_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

WANG Ting , PAN Jianguang ,  ZHANG Hongying , WENG Ling , HAN Ximei , ZHENG Rongshan

Department of Pulmonary and Critical Care Medicine, Fuzhou Pulmonary Hospital of Fujian Province, Teaching Hospital of Fujian Medical University, Fuzhou, Fujian 35000, P. R. China;

Corresponding?author：

ZHANG Hongying, Email: 290923206@qq.com

Keywords：

Anti-MDA5 antibody; Dermatomyositis; Interstitial lung disease; Prognostic factors

DOI：

10.7507/1671-6205.202510046

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Objectivev To investigate the clinical characteristics of patients with anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease (MDA5⁺ DM-ILD), explore prognostic risk factors, and evaluate the predictive value of relevant indicators for mortality, thereby providing a basis for the early identification of high-risk patients and precise intervention. Methods The clinical data and relevant laboratory test results within 24 hours of admission of 33 patients with MDA5⁺ DM-ILD diagnosed in Fuzhou Pulmonary Hospital of Fujian Province from January 2021 to December 2024 were retrospectively analyzed. According to medical records and telephone follow-up results, the patients were divided into a survival group (n=19) and a death group (n=14). Univariate analysis and multivariate COX regression analysis were conducted to identify the risk factors in the death group. The statistically significant risk factors from the multivariate analysis were used to draw receiver operating characteristic (ROC) curves. Results The median follow-up time of the 33 patients was 18.5 months (interquartile range: 10.2-26.8 months). Univariate analysis showed that the neutrophil count, serum ferritin (SF), erythrocyte sedimentation rate (ESR), carcinoembryonic antigen (CEA), pro-gastrin-releasing peptide (ProGRP), cytokeratin fragment antigen 21-1 (Cyfra21-1), neuron-specific enolase (NSE), lactate dehydrogenase (LDH), cancer antigen 153 (CA153), chest CT lesion extent score, and ground-glass opacity (GGO) score for each lung lobe were significantly higher in the death group than those in the survival group. Conversely, the oxygenation index (PaO₂/FiO₂) was significantly lower in the death group. These differences were statistically significant (P<0.05). Multivariate Cox regression analysis identified SF (OR=1.001, 95%CI 1.000-1.002, P<0.05) and Cyfra21-1 (OR=1.100, 95%CI 1.037-1.166, P<0.05) as independent risk factors for death. ROC curve analysis determined the optimal cutoff values as 1688 ng/mL for SF (AUC=0.778, sensitivity=57.1%, specificity=94.7%) and 5.63 ng/mL for Cyfra21-1 (AUC=0.853, sensitivity=100%, specificity=73.7%). Conclusion SF and CYFRA21-1 serve as independent predictors of mortality in patients with MDA5⁺ DM-ILD. These biomarkers enable early identification of high-risk subgroups, and guide targeted therapeutic interventions to improve prognoses when integrated with clinical and computed tomography characteristics.

Citation： WANG Ting, PAN Jianguang, ZHANG Hongying, WENG Ling, HAN Ximei, ZHENG Rongshan. Clinical features and prognosis of anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease: a retrospective cohort study. Chinese Journal of Respiratory and Critical Care Medicine, 2026, 25(7): 494-501. doi: 10.7507/1671-6205.202510046 Copy

1.	Motegi SI, Sekiguchi A, Toki S, et al. Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease. Eur J Dermatol, 2019, 29(5): 511-517.
2.	Xie HY, Zhang DD,Wang YZ, et al. Risk factors for mortality in patients with anti-MDA5 antibody-positive dermatomyositis: A meta-analysis and systematic review. Semin Arthritis Rheum, 2023, 62: 152231.
3.	Bohan A, Peter JB. Polymyositis and Dermatomyositis (First of Two Parts). N Engl J Med, 1975, 292(7): 344-347. DOI:10. 1056/NEJM197502132920706.
4.	Mammen AL, Allenbach Y, Stenzenl W, et al. 239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14-16December2018. Neuromuscul Diord, 2020, 30(1): 70-92.
5.	Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin, 2002, 20: 387-408.
6.	Casarini M, Ameglio F, Alemanno L, et al. Cytokine levels correlate with a radiologic score in active pulmonary tuberculosis. Am J Respir Crit Care Med, 1999, 159(1): 143-148.
7.	Kazerooni EA, Martinez FJ, Flint A, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: correlation with pathologic scoring. AJR Am J Roentgenol, 1997, 169: 977-983.
8.	呂成銀, 王佳佳, 王磊, 等. 抗黑色素瘤分化相關基因5抗體陽性皮肌炎患者272例臨床特征分析. 中華風濕病學雜志, 2024, 28(1): 31-36.
9.	Kotla NK, Dutta P, Parimi S, et al. The Role of Ferritin in Health and Disease: Recent Advances and Understandings. Metabolites, 2022;12(7).
10.	DePalma RG, Hayes VW, O’Leary T J. Optimal serum ferritin level range: iron status measure and inflammatory biomarker. Metallomics, 2021, 13(6): mfab030.
11.	Sioutas A, Vainikka LK, Kentson M, et al. Oxidant-induced autophagy and ferritin degradation contribute to epithelial-mesenchymal transition through lysosomal iron. J Inflamm Res, 2017, 10: 29-39.
12.	Zhang J, Zheng YP, Wang Y, et al. YAP1 alleviates sepsis-induced acute lung injury via inhibiting ferritinophagy-mediated ferroptosis. Front Immunol, 2022, 13: 884362.
13.	Liu XJ, Pan BH, Wang XT, et al. Ischemia/reperfusion-activated ferroptosis in the early stage triggers excessive inflammation to aggregate lung injury in rats. Front Med (Lausanne), 2023, 10: 1181286.
14.	Xu L, Bian W, Gu XH, et al. Differing expression of cytokines andtumor markers in combined pulmonary fibrosis and emphysema compared to emphysema and pulmonary fibrosis. Copd, 2017, 14(2): 245-250.
15.	Molyneaux PL, Fahy WA, Byrne AJ, et al. CYFRA 21-1 predicts progression in idiopathic pulmonary fibrosis: A prospective longitudinal analysis of the PROFILE cohort. Am J Respir Crit Care Med, 2022, 205(12): 1440-1448.
16.	Gui XH, Ma M, Ding JJ, et al. Cytokeratin 19 fragment is associaled with severity and poor prognosis of interstitial lung disease in anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford), 2021, 60(8): 3913-3922.
17.	He SJ, Zhou YH, Fan CY, et al. Differences in sex- and age-associated mortality in patients with anti-MDA5 positive dermatomyositis. Mod Rheumatol, 2023, 33(5): 975-981.
18.	Yamaguchi K, Yamaguchi A, Onuki Y, et al. Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age. Mod Rheumatol, 2021, 31(1): 177-185.
19.	Youhei F, Takuya K, Kentaro I, et al. Evaluation of clinical prognostic factors for interstitial pneumonia in anti-MDA5 antibody-positive dermatomyositis patients. Mod Rheumatol, 2018, 28(1): 133-140.
20.	錢運紅, 韓鵬, 呂夢宇, 等. 抗MDA-5抗體相關皮肌炎合并間質性肺病的HRCT表現. 中國CT和MRI雜志, 2025, 23(5): 60-63.

1. Motegi SI, Sekiguchi A, Toki S, et al. Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease. Eur J Dermatol, 2019, 29(5): 511-517.
2. Xie HY, Zhang DD,Wang YZ, et al. Risk factors for mortality in patients with anti-MDA5 antibody-positive dermatomyositis: A meta-analysis and systematic review. Semin Arthritis Rheum, 2023, 62: 152231.
3. Bohan A, Peter JB. Polymyositis and Dermatomyositis (First of Two Parts). N Engl J Med, 1975, 292(7): 344-347. DOI:10. 1056/NEJM197502132920706.
4. Mammen AL, Allenbach Y, Stenzenl W, et al. 239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14-16December2018. Neuromuscul Diord, 2020, 30(1): 70-92.
5. Sontheimer RD. Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin, 2002, 20: 387-408.
6. Casarini M, Ameglio F, Alemanno L, et al. Cytokine levels correlate with a radiologic score in active pulmonary tuberculosis. Am J Respir Crit Care Med, 1999, 159(1): 143-148.
7. Kazerooni EA, Martinez FJ, Flint A, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: correlation with pathologic scoring. AJR Am J Roentgenol, 1997, 169: 977-983.
8. 呂成銀, 王佳佳, 王磊, 等. 抗黑色素瘤分化相關基因5抗體陽性皮肌炎患者272例臨床特征分析. 中華風濕病學雜志, 2024, 28(1): 31-36.
9. Kotla NK, Dutta P, Parimi S, et al. The Role of Ferritin in Health and Disease: Recent Advances and Understandings. Metabolites, 2022;12(7).
10. DePalma RG, Hayes VW, O’Leary T J. Optimal serum ferritin level range: iron status measure and inflammatory biomarker. Metallomics, 2021, 13(6): mfab030.
11. Sioutas A, Vainikka LK, Kentson M, et al. Oxidant-induced autophagy and ferritin degradation contribute to epithelial-mesenchymal transition through lysosomal iron. J Inflamm Res, 2017, 10: 29-39.
12. Zhang J, Zheng YP, Wang Y, et al. YAP1 alleviates sepsis-induced acute lung injury via inhibiting ferritinophagy-mediated ferroptosis. Front Immunol, 2022, 13: 884362.
13. Liu XJ, Pan BH, Wang XT, et al. Ischemia/reperfusion-activated ferroptosis in the early stage triggers excessive inflammation to aggregate lung injury in rats. Front Med (Lausanne), 2023, 10: 1181286.
14. Xu L, Bian W, Gu XH, et al. Differing expression of cytokines andtumor markers in combined pulmonary fibrosis and emphysema compared to emphysema and pulmonary fibrosis. Copd, 2017, 14(2): 245-250.
15. Molyneaux PL, Fahy WA, Byrne AJ, et al. CYFRA 21-1 predicts progression in idiopathic pulmonary fibrosis: A prospective longitudinal analysis of the PROFILE cohort. Am J Respir Crit Care Med, 2022, 205(12): 1440-1448.
16. Gui XH, Ma M, Ding JJ, et al. Cytokeratin 19 fragment is associaled with severity and poor prognosis of interstitial lung disease in anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford), 2021, 60(8): 3913-3922.
17. He SJ, Zhou YH, Fan CY, et al. Differences in sex- and age-associated mortality in patients with anti-MDA5 positive dermatomyositis. Mod Rheumatol, 2023, 33(5): 975-981.
18. Yamaguchi K, Yamaguchi A, Onuki Y, et al. Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age. Mod Rheumatol, 2021, 31(1): 177-185.
19. Youhei F, Takuya K, Kentaro I, et al. Evaluation of clinical prognostic factors for interstitial pneumonia in anti-MDA5 antibody-positive dermatomyositis patients. Mod Rheumatol, 2018, 28(1): 133-140.
20. 錢運紅, 韓鵬, 呂夢宇, 等. 抗MDA-5抗體相關皮肌炎合并間質性肺病的HRCT表現. 中國CT和MRI雜志, 2025, 23(5): 60-63.

Chinese Journal of Respiratory and Critical Care Medicine

Clinical features and prognosis of anti-MDA5 antibody-positive dermatomyositis with interstitial lung disease: a retrospective cohort study

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