The aim of this study is to evaluate the factors associated to 1-year mortality in clinically amyopathic dermatomyositis (CADM) patients with acute interstitial lung disease (ILD). A single center of 37 cases of Chinese patients with CADM was reviewed retrospectively in Renji hospital. All CADM patients were diagnosed with ILD; there were 24 cases of acute interstitial pneumonia (AIP) and 13 cases of acute exacerbation of non-acute interstitial pneumonia non-AIP. The clinical features, including blood tests, chest high-resolution computed tomography (HRCT) score, and lung function, were analyzed, respectively. Neutrophil lymphocyte ratio (NLR), serum ferritin level, serum lactate dehydrogenase (LDH) level, and HRCT score were statistically significant factors on univariate analysis. Multivariate analysis revealed that the overall HRCT score (HR 1.134, 95 % confidence interval 1.009–1.275, P = 0.017) and serum ferritin level (HR 1.001, 95 % confidence interval 1.002–1.007, P = 0.010) were independently significant factors of 1-year mortality. C statistic value of HRCT score (c statistic value 0.867, P < 0.0001) and serum ferritin level (c statistic value 0.808, P = 0.002) were statistically significant in the classification of non-survivors. Patients with calcineurin inhibitor presented a better outcome than those without calcineurin inhibitor (log-rank test, P = 0.006). HRCT score and serum ferritin level are factors associated to the 1-year mortality of acute ILD in CADM patients. Calcineurin inhibitor might improve the outcome of CADM patients with acute ILD.
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We are grateful to Masataka Kuwana (Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Japan) for excellent technical assistance in measuring anti-MDA5 antibodies. We would like to thank Dr. Jiachang Chi and Dr. Huawei Wu (Department of Radiology, Ren Ji hospital, School of Medicine, Shanghai Jiao Tong University) for reviewing the chest HRCT image.
Conflict of interest
The authors declare they have no conflicts of interest.
All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. BCD and ZJ were involved with conception and design; ZJ, CJC, and WHW acquisition of the data; and BCD, ZJ, and GQ analysis and interpretation of the data.
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