Noninvasive positive pressure ventilator deteriorates the outcome of pneumomediastinum in anti-MDA5 antibody-positive clinically amyopathic dermatomyositis
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive clinically amyopathic dermatomyositis (CADM) with pneumomediastinum (PNM) is a life-threatening condition. We aim to determine the prognostic factors affecting survival of patients with anti-MDA5 Ab-positive CADM complicated with PNM.
We retrospectively established a cohort of patients with anti-MDA5 Ab-positive CADM complicated with PNM from April 2013 to July 2019. Demographic data and clinical characteristics from medical records were analyzed and variables were compared between survivors and nonsurvivors. We performed univariate and multivariate survival analyses by Cox regression. Survival curves were depicted by the Kaplan–Meier method.
Among 133 patients with anti-MDA5 Ab-positive CADM, 20 were diagnosed with PNM. The cumulative estimated Kaplan–Meier survival rate was 85% at 1 week, 55% at 1 month, and 40% at 1 year. Univariate analysis indicated several factors associated with survival. Worse liver function (AST, p = 0.043; LDH, p = 0.002; TBIL, p = 0.038), higher CRP level (p = 0.044), higher HRCT score (p = 0.022), and using noninvasive positive pressure ventilation (NPPV) (p < 0.01) were associated with poor prognosis. In a multivariate Cox regression model, AST level and using NPPV were indicated to be independent predictors of poor prognosis.
• Anti-MDA5 Ab-positive CADM complicated with PNM is a life-threatening condition with an incidence rate of 15.5%.
• The application of NPPV and worse liver function were independent risk factors for survival of anti-MDA5 Ab-positive CADM patients complicated with PNM.
KeywordsClinically amyopathic dermatomyositis MDA5 Pneumomediastinum Prognosis
We acknowledge the patients and clinicians involved in the project.
This work was supported by the National Natural Science Foundation of China (grants 81571575, 81771737).
Compliance with ethical standards
The study was approved by the Ethics Committee of Renji Hospital, Shanghai, China. All patients have given their informed consent prior to their inclusion in the study.
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