Following the discovery of anti-MDA5 Ab, the clinical understanding of dermatomyositis has been improved

Abstract

Knowledge about clinically amyopathic dermatomyositis has exploded in the last two decades, especially after the discovery of anti-MDA5 Ab. The disease's likely genesis and pathophysiological processes have been studied in a number of scientific and epidemiological studies. Thanks to a slew of new large-scale cohort studies, the clinical characteristics of MDA5+ DM may be better identified. In addition, several prognostic indicators have been discovered, such as respiratory physiology measures and serum biomarkers, which have helped to enhance risk categorization in clinical practice and restrict the design of future clinical studies. Quantitative evaluation of pulmonary HRCT data may be the trend of imaging testing in MDA5+ DM-ILD. The current standard of treatment for MDA5+ DM-ILD is "triple treatment" with JAK inhibitors, which has been proven in recent trials to significantly improve the survival of early-stage patients. Total mortality in this incurable disease, however, is still shockingly high, especially in advanced-stage patients. There is still a pressing need for a more effective and well-evidenced treatment approach for MDA5+ DM-ILD.