Editorial: Anti-MDA5-Positive Dermatomyositis

Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5-positive DM) is a unique clinical phenotype of DM characterized by less involvement of the skeletal muscles, vasculopathy-related cutaneous manifestations such as skin ulcers and palmar papules, and a high incidence, fatal complication with rapidly progressive interstitial lung disease (RP-ILD) (1,2). The discovery of anti-MDA5 antibody and the development of a convenient measurement procedure for this autoantibody have led physicians to manage anti-MDA5-positive DM patients earlier and more readily before devastating RP-ILD develops (2,3). Researchers across the world have been encouraged to determine new aspects regarding the etiology, pathogenesis, pathophysiology, variability of clinical features, independent prognostic factors, and therapeutic strategies of this disease (4-9). However, sufficient research has not yet been carried out to achieve satisfactory outcomes for anti-MDA5-positive DM patients. There are still urgent demands and unmet needs for more efficacious and more ). This model was composed of three items, fever, ferritin ≥1,250 μg/L, and positive carcinoembryonic antigen, and classified three risk groups, a high-risk group (all-cause mortality rate, 78%), a moderate-risk group (all-cause mortality rate, 43%), and a low-risk group (all-cause mortality rate, 25%).The predictive modalities mentioned above should be validated in another cohort to confirm the consistent usefulness of the predictive risk models for all anti-MDA5positive DM patients worldwide or to adjust those models depending on race.We should also consider numerous complications, such as infections, Treatments for anti-MDA5-positive DM are largely empirical and have not been fully addressed in this topic collection. Nevertheless, a real-world JAMI cohort study just came out (12). Unfortunately, the data failed to demonstrate the survival benefits of classic initial triple-combination therapy, i.e., high-dose glucocorticoids, cyclophosphamide, and tacrolimus, in different anti-MDA5-positive DM clusters. The effect of antifibrotics on anti-MDA5-positive DM related RP-ILD has not been fully illustrated, only presented in a case report (13) and a retrospective study with a small sample size (14), suggesting that the addition of antifibrotic may play improve the survival in these patients.Regarding future perspectives, we need to learn more appropriate management as well as clarify the pathophysiology related to treatment targets for anti-MDA5positive DM patients through numerous, remarkable further advances. We believe that we can develop novel therapeutic strategies and implement personalized medicine based on the risk of mortality using several uniform predictive models across the world, considering the prevention of various life-threatening complications, to improve the quality of life as well as the prognosis of anti-MDA5-positive DM patients.