The clinical course of IIMs-ILD ranges from chronic forms with slowly progressive symptoms to acute and rapidly progressive forms leading to respiratory failure—typical of patients with clinically amyopathic dermatomyositis (CADM) and anti-MDA5 antibodies, occasionally associated with the development of pneumomediastinum (Fig. 3) [53, 54]. Here, IFIH1 is linked to interstitial lung disease.