The following is a summary of the “Successful Rescue Therapy With Daratumumab in Rapidly Progressive Interstitial Lung Disease Caused by MDA5-Positive Dermatomyositis,” published in the January 2023 issue of Chest by Holzer, et al.

Rare systemic autoimmune disease known as dermatomyositis positive for melanoma differentiation-associated gene 5 (MDA5) is linked to rapidly progressive interstitial lung disease. A 19-year-old male presented with a life-threatening disease course due to rapidly progressive interstitial lung disease that resulted in respiratory failure despite intensive immunosuppression with multiple agents (steroids, IV immunoglobulins, tofacitinib, cyclophosphamide, mycophenolate mofetil, ciclosporin and rituximab). 

Anti-CD38 antibody daratumumab was used as a rescue therapy. After 4 injections of 1,800 mg weekly, significant pulmonary improvement was observed. Stable disease remission with marked pulmonary improvement, as well as persistent depletion of CD38+ plasma cells and MDA5-antibody titers, was observed after 6 months of follow-up. 

An effective treatment with daratumumab for dermatomyositis has never been reported before. Dermatomyositis, a severe antibody-mediated autoimmune disease, may benefit from CD38-targeted therapies.