• Marie-Therese Holzer, Jasper F Nies, Tim Oqueka, Tobias B Huber, Ina Kötter, and Martin Krusche.
• III Department of Medicine for Nephrology, Rheumatology and Endocrinology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
• Chest. 2023 Jan 1; 163 (1): e1e5e1-e5.

AbstractMelanoma differentiation-associated gene 5 (MDA5) positive dermatomyositis is a rare systemic autoimmune disease that is associated with life-threatening rapidly progressive interstitial lung disease. We report the case of a 19-year-old male patient with a life-threatening disease course caused by rapidly progressive interstitial lung disease that caused respiratory failure despite intensive immunosuppression with multiple agents (steroids, IV immunoglobulins, tofacitinib, cyclophosphamide, mycophenolate mofetil, ciclosporin and rituximab). Rescue therapy with daratumumab, an anti-CD38-antibody, was initiated. Significant pulmonary improvement was noticed after 4 weekly injections of 1,800 mg. After 6 months of follow up, stable disease remission with significant pulmonary improvement and persistent depletion of CD38+ plasma cells and MDA5-antibody titers were seen. This is the first report of the successful use of daratumumab in dermatomyositis. It highlights the potential of CD38 targeted therapies for severe antibody-mediated autoimmune diseases such as dermatomyositis.Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

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