Journal
CHEST
Volume 163, Issue 1, Pages E1-E5Publisher
ELSEVIER
DOI: 10.1016/j.chest.2022.08.2209
Keywords
KEY WORDS; anti-CD38; daratumumab; dermatomyositis; interstitial lung disease; MDA5
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This is a case report of a 19-year-old male patient with rapidly progressive interstitial lung disease that did not respond to intensive immunosuppression. Rescue therapy with daratumumab, an anti-CD38-antibody, resulted in significant pulmonary improvement and stable disease remission after 6 months of follow up.
Melanoma 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 thorn 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. CHEST 2023; 163(1):e1-e5
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