Chest
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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. ⋯ 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.
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A 33-year-old man with a medical history of childhood exercise-induced asthma presented to the ED with 2 days of hemoptysis. He described the hemoptysis as bright red blood with clots, approximately 100 cm3 in total. He denied prior hemoptysis, pulmonary infections, or exposure to TB. Years ago he had an episode of gross hematuria, but a urologic workup was unrevealing.