Successful Rescue Therapy With Daratumumab in Rapidly Progressive Interstitial Lung Disease Caused by MDA5-Positive Dermatomyositis

被引：31

作者：

Holzer, Marie-Therese ^{[1
]}

Nies, Jasper F. ^{[1
]}

Oqueka, Tim ^{[1
,2
]}

Huber, Tobias B. ^{[1
]}

Koetter, Ina ^{[1
]}

Krusche, Martin ^{[1
]}

机构：

[1] Univ Med Ctr Hamburg Eppendorf, Dept Med Nephrol Rheumatol & Endocrinol 3, Hamburg, Germany

[2] Univ Med Ctr Hamburg Eppendorf, Dept Med Oncol & Pulmonol 2, Hamburg, Germany

来源：

CHEST | 2023年 / 163卷 / 01期

关键词：

KEY WORDS; anti-CD38; daratumumab; dermatomyositis; interstitial lung disease; MDA5; AMYOPATHIC DERMATOMYOSITIS;

D O I：

10.1016/j.chest.2022.08.2209

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

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

引用

页码：E1 / E5

页数：5

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