Macrophage activation syndrome in MDA5 antibody-positive dermatomyositis and COVID-19 infection.

MedStar author(s):
Citation: Bmc Rheumatology. 5(1):59, 2021 Dec 13.PMID: 34895325Institution: MedStar Union Memorial HospitalDepartment: Internal Medicine ResidencyForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: IN PROCESS -- NOT YET INDEXEDYear: 2021ISSN:
  • 2520-1026
Name of journal: BMC rheumatologyAbstract: BACKGROUND: Macrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis.CASE PRESENTATION: Here, we report the case of a 65-year-old Caucasian female with a history of psoriasis and a recent diagnosis of Coronavirus disease 2019 (COVID-19) who presented with progressive generalized weakness, joint pains, an erythematous rash, shortness of breath, and weight loss. She was ultimately diagnosed with biopsy-confirmed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS, requiring intravenous immunoglobulin and high-dose methylprednisolone.CONCLUSIONS: This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5-positive dermatomyositis and MAS, as well as highlights the potential contribution of other immune system activating diseases, such as COVID-19, associated with a cytokine storm and hyperinflammatory state. Copyright (c) 2021. The Author(s).All authors: Balagani A, Chhetri S, Haas CJ, Keshtkarjahromi M, Tayyab UBFiscal year: FY2022Digital Object Identifier: ORCID: Date added to catalog: 2022-01-25
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Journal Article MedStar Authors Catalog Article 34895325 Available 34895325

BACKGROUND: Macrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis.

CASE PRESENTATION: Here, we report the case of a 65-year-old Caucasian female with a history of psoriasis and a recent diagnosis of Coronavirus disease 2019 (COVID-19) who presented with progressive generalized weakness, joint pains, an erythematous rash, shortness of breath, and weight loss. She was ultimately diagnosed with biopsy-confirmed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS, requiring intravenous immunoglobulin and high-dose methylprednisolone.

CONCLUSIONS: This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5-positive dermatomyositis and MAS, as well as highlights the potential contribution of other immune system activating diseases, such as COVID-19, associated with a cytokine storm and hyperinflammatory state. Copyright (c) 2021. The Author(s).

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