Abstract

BackgroundMacrophage activation syndrome (MAS) is a rare multiorgan system disorder that may present as a fatal complication of underlying rheumatological disease, including dermatomyositis.Case presentationHere, 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.ConclusionsThis 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.

Highlights

  • 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

  • It is commonly associated with systemic juvenile idiopathic arthritis (SJIA), adult onset still disease, Systemic lupus erythematous (SLE), Kawasaki disease, and rarely, dermatomyositis [1,2,3]

  • Our case describes a newly diagnosed melanoma differentiation-associated protein 5 (MDA5)-positive dermatomyositis complicated by MAS with recent diagnosis of COVID-19 infection

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Summary

Conclusions

This report serves as a clinical reminder of the rare, yet clinically relevant association between MDA5positive 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

Background
Discussion and conclusions

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