Abstract Background Clinically amyopathic dermatomyositis (CADM) is a disease with the typical skin manifestations of dermatomyositis (DM) and few or no obvious muscle weakness. The anti-melanoma differentiation associated protein 5(MDA5)antibody positive CADM patients are at a high risk of developing rapidly progressive interstitial lung disease (RP-ILD),and exhibit immensely poor prognosis and high mortality. The disease course and severity of MDA5+CAMD are highly different, wherein some patients presented marked treatment response and didn’t occur exacerbation. Analysis to these special groups bring us better understanding of MDA5+CADM. Case presentation We presented a patient with anti-MD5 anti-Ro52 anti-EJ-triple positive DM patient. She was initially characteristic with darken skin for 2 years before gradually occurred dry cough, fever and dyspnea. She had a low oxygen saturation and diffusing capacity of the lungs for carbon monoxide. With combination therapy of GCs and two immunosuppressants, the woman obtained a rapid remission and persisted for a long time. Conclusions Patients with anti-MD5 anti-Ro52 anti-EJ-triple positive DM-ILD may present a less aggressive disease course and an individual appropriate therapy may be promising for long-term maintenance.
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Guo et al. (2024) studied this question.
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