Recurrence in long-term survivor of anti-MDA5 antibody-positive clinically amyopathic dermatomyositis: case series and literature review

Abstract

Anti-MDA5 antibody-positive clinically amyopathic dermatomyositis (CADM) is often complicated by rapidly progressive interstitial lung disease and is associated with poor prognosis. However, even though recurrence is reported to be infrequent if successful medical treatment is administered, the long-term prognosis remains unclear. In this case report, we examined the clinical features and treatment details of three patients with anti-MDA5 antibody-positive CADM with multiple recurrences during long-term survival at Juntendo University Urayasu Hospital. Of the three patients, two failed to convert to an anti-MDA5 antibody-negative status, and one patient died. One of the remaining patients experienced two relapses but eventually tested negative for anti-MDA5 antibodies and showed a relatively stable clinical course. Although cases of recurring anti-MDA5 antibody-positive CADM rarely occur, they may occasionally be fatal. The prognosis for anti-MDA5 antibody-positive CADM has improved over time owing to its establishment as a disease. However, further information and research is necessary to ascertain its long-term prognosis.

Keywords:

• Anti-MDA5 antibody
• dermatomyositis
• interstitial lung disease
• cyclophosphamide
• apheresis

Acknowledgements

The authors are grateful to Ms. Kazumi Goto for providing secretarial assistance. The authors thank Editage (www.editage.com) for English language editing.

These cases were presented at the 63rd Congress of the Japan College of Rheumatology in 2019.

Patient consent

All patients and next to kin of patient number 2 provided written informed consent for their record to be published in the present study.

Ethical approval

Not applicable.

Conflict of interest

None.

Data availability statement

The datasets generated during and/or analysed in this study are available from the corresponding author upon reasonable request.