Inflammatory myopathies

Short-term and long-term outcomes of patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis

Rheumatology (Oxford)Published Retrospective cohort study

Authors' conclusion

Although relapse often occurs, the long-term survival of MDA5-DM patients who survived the remission induction phase is generally favourable. The status of the anti-MDA5 antibody is associated with relapse. JAKi may improve the survival of refractory patients with severe MDA5-DM.

Study context

Population
Patients with anti-MDA5 antibody-positive dermatomyositis (MDA5-DM)
Participants
154 patients
Outcome
Short-term and long-term outcomes, including survival, relapse and anti-MDA5 antibody titre

Main finding

As reported in the abstract

Death during remission induction phase (respiratory failure most common cause)
40 (26.0%)
5-year cumulative survival among 114 surviving remission induction
96.8%
5-year relapse-free survival among 114 surviving remission induction
77.4%
Complete drug-free remission
7.9%
Normalization of anti-MDA5 antibody titres; only two relapsed after normalization
54

Observational data: associations, not effects.

Further findings

6-month survival in severe patients, after vs before emergence of Janus kinase inhibitors (JAKi) treatment

6-month survival significantly higher after emergence of JAKi treatment
P = 0.03

Original publication

Short-term and long-term outcomes of patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis. Rheumatology (Oxford). Published 12 January 2024. doi:10.1093/rheumatology/keae011

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