Summary
Background
Anti-MDA5 antibodies are associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM).
Objective
We aimed to evaluate the relevance of monitoring anti-MDA5 antibody levels for the management of RP-ILD in patients with CADM or DM.
Methods
Twelve patients with CADM or DM (CADM, 10; DM, 2) accompanied by RP-ILD were included. Baseline characteristics and outcomes were recorded. Serial measurements of anti-MDA5 antibody levels were measured. All patients were treated with corticosteroids, tacrolimus, and intravenous cyclophosphamide.
Results
All patients achieved RP-ILD remission after combined immunosuppressive therapy for a mean time of 6.8 months, with significant decreases noted in the mean anti-MDA5 antibody levels at remission. Six (50%) patients became anti-MDA5-antibody negative conversion after therapy. Upon a mean follow-up of 31 months, RP-ILD relapse was observed in 4 (33%) patients in both the anti-MDA5-antibody-sustained positive group and the negative conversion group. However, relapsed patients in the sustained positive group relapsed earlier than those in the negative conversion group. Thus, a decrease in anti-MDA5 antibody levels during remission was associated with longer remission. Relapses were associated with a re-increase of anti-MDA5 antibody levels in 4/4 (100%) patients. In contrast, none of the patients without re-increase in anti-MDA5 antibody exhibited symptoms of relapse during follow-up. Therefore, re-increase in anti-MDA5 antibody levels was associated with relapse.
Conclusions
The anti-MDA5 antibody level is a novel parameter for monitoring and a good predictor of RP-ILD relapse in patients with CADM or DM.
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