Anti-CADM-140/MDA5 autoantibody titer correlates with disease activity and predicts disease outcome in patients with dermatomyositis and rapidly progressive interstitial lung disease

We examined the relationship between disease activity and anti-CADM-140/MDA5 titer measured by enzyme-linked immunosorbent assay (ELISA). Sera from 63 patients with dermatomyositis (DM) [46 classic DM, 17 clinically amyopathic DM (CADM)] were screened for autoantibody using immunoprecipitation assay. Anti-CADM-140/MDA5-positive sera were examined for their titer by anti-CADM-140/MDA5 ELISA. Potential associations between anti-CADM-140/MDA5 titer and clinical course or outcome were analyzed. Sera from 14 patients with DM (2 classic DM, 12 CADM) had anti-CADM-140/MDA5. Of ten patients with DM and rapidly progressive interstitial lung disease (RP-ILD), the mean titer of anti-CADM-140/MDA5 before treatment was significantly lower in patients who responded to therapy and survived (responder group, n = 4) than in those who did not respond and died (nonresponder group, n = 6) (110.3 vs. 356.9, P = 0.019). In the responder group, the mean titer of anti-CADM-140/MDA5 significantly decreased down to below the cutoff level after treatment (n = 3, 113.4 vs. 1.6, P = 0.033), whereas that of the nonresponder group did not decrease sufficiently and sustained high level (n = 4, 372.5 vs. 198.4, P = 0.31). These results emphasize the clinical importance of anti-CADM-140/MDA5 antibody levels to predict outcomes of RP-ILD as well as to monitor disease activity in patients with DM and RP-ILD.