{Reference Type}: Journal Article
{Title}: Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies.
{Author}: Ramos-Martinez E;Rodríguez-Vega EA;Rivera-Matias PA;Falfán-Valencia R;Pérez-Rubio G;Mejia M;González-Pérez MI;Buendia-Roldán I;Mateos-Toledo HN;Serrano JR;
{Journal}: Med Clin (Barc)
{Volume}: 161
{Issue}: 12
{Year}: 2023 12 22
{Factor}: 3.2
{DOI}: 10.1016/j.medcli.2023.07.013
{Abstract}: The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival.<BR>This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival.<BR>Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02-13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT.<BR>Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD.