In patients with ILD at baseline, changes in anti-Jo-1 antibody levels were correlated with changes in PGA, pulmonary, and joint VAS scores; IgG, IgA, IgM, and CRP levels; %FVC; %FEV1; and percent predicted carbon monoxide diffusion capacity (%DLco) (all p < 0.05). Here, CD40LG is linked to interstitial lung disease.