Our study showed that (1) serum IL-6 was not specifically high only in DM RP-ILD but also in patients with DM without RP-ILD; (2) unlike IFN-γ, high serum IL-6 did not correlate with CT scores; and (3) numerous IL-6-positive plasma cells were found in hilar lymph nodes but not in the lungs. Here, IL6 is linked to retinitis pigmentosa 1.