Progranulin correlated with rapid progressive interstitial lung disease in dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody.

Objective: This study aimed to detect the expression of progranulin (PGRN) and elucidate associations with clinical features in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody.

Methods: We enrolled 40 DM patients with anti-MDA5 antibody, 20 patients with antisynthetase syndrome (ASS; disease control), and 20 healthy individuals (healthy control, HC). The clinical features of patients with anti-MDA5 antibody and anti-histidyl-tRNA antibody were collected. The level of PGRN in the serum was tested by ELISA.

Results: The PGRN levels in DM patients with anti-MDA5 antibody (166.74 ± 97.95 ng/ml) were significantly higher than those in patients with ASS (82.66 ± 40.50 ng/ml; p < 0.001) and in HC (42.34 ± 18.69 ng/ml; p < 0.001). Patients with rapid progressive interstitial lung disease (RP-ILD) in DM with anti-MDA5 antibody (213.57 ± 114.05 ng/ml) had higher levels of PGRN than those without RP-ILD (135.51 ± 72.41 ng/ml; p = 0.012). ROC analysis showed an AUC value at 0.715 (95% CI, 0.541-0.888) for diagnosis of RP-ILD in DM patients with anti-MDA5 antibody. The expression of PGRN was positively correlated with the levels of ALT, AST, CK, LDH and ferritin (all p < 0.05).

Conclusions: Our results indicated PGRN had great potential as a valuable serum marker of RP-ILD in DM with anti-MDA5 antibody. Key Points The level of PGRN was elevated in DM patients with anti-MDA5 antibody, especially for those with RP-ILD.