The oral
Janus kinases inhibitor (JAKi) has improved the management of
skin manifestations in
systemic sclerosis (SSc), and our study aimed to explore the efficacy of non-selective JAKi
tofacitinib in ameliorating
interstitial lung disease (ILD) in the patients with SSc. The hospitalization data of the SSc-ILD patients from April 2019 to April 2021 were collected, and the changes of pulmonary function and the radiological findings in pulmonary high-resolution CT (HRCT) from the 9 patients who received
tofacitinib for at least 6 months and a matched group of 35 SSc-ILD patients treated with conventional
immunosuppressants or
glucocorticoids, were compared and analyzed. There were no significant differences in demographic data and clinical characteristics between the
tofacitinib-treated group (
tofa-group) and the matched group. However, in the
tofa-group, the changes in serum
lactate dehydrogenase (LDH) concentration and serum
interleukin-6 levels were significantly lower than those in the matched group. Moreover, the
tofa-group showed amelioration in decreased diffusing capacity of the lung for
carbon monoxide (DLCO) (62.05 ± 9.47 vs. 66.61 ± 12.39, p = 0.046), reductions in ground-glass attenuation involvement (1.00 ± 0.86 vs. 0.33 ± 0.50, p = 0.024) and irregular pleural thickening (1.33 ± 0.50 vs. 0.67 ± 0.51, p = 0.004) in pulmonary HRCTs, alleviated modified Rodnan skin score (mRSS) of skin
sclerosis (9.22 ± 3.81 vs. 7.11 ± 3.92, p = 0.048), and reduced HRCT scores of
pulmonary fibrosis (15.00 ± 3.87 vs. 12.66 ± 4.92, p = 0.009). Logistic regression analysis showed that the involvement of ground-glass attenuation (OR 11.43) and the add-on
therapy of
tofacitinib (OR 9.98) were the relevant factors in the amelioration of HRCT. Our results indicate that the use of JAKi (
tofacitinib) may be relevant to significant improvement of the
sclerosis and early radiological abnormalities in SSc-ILD patients. Further studies are needed to confirm these findings and to explore its efficacy more precisely. Key Points • The currently available
therapies for SSc-ILD have limited therapeutic benefits. • The add-on
therapy of the oral
JAK inhibitor is available in the real world. • The
tofacitinib was promising in the improvement of the
sclerosis and early radiological abnormalities in SSc-ILD patients.