A new study has assessed the course of interstitial lung disease (ILD) among individuals with limited cutaneous systemic sclerosis (lcSSc) and found that nintedanib may slow lung function decline in this patient group. The study, published in Rheumatology, highlights the importance of prompt treatment of pulmonary fibrosis in patients with lcSSc.

“Although interstitial lung disease (SSc-ILD) is more common in patients with diffuse cutaneous SSc (dcSSc), a substantial proportion of patients with lcSSc develop SSc-ILD,” the authors wrote. “Nintedanib has been licensed for the treatment of SSc-ILD, idiopathic pulmonary fibrosis and other chronic fibrosing ILDs with a progressive phenotype.”

The research team analyzed data from the SENSCIS study, which enrolled 277 individuals with SSc-ILD, including those with lcSSc. Participants were randomized to receive nintedanib 150 mg twice a day or placebo for at least 52 weeks but less than 100 weeks. SENSCIS-On was an open-label extension for 225 eligible patients completing the first trial. Data on baseline characteristics and the rate of forced vital capacity (FVC) decline over 52 weeks were collected.

Overall, despite marked fibrosis and considerable lung function impairment, approximately 30% of the patients studied had no dyspnea and 20% had no cough. This finding suggests that respiratory symptoms can be a late presentation of SSc-ILD and highlights the importance of screening for ILD at diagnosis.

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The analyses found that pulmonary fibrosis may progressively develop in individuals with lcSSc several years after their SSc diagnosis. Nintedanib slowed the rate of FVC decline over the study of 52 weeks in individuals with both lcSSC and ILD compared with placebo, with a manageable adverse event profile.

The authors caution that the trials were not sufficiently facilitated to assess outcomes and there was no placebo comparator in the SENSCIS-ON trial, but they nonetheless conclude that screening for ILD and prompt treatment initiation are essential for patients with lcSSc to preserve their lung function.


Allanore Y, Khanna D, Smith V et al. Effects of nintedanib in patients with limited cutaneous systemic sclerosis and interstitial lung disease. Rheumatology. Published online June 9, 2023. doi.org/10.1093/rheumatology/kead280