#4-46E. Changing the Prognosis: Nerandomilast for Autoimmune Progressive Pulmonary Fibrosis (Ann Rheum Dis, 2026)

Are we on the verge of a breakthrough in altering the grim prognosis of progressive pulmonary fibrosis (PPF) associated with autoimmune diseases? This episode examines the FIBRONEER-ILD trial, which evaluated the efficacy and safety of the phosphodiesterase 4B inhibitor nerandomilast in patients with autoimmune disease-related PPF. The study demonstrated that nerandomilast significantly attenuated the decline in forced vital capacity (FVC) at week 52 while maintaining a favorable safety profile. The most striking finding is that the 18 mg twice-daily dose of nerandomilast yielded a remarkable 72% reduction in the risk of mortality, demonstrating consistent efficacy regardless of concurrent immunomodulatory therapy. However, when critically appraising this paper, we must note that the trial lacked sufficient statistical power to evaluate efficacy within specific autoimmune disease subtypes, and the safety and efficacy of combining nerandomilast with certain excluded immunosuppressants, such as mycophenolate mofetil, remain unclear. For clinicians, these findings are highly relevant as they introduce a promising novel therapeutic option for refractory PPF that progresses despite standard treatments, directly impacting our strategies to improve patient survival and long-term clinical outcomes.

Citation: Hoffmann-Vold AM, et al. Efficacy and safety of nerandomilast in patients with autoimmune disease-related progressive pulmonary fibrosis in the FIBRONEER-ILD trial. Annals of the Rheumatic Diseases. 2026. DOI: https://doi.org/10.1016/j.ard.2026.03.027

Disclaimer: This audio summary is based on personal interpretation and does not guarantee the exact content of the original paper. Please refer to the original article for details.