• Lars Hagmeyer, Marcel Treml, Christina Priegnitz, and Winfried J Randerath.
• Clinic for Pneumology and Allergology, Center of Sleep Medicine and Respiratory Care, Bethanien Hospital, Solingen, Germany.
• Respiration. 2016 Jan 1; 91 (4): 327-32.

AbstractPirfenidone and nintedanib are both pleiotropic anti-fibrotic agents approved for the treatment of idiopathic pulmonary fibrosis (IPF) as monotherapy. To date, evidence supporting their efficacy as concomitant therapy has not been reported. Here, we present the first case of a Caucasian male patient with IPF treated with both pirfenidone and nintedanib following 2 years of treatment with pirfenidone monotherapy. Over a 24-month period, there was a clear decline in the patient's forced vital capacity from 3.5 liter before initiation of treatment to 2.5 liter after 24 months. Concomitant nintedanib treatment was initiated in March 2015. Lung function stabilized, and the two treatments were well tolerated. Treatment with pirfenidone and nintedanib has currently been ongoing for nearly 12 months. This is the first report of a successful long-term treatment with pirfenidone and nintedanib and suggests that in selected cases, concomitant anti-fibrotic therapy may represent a safe and therapeutically valuable escalation option after pirfenidone monotherapy. © 2016 S. Karger AG, Basel.

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