Treatment with nintedanib in patients with pleuroparenchymal fibroelastosis

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a progressive fibrotic lung disease characterized by pleural thickening, pleural and parenchymal fibrosis predominately involving upper parts of the lungs. Survival is dismal and no drug has been shown to modify disease course. Aim and objectives Since parenchymal fibrosis is an important histological feature, we hypothesized that anti-fibrotic treatments might be effective in reducing disease progression. Here, we report our experience using nintedanib. Patients We reviewed patients diagnosed with PPFE and admitted to our center. Out of 11 patients, 5 received nintedanib (150 mg twice daily), were followed for at least 3 months. PPFE was idiopathic in 3 patients and secondary to chemotherapy in 2 patients. Patients were treated off-label at the discretion of the physician. Nintedanib was combined with 10 mg/day of prednisone inpatients #4 and #5. Results: In all 5 patients, including2 of them who had previously received pirfenidone, FVC declined steadily before initiation of nintedanib. Treatment with nintedanib was followed by apparent stabilization of FVC (Fig.1) with a median follow-up of 10 months (range, 3-13 months). Tolerability of nintedanib was generally good. Conclusion: Nintedanib may reduce FVC decline in patients with idiopathic and secondary PPFE.