Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis: A case–control study

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Abstract

Background and objective

Treatment with pirfenidone may slow the decline in vital capacity and increase progression-free survival (PFS) in idiopathic pulmonary fibrosis (IPF). The effects of combination therapy with inhaled N-acetylcysteine (NAC) and pirfenidone are unclear. We assessed the effects of this combination therapy in patients with advanced IPF.

Methods

Patients with a diagnosis of advanced IPF (Japanese Respiratory Society stage III/IV IPF) and a relative decline in forced vital capacity (FVC) of ≥10% within the previous 6 (±2) months were enrolled. Outcomes were evaluated in a 12-month follow-up pulmonary function test. Treatment was considered ineffective if the decline in FVC was ≥10% and effective if the decline was <10%. We compared clinical characteristics, effectiveness and PFS between patients receiving inhaled NAC plus pirfenidone (n= 24) and those receiving pirfenidone alone (control;n= 10).

Results

Data from 34 IPF patients (age range, 59–82 years) were analysed. At the 12-month follow-up examination, treatment was deemed effective in 8 of 17 (47%) patients receiving NAC plus pirfenidone and in 2 of 10 (20%) receiving pirfenidone alone. The annual rate of change in FVC was −610 mL in the NAC plus pirfenidone group and −1320 mL in the pirfenidone group (P< 0.01). PFS was longer (304 days) in the NAC plus pirfenidone group than in the pirfenidone group (168 days;P= 0.016).

Conclusions

Combination treatment with inhaled NAC and oral pirfenidone reduced the rate of annual FVC decline and improved PFS in patients with advanced IPF.

SUMMARY AT A GLANCE

We assessed the effects of combination therapy with inhaled NAC and pirfenidone in advanced IPF. We compared effectiveness and progression-free survival between patients receiving NAC plus pirfenidone (n = 24) and those receiving pirfenidone alone (n = 10). Patients with advanced IPF had favourable outcomes after combined treatment with pirfenidone and NAC.

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