Table 1

Summary of the clinical trials that used pirfenidone for fibrosis-related diseases

Disease

Dose and time

Type of study

Number of patient

Effects

Reference

Interstitial pulmonary fibrosis

3600 mg/daily for 2 years

Open-label study

54

Increase in 1 and 2 year survival. Stabilized lung function.

[42]

Interstitial pulmonary fibrosis

600 mg three times daily for 12 months

Double-blind, randomized, placebo- controlled trial

107

Improvement in SpO₂ during a 6-minute exercise test. No episodes of acute exacerbation of IPF in PFD group.

[43]

Hermansky-Pudlak syndrome

800 mg three times daily for 44 months

Randomized, placebo-controlled trial

11

Loss of pulmonary functions occurred at a slower rate.

[44]

HCV related liver disease

1200 mg/daily for 12 months

Open-label pilot study

15

30% of patients had less fibrosis after treatment. Downregulation of Col I, TGFβ and TIMP-1. Improvement in quality of life (SF-36 test).

[55]

Focal segmental glomerulosclerosis

800 mg three times daily for 5 to 37 months

Open-label pilot study

18

Slowed renal function decline; improvement of 25%

[67]

Hypertropic scars

PFD 8% gel for 6 months

Open-label pilot study

33

Improvement in the Vancouver scar score in 66.6% of patients.

[70]

HCV = hepatitis C virus; PFD = pirfenidone; SF = Short Form; SpO₂ = lowest oxygen saturation by pulse oximetry; TGF = transforming growth factor; TIMP = tissue inhibitor of metalloproteinase.

Macías-Barragán et al. Fibrogenesis & Tissue Repair 2010 3:16   doi:10.1186/1755-1536-3-16