Open-label Compassionate Use One Year-treatment with Pirfenidone to Patients with Chronic Pulmonary Fibrosis.

Abstract

Objective Weattempted to evaluate the feasibility (therapeutic efficacy, tolerance, and clinical courses after treatment) of pirfenidone, an anti-fibrotic drug for patients with chronic pulmonary fibroses such as idiopathic pulmonary fibrosis (IPF).Methods Open-label one-year treatment for compassionate-use. Patients or Materials: Oral pirfenidone (40 mg/ kg body weight) was administered to 8 patients with advanced IPF and 2 with interstitial pneumonia associated with diffuse systemic sclerosis. The plasma concentration of the drug was serially followed. Radiographic scores, pulmonary functions, and arterial blood oxygen pressure were compared at three time points: at one-year before treatment, at the time of entry, and at one-year after entry. Results While pirfenidone did not show a definite therapeutic effect on overall survival (2 years after entry). During one-year treatment, there was no significant deterioration in terms of chest radiographic scores and arterial oxygen pressure, and the drug was well tolerated with minimal adverse effects within the ranges observed in this study, plasma pirfenidone concentrations did not seem to relate to the appearance of adverse effects or differences in therapeutic effects.

Pirfenidone Is Renoprotective in Diabetic Kidney Disease

“…Of note, one clinical study with PFD in patients with FSGS did not find any reduction of BP. 46 Thus far, the only clinical study reported to date for PFD in kidney disease is an open-label study of patients with advanced FSGS. 19 This study demonstrated that patients with refractory FSGS, whose disease did not respond to steroids, may have a slower decline in the renal function with PFD.…”

Pirfenidone Slows Renal Function Decline in Patients with Focal Segmental Glomerulosclerosis

“…Regression to the mean is most a The presence of gastrointestinal adverse events (dyspepsia, nausea), sedation or fatigue, or photosensitivity dermatitis at any time during pirfenidone therapy in this study. For comparison, the rates of adverse events are provided for other clinical studies (19,36,37), excluding those that used a higher pirfenidone dosage or studied patients with advanced liver disease, which is expected to impair pirfenidone metabolism.…”

The bleomycin animal model: A useful tool to investigate treatment options for idiopathic pulmonary fibrosis?

“…Some of them were retrospective analyses or case series only. Among the drugs tried or on trial are Etanercept, Imatinib, Prednisone (Daniil et al, 1999;Douglas et al, 1997;Douglas, Ryu, & Schroeder, 2000;Douglas et al, 1998;Nicholson AG, 2000;Riha et al, 2002;Ziesche, Hofbauer, Wittmann, Petkov, & Block, 1999), N-Acetylcysteine (Demedts et al, 2005), TGF-β antibody (Genzyme, 2007), Interferon-γ (Antoniou et al, 2006;Raghu et al, 2004;Raghu R, 2001), Interferon-β (Raghu, Bozic, & Brown, 2001), Pirfenidone (Azuma, Nukiwa et al, 2005;S. Nagai et al, 2002;Raghu, Johnson, Lockhart, & Mageto, 1999), Colchicine (Douglas, Ryu, & Schroeder, 2000;Douglas et al, 1998;Selman et al, 1998), Bosentan, Cyclosporin-A (Alton, Johnson, & Turner-Warwick, 1989;Moolman, Bardin, Rossouw, & Joubert, 1991), D-Penicillamin (Chapela, Zuniga, & Selman, 1986;Selman et al, 1998), Heparin (Kubo et al, 2005), Relaxin (ATS, 2002), Angiotensin converting enzyme (ACE) inhibitors (Nadrous, Ryu, Douglas, Decker, & Olson, 2004), and CTGF antibodies (Mageto Y, 2004).…”