PURPOSE: It has recently been shown that decreases in forced vital capacity (FVC) of 10% or more from baseline over a period of 6 months are associated with an increased risk of a poorer prognosis in patients with idiopathic pulmonary fibrosis (IPF). To assess the clinical efficacy of pirfenidone in the advanced stage of IPF, we conducted a retrospective study of 17 cases with IPF who received pirfenidone therapy (1200-1800 mg/day) from March 2009 to April 2011.
METHODS: Eligible patients were aged 63-82 years, had a confident clinical and radiologic diagnosis of IPF with disease severity classified as Stage III or Stage IV, indicating that the arterial oxygen partial pressure was less than 69 torr at rest, according to the Japanese Respiratory Society criteria. An effect of pirfenidone on the primary endpoint was defined as the absolute change in forced vital capacity (FVC) from baseline to 6 months. The decreases in FVC of 10% or more from baseline over a period of 6 months was defined as “ineffective” group and others as “effective” group.
RESULTS: Baseline characteristics of pulmonary function tests before pirfenidone therapy were 2.08±0.72L of FVC, 68±18.6% of % predicted FVC and 34.2±11.2% of % predicted DLco, respectively. Pirfenidone therapy significantly stabilized declines in level of FVC at 6 months in 7 of 17 cases (41%). The mean changes in FVC were 70ml and -420ml at 6 months in the effective (n=7) and the ineffective (n=10) groups respectively, with the difference of 490ml being significant (p=0.02). The mean survival period after the pirfenidone therapy was 424±233 days in the effective group and 240±151 days in the ineffective group, respectively (p=0.03). Although photosensitivity was a well-established major side-effect of pirfenidone, it was mild in severity in most of the patients. Four patients discontinued the therapy with gastrointestinal discomfort such as nausea and/or anorexia. However, most of the adverse events disappeared with a decrease in the dose or temporary withholding of the medication.
CONCLUSIONS: This results imply that pirfenidone therapy may decrease the risk of a poorer prognosis even in the advanced stage of IPF.
CLINICAL IMPLICATIONS: This results imply that pirfenidone therapy may decrease the risk of a poorer prognosis even in the advanced stage of IPF.
DISCLOSURE: The following authors have nothing to disclose: Susumu Sakamoto, Fumiaki Ishida, Keita Satoh, Shingi Sakaguchi, Keishi Sugino, Kazutoshi Isobe, Sakae Homma
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