Abstract
Background: Several reports reveal the favorable effect of pirfenidone on early stage of idiopathic pulmonary fibrosis (IPF), but impacts on non-specific interstitial pneumonia (NSIP) and interstitial pneumonia (IP) associated with collagen vascular diseases (IP-CVD) are not clear.
Objectives: To examine the effect of pirfenidone on chronic IP including IPF, NSIP and IP-CVD.
Methods: Thirty-two patients were enrolled in the study evaluating the safety and efficacy of pirfenidone in IPF, NSIP and IP-CVD. Clinical diagnosis are IPF(14), NSIP(14), IP associated with scleroderma(3), and rheumatoid arthritis(1). Based on PaO2 at rest and SpO2 after 6 minutes walk test (6MWT), disease severity of those were classified into four groups [Table 1]. We retrospectively analyzed subjective symptom in British Medical Research Council scale, pulmonary function, KL-6, SP-D and CT findings before and during pirfenidone administration.
Results: Correlations was observed between subjective symptom and VC, but was not between subjective symptom and KL-6, SP-D, CT findings. After administration of pirfenidone, VC improved above 5% in 22%, 50%, 25%, 45% of patients (severity grade I, II, III, IV, respectively). Subjective symptoms highly improved in patients with scleroderma (improvement ratio: 67% in scleroderma vs. 14%, 14%, 0% in IPF, NSIP, RA, respectively).
Conclusions: Pirfenidone might have the favorable efficacy independent of disease severity, without severe adverse events, in chronic IP, especially in IP associated with scleroderma.
- © 2012 ERS