Abstract
BACKGROUND: To review the experience of our Department’s ILD clinic with the use of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF). METHODS: Patients that were treated at the ILD clinic of the NHS Pulmonology Department. RESULTS: The drug was administered to 21 patients. Of these, six are not included in the analysis (2 were lost to follow-up, and 4 began treatment during the last 2 months). The study concerns 15 elderly patients [70.9 (9.4) years old], mainly male (n=12), ex-smokers (n=12), with 29.4 (22.3) p-y smoking history, who were diagnosed 40.3 (29.8) months ago. Five were receiving long term oxygen therapy, all received proton pump inhibitors and three had been treated with corticosteroids in the past. The IPF diagnosis was reached by VATS lung biopsy in four patients and with compatible HRCT and BAL findings in the rest. Mean duration of follow-up while on treatment was 19.5 (7.3) months. Three patients discontinued treatment due to adverse effects: (Skin rush:1, Diarrheas and skin rush:1, generalized symptoms:1). The last patient resumed treatment, albeit at a reduced dose (2 caps t.i.d.). One patient developed an allergic skin rush after reintroduction of pirfenidone that was treated with corticosteroids and antihistamines. One patient passed away. In one patient there was change of treatment due to disease progression. There were no episodes of hepatotoxicity and there was only one case of significant weight loss in a patient that was on the drug for 25 months. No acute exacerbations requiring hospitalization were recorded in the study population. CONCLUSIONS: Although the number of patients is small, experience with the drug shows a good safety profile, without significant side effects in the majority of patients. The study population remained spirometrically stable during the study.