Expectations, symptoms and quality of life before and after one year of pirfenidone treatment in patients with idiopathic pulmonary fibrosis

<b>Background</b>: Anti-fibrotic therapies slow down the progression of idiopathic pulmonary fibrosis (IPF) by reducing lung function decline. However, little is known about the impact of anti-fibrotic drugs on health-related quality of life. This study was aimed to determine the influence of anti-fibrotic treatment on patients’ reported symptoms, along with their experiences and expectations of the therapy. <b>Method</b>: A total of 52 patients with confirmed IPF performed pulmonary function tests (spirometry, TL_COand 6MWT) and surveys (SF-36, SGRQ, BDI, FAS, LCQ and patient's needs and expectations authors’ survey) at baseline and after 12 months of pirfenidone treatment. <b>Results: </b>No significant changes of FVC, TL_CO, and distance in 6MWT were observed after 1 year of therapy. Patients’ needs and expectations in the course of the study did not change, except for the right of deciding on disease management, which was significantly more important after 12 months of treatment as compared with the baseline (p = 0.014). The most important needs for patients were access to novel therapy, fast and easy access to health centres specializing in IPF treatment, and improvement of the general condition or maintenance of its level. The least essential needs were everyday social assistance, psychological support, and access to oxygen therapy at home. Quality of life, measured by the SF-36, after 1 year of pirfenidone treatment significantly improved in mean values of the physical cumulative score (p = 0.004) and mental cumulative score (p = 0.003), namely in role-physical, role-emotional, general health, and mental health. Whereas significant deteriorations were observed in bodily pain and vitality. For the remaining questionnaires (SGRQ, BDI, FAS and LCQ), no significant changes in the course of the study were noticed. However, taking into account individual answers, the majority of patients with IPF declared improvement or stabilization rather than a deterioration in the quality of life, perception of dyspnoea, incidence of cough and fatigue. Additionally, after 1 year of therapy, 12% of patients had simultaneous improvements in the quality of life and reduced dyspnoea and cough intensity. <b>Conclusions: </b>We conclude that 1 year of anti-fibrotic treatment results in a general improvement in quality of life in the SF-36 questionnaire. Patients’ expectations of disease management did not change and access to novel therapies and easy access to health centres specializing in IPF management remained their top needs.