The burden of idiopathic pulmonary fibrosis: An unmet public health need

• Published in: Respiratory medicine 2014-07, Vol.108 (7), p.955-967
• Main Authors: Lee, Augustine S, Mira-Avendano, Isabella, Ryu, Jay H, Daniels, Craig E
• Format: Article
• Language: English
• Subjects: Asthma; Burden of illness; Cancer therapies; Cardiovascular disease; Comorbid illness; Comorbidity; Cost of Illness; Drug therapy; Embolisms; Epidemiology; Gastroesophageal reflux; Health Resources - utilization; Health Services Needs and Demand - statistics & numerical data; Health-related quality of life; Healthcare utilization; Heart attacks; Humans; Idiopathic pulmonary fibrosis; Idiopathic Pulmonary Fibrosis - epidemiology; Idiopathic Pulmonary Fibrosis - therapy; Illnesses; Infections; Lung cancer; Mortality; Palliative care; Palliative Care - methods; Population; Public health; Public Health Administration; Pulmonary fibrosis; Pulmonary hypertension; Pulmonary/Respiratory; Quality of Life; Transplants & implants; Ventilation
• ISSN: 0954-6111; 1532-3064
• DOI: 10.1016/j.rmed.2014.03.015

Summary Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease of unknown cause characterized by relentlessly progressive restrictive-ventilatory limitation, hypoxia, dyspnea, and cough. Both the incidence and prevalence of IPF appears to be increasing, with little impact on its dismal 3-year median survival, despite two decades of clinical trials. Increasingly recognized are the serious associated comorbid illnesses, including pulmonary hypertension, chronic obstructive pulmonary disease, gastroesophageal reflux disease, obstructive sleep apnea, obesity, lung cancer, and depression that further contribute to the substantial rise in the use of IPF-related healthcare resources. At present, lung transplantation remains the sole viable treatment for the few who qualify. Pharmacologic interventions targeting lung function and survival have remained largely disappointing, and very few investigations have specifically targeted comorbid conditions, symptoms, quality-of-life, and healthcare resource utilization. In reviewing the burden of illness associated with IPF, including the epidemiology, comorbidities, quality-of-life and the physical, psychosocial, and economic costs of this devastating disease, we hope to highlight some of the unmet medical needs associated with IPF, and encourage both public support and further investigations into these and other patient-centered outcomes and not just that of survival and lung function.