1,11 As such, it is important to understand the economic burden associated with the disease and understand the impact on budgets from the only approved pharmacologic treatments, with no generic substitutions available.Īn examination of health resource usage and costs indicates that patients with IPF have significantly increased healthcare usage compared with demographically matched controls and incur substantial costs to payers both before and after the time of diagnosis.

9,10 Additionally, IPH has 2 costly treatment options: lung transplant or a choice of 2 recently approved pharmacologic treatments for IPF. In the case of IPF, the complexity of reaching a confirmed diagnosis and the need for managing complications and concomitant comorbidities makes managing patients with IPF cost intensive and resource intensive. The rise in incidence is coupled with the usual high cost of treating chronic conditions and the comorbidities that follow, along with the typical health concerns that go along with aging. 2,4,6 This trend may be partly attributed to the aging population, increased awareness of the disease, and improved guidelines in defining and diagnosing the disease. Over the past decade, Medicare data and death certificate data have also shown a trend toward increasing prevalence of IPF among Americans older than 65 years, with increasing survival. This may be due to limitations in coding in the Medicare database. 4,5 These data, compared with previous studies, showed an unusually higher proportion of female patients.

It is interesting to note that among newly diagnosed patients with Medicare, the majority tend to be white (91%) and female (54%). 3 However, male gender is associated with a higher incidence of disease, higher mortality, and shorter survival time after diagnosis. 1 The disease is typically fatal, with median survival estimated at 3 to 5 years from the initial diagnosis. IPF primarily impacts middle-aged to older adults, most with a history of cigarette smoking. However, a minority of patients may remain stable or decline rapidly, with some experiencing episodes of acute respiratory worsening despite previous stability. 1,2 In most patients, the disease progresses with a gradual worsening of lung function over years. IPF is characterized by worsening dyspnea, declining lung function, nonspecific respiratory symptoms, a wide array of associated multiple comorbidities, and a varied clinical course randomly punctuated by episodes of acute exacerbations. 1,2 While it has a histopathologic pattern of usual interstitial pneumonia (UIP)-characterized by diffuse fibrosis and scarring of the interstitium-its etiology is unknown and its natural history is variable and unpredictable. Idiopathic pulmonary fibrosis (IPF) is a complex, progressive disease, challenging to diagnose, due to the need to exclude alternative diagnoses, and challenging to manage. As such, it is imperative that a structured, comprehensive, multidisciplinary management approach is used in the treatment and management of IPF and its associated comorbidities to limit costs and provide effective and quality healthcare. As potentially effective therapies emerge, attention should be given to healthcare resource usage and healthcare processes that ensure patient-centered management with sustainable, cost-effective, and quality care. Both were approved for the treatment of IPF in 2014. Newer treatments, pirfenidone and nintedanib, demonstrate a modest effect on slowing decline in lung function in patients with IPF. Without a lung transplant, the median survival was estimated at 3 to 5 years from the initial diagnosis. Until recently, palliative care and lung transplant were the only options for management of IPF.

The comorbidities, which increase impairment and disability, and compromise patient quality of life and survival, include pulmonary and cardiac conditions, sleep apnea, gastroesophageal reflux disease, depression and anxiety, and lung cancer. The disease, characterized by worsening dyspnea, declining lung function, nonspecific respiratory symptoms, and a varied clinical course randomly punctuated by episodes of acute exacerbations, is also accompanied by a host of comorbid conditions that contribute significantly to increased healthcare usage and cost. Medicare estimates for this disease are as high as $3 billion, not including cost of treatment.

Patients with IPF have significantly increased healthcare usage compared with similar patients without the disease. Clinicians are faced with the need to exclude alternative diagnoses, limited treatment and management guidelines, and few treatment options. Idiopathic pulmonary fibrosis (IPF) is a diagnostically challenging disease.

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