Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive disease

Of idiopathic interstitial lung diseases, IPF has the worst prognosis 3

  • IPF is marked by progressive and irreversible respiratory decline 4
  • While IPF is progressive, there is no way to predict how quickly a patient will progress 4
  • Patients hospitalized as a result of a chronic condition often do not return to prehospitalization levels of functionality, with many experiencing continued functional decline 5*

*The ATS/ERS/JRS/ALAT evidence-based guidelines for the diagnosis and management of IPF defines IPF as a chronic, progressive lung disease.
ATS=American Thoracic Society; ERS=European Respiratory Society; JRS=Japanese Respiratory Society; ALAT=Latin American Thoracic Association.

Progression is unpredictable and can occur at any point 6

Adapted from Ley et al (2011).
Mixed decline refers to periods of relative stability with periods of acute worsening and rapid decline.

The unpredictability of disease progression is a critical concern because once lost, lung function cannot be restored 4

Perception of stability may be misleading in IPF

Symptoms alone are not an accurate way to measure disease stability 7    

  • Lung damage is present before patients experience progression of their symptoms 7
  • Underlying disease progression may continue despite the stable appearance of symptoms 7
  • As fibrosis spreads, lung function becomes more compromised 7
  • Patients with IPF are commonly hospitalized, with the majority of hospitalizations being respiratory-related 8

Regularly monitoring the clinical course of IPF in patients is necessary even when symptoms appear stable 9


Comorbidities are commonly associated with IPF

Prevalence of select comorbidities in a general IPF patient population (N=4716) 10‡§

Adapted from BMC Pulm Med. Mortimer K, Hartmann N, Chan C, Norman H, Wallace L, Enger C. 2019;19(1):11.
Optum Research Database–Medicare Advantage Population. Cohort Entry: January 1, 2008-September 30, 2014.
COPD=chronic obstructive pulmonary disease; GERD=gastroesophageal reflux disease; ICD-9=International Classification of Diseases, Ninth Revision.
Non-interventional cohort study from a Medicare Advantage and Part D plan of medical and pharmacy claims data (ICD-9 code) for patients newly diagnosed with IPF with ≥1 additional medical claim and ≥65 years of age (N=4716).
§Comorbidities of interest were defined using validated algorithms (when available) or with clinical input and searches of medical claims coding systems. Coverage was for at least 12 months.
||Mean follow-up time: 0.8 years. Follow-up time for each cohort member extended from the day after the index date until the earliest of disenrollment from the health plan, death, occurrence of baseline exclusion criterion during follow-up, or the end of the study period. For each outcome, patients were censored for any second occurrences of that outcome, but remained eligible for a different outcome.

Comorbidities are common in patients with IPF and may be an important consideration when developing a treatment plan as they may continue to develop 11