IPF overview

Idiopathic pulmonary fibrosis (IPF) is a debilitating and fatal lung disease with high mortality. It causes permanent scarring of the lungs, difficulty breathing and decreases the amount of oxygen the lungs can supply to the major organs of the body.1 IPF progression is variable and unpredictable and over time the lung function of an IPF patient gradually and irreversibly declines.1 IPF exacerbations – events of acute respiratory worsening – can also impact the course of the disease, often leading to death within a few months.2 Approximately 5-14% of all IPF patients may experience an exacerbation over a year. 3,4 Acute IPF exacerbations are a risk to all IPF patients because they may occur at any time during the course of the disease without warning or known cause.3
 

Prevalence and possible causes

IPF affects as many as 14-43 people per 100,000 worldwide.5 The disease primarily affects patients over the age of 50 and affects more men than women.6,7 The cause of IPF is unknown, but some risk factors may include smoking, lung injury, family history of the disease, abnormal acid reflux, environmental exposures and chronic viral infections. 1,4
 

Symptoms of IPF

Individuals with IPF may experience symptoms such as breathlessness during activity, a dry and persistent cough, chest discomfort and finger clubbing. 4 2 Adapted from Meltzer & Noble Orphanet Journal of Rare Diseases 2008
 

Diagnosing IPF

Diagnosing IPF can be difficult because it requires specific diagnostic testing, such as lung imaging using a high-resolution CT scan.4

The average time from first symptoms to diagnosis is between one and two years.8,9 Initial misdiagnosis occurs in around half of patients because symptoms are similar to other respiratory diseases like COPD, asthma and to congestive heart failure.8

Over 80% of patients with IPF have a distinctive, Velcro^®-like crackling sound (the “Sound of IPF”) that can be detected through a stethoscope.9,10
 

Management options

Early and accurate diagnosis of IPF is important, as management options such as pharmacological treatment, supplemental oxygen treatment, cough management and pulmonary rehabilitation (which can include special exercises or breathing techniques) may help patients manage their condition and maintain their quality of life.4
 

Prognosis

The prognosis for those diagnosed with IPF varies, but approximately half of the patients die within 2–3 years after diagnosis.4

It is increasingly apparent that sudden deterioration in respiratory function with unidentified cause, known as an acute IPF exacerbation, significantly reduces a patient’s chances of survival. 11
 

Treatments for IPF

Until recently, treatment options for IPF have been limited. Since scarring of the lung that has already occurred cannot be reverted, the only current chance for a cure is lung transplantation. 12 This however is only an option for a very limited number of patients.

There is a high unmet need for safe and effective treatments that can alter the course of IPF by slowing disease progression. Two treatments have now been approved in the United States, European Union, Japan and other territories for the treatment of IPF and several other compounds are currently being studied in clinical trials. 13

The 2015 international evidence-based IPF guideline, 14 jointly developed by an international committee including the American Thoracic Society (ATS), the European Respiratory Society (ERS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT) provides physicians with updated treatment recommendations for patients with IPF.

The guideline recommends the use of the following agents (conditional recommendation**):14

• Nintedanib, a tyrosine kinase inhibitor that targets multiple tyrosine kinases, including vascular endothelial growth factor, fibroblast growth factor, and PDGF receptors
• Pirfenidone

The committee noted the high value of nintedanib on patient-important outcomes such as disease progression as measured by rate of forced vital capacity (FVC) decline and mortality. The recommendation also takes into account the expected cost of treatment and potentially significant adverse effects.

* Nintedanib is approved under the brand name OFEV^® in the US, EU, Japan and other territories for use in patients with IPF. Nintedanib is under regulatory review by health authorities in other countries. 4
**This is a conditional recommendation. This means that clinicians must discuss preferences with their patients to help patients reach treatment decisions consistent with their values and preferences.

For more information on IPF

Please visit:

www.inipf.com
www.lifewithipf.com