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IPF: promising new possibilities might offer help with deadly disease

Idiopathic pulmonary fibrosis (IPF) is a disease that can be lethal and can gravely affect the lives of patients. It is a chronic and progressive form of lung disease that results in scarring of the lungs, which restricts breathing and oxygen exchange. How many people are affected by this disease? What are the causes and what are (possible) remedies? In this article we examine some of the basic characteristics of IPF with the aid of an insightful infographic. We also look at the current state of affairs concerning one of the most lethal aspects of IPF: the acute exacerbation.

In fact, IPF is a disease that's very hard to distinguish from other conditions and 50 percent of the patients are misdiagnosed.

IPF currently affects 14 to 43 people per 100.000 population and is seen more frequently in men than in women. The most common symptoms of IPF are shortness of breath, a chronic dry cough and finger clubbing. This last symptom is also known as 'drumstick fingers', a rather self-explanatory term that describes the phenomenon well: the end of the fingers get larger and the nails start to curve, giving the impression of 'drumsticks'. Occasional symptoms of IPF include fatigue, weakness and weight loss. Unfortunately, a symptom like 'shortness of breath', while pointing to the lungs, is not very specific for IPF. In fact, IPF is a disease that's very hard to distinguish from other conditions and 50 percent of the patients are misdiagnosed. 


The cause of IPF is unknown, but risk factors may include smoking, environmental exposures and chronic viral infections. It can be a lethal condition: 50 percent of the patients die within 2 to 3 years after the diagnosis. One of the most serious aspects of IPF is acute exacerbations (AE's), these are defined as a sudden acceleration of the disease that leads to a significant decline in lung function. They are associated with a mortality rate as high as 85 percent, with mean survival periods of between 3 to 13 days. In a review article that was published in the Journal of Thoracic Disease, Maya Juarez and colleagues point to some promising new possibilities for the treatment of AE-IPF, such as the combined use of tacrolimus and corticosteroids, the use of antifibrotics and the removal of select immune system cells.

Juarez MM, Chan AL, Norris AG, Morrissey BM, Albertson TE. Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies. J Thorac Dis 2015;7(3):499-519. doi:10.3978/j.issn.2072-1439.2015.01.17

Infographic over IPF Boehringer Ingelheim.