Patient Registry Reveals Critical Information About Idiopathic Pulmonary Fibrosis

According to a story from blogs.biomedcentral.com, the findings from the IPF-PRO Registry, which is a US-based registry for idiopathic pulmonary fibrosis patients, were recently published. Patient registries are a critical resource for rare disease research, and data from them can help contribute to the development of successful therapies. The IPF-PRO Registry has helped reveal critical information about the progression of idiopathic pulmonary fibrosis.

About Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis is a deadly, chronic, progressive lung disease which is characterized by lung tissue scarring, leading to a decline in lung function over time. The cause of idiopathic pulmonary fibrosis is unknown. With that being said, there are a few risk factors that have been identified, such as smoking cigarettes, exposure to various dusts (metal, wood, stone, and coal dust), occupations related to farming, family history, and potentially certain viral infections. Symptoms include shortness of breath, a dry cough, a distinctive crackling sound detected with a stethoscope, oxygen deficiency in the blood, and clubbed digits. There are few treatment options that can have a significant impact on the progression of idiopathic pulmonary fibrosis. Treatment may include certain medications, pulmonary rehabilitation, oxygen therapy, and lung transplant. Early intervention can make a major difference in outcomes; five year survival rate is between 20 and 40 percent. To learn more about idiopathic pulmonary fibrosis, click here.

Research Using Registry Data

The course of idiopathic pulmonary fibrosis varies considerably for each patient and it is effectively impossible to predict with any degree of precision. The IPF-PRO Registry currently has 1000 patients from across the US enrolled. The study looked at data from a 30 month period following registration for a total of 662 patients. The researchers found that patients had a 50 percent chance of either needing a lung transplant or dying outright during that time frame. 

Risk factors included the need for supplemental oxygen while resting, reduced forced vital capacity, or reduced diffusing capacity of the lungs. Younger patients tended to be at highest risk, followed by the oldest patients. Patients around age 60 did not see their risk increase because of their age.

Future research utilizing data from the registry will compare whether clinical changes or changes in symptoms as reported by patients are more accurate in predicting mortality.

These studies are just a sample of how data from registries can be utilized. The original study was published in the journal Respiratory Research.


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