Raising pulmonary fibrosis awareness for Rare Disease Day

Almost 90% of Americans are unaware of the symptoms that signal pulmonary fibrosis due to the disease’s rarity, according to a Pulmonary Fibrosis Foundation survey.

The foundation also noted that even though there are more than 200 types of pulmonary fibrosis, most people do not know about this disease, and this leads to late diagnoses and advanced disease progression.

Rare Disease Day on Feb. 28 serves as a reminder to raise awareness of this disease and others that are widely unknown.

To mark the day, Healio compiled a list of recent stories on pulmonary fibrosis:

‘Time lost is lung lost’: Early diagnosis critical in pulmonary fibrosis

Delayed diagnosis of pulmonary fibrosis is an unfortunate reality for many patients, but experts are seeking to address this problem by closing knowledge gaps and increasing awareness of the disease. Read more.

FDA grants orphan drug designation to AI-created drug for idiopathic pulmonary fibrosis

The FDA granted orphan drug designation to INS018_055 (Insilico Medicine), an antifibrotic small molecule inhibitor, for treating patients with idiopathic pulmonary fibrosis, according to a press release from the drug’s manufacturer. Read more.

Q&A: Navigating pregnancy with interstitial lung disease

Because interstitial lung disease is often associated with an older patient population, there is limited knowledge on how ILD impacts young women who are pregnant, according to a review published in CHEST. Read more.

Air pollution raises mortality risk, worsens lung function in fibrotic ILD

In patients with fibrotic ILD, exposure to small particulate matter was linked to increased mortality, poor lung function and faster disease progression, according to a study in JAMA Internal Medicine. Read more.

X-ray technology creates clearer picture of lung changes that occur in long COVID

With advanced X-rays and blood biomarkers, researchers have found how lung tissue scarring in patients with COVID-19 and pulmonary fibrosis forms a distinctive form of fibrotic ILD, according to a study published in eBioMedicine. Read more.

FDA grants orphan drug designation to ifenprodil for IPF

The FDA granted orphan drug designation to ifenprodil (NP-120, Algernon Pharmaceuticals) for the treatment of patients with IPF, according to a press release from the drug’s manufacturer. Read more.

Patients with IPF who experience in-hospital cardiac arrest face increased mortality rate

IPF appeared associated with increased mortality rates and worse outcomes among in-hospital cardiac arrest survivors, according to study results presented at the CHEST Annual Meeting. Read more.

Q&A: Investigational therapy shows potential for treating patients with IPF

Saracatinib appeared to be “equal or superior” in stopping fibrotic responses compared with two FDA-approved antifibrotic drugs, according to a preclinical study published in American Journal of Respiratory and Critical Care Medicine. Read more.

Novel screening tool enables earlier diagnosis of IPF

A new screening tool, the zero-burden comorbidity risk score for IPF, led to earlier diagnosis of IPF and improved outcomes of disease-modifying therapies and other interventions, according to published data in Nature Medicine. Read more.

References:

• Pulmonary Fibrosis Foundation: What is pulmonary fibrosis? https://www.pulmonaryfibrosis.org/understanding-pff/about-pulmonary-fibrosis/what-is-pulmonary-fibrosis. Accessed Feb. 23, 2022.
• PFF encourages patient advocacy and awareness on rare disease day. https://www.newswise.com/articles/pff-encourages-patient-advocacy-and-awareness-on-rare-disease-day?sc=dwhr&xy=10050145. Published Feb. 22, 2023. Accessed Feb. 23, 2023.