New Cystic Fibrosis Medication Might Aid in the Treatment of Pneumonia - medtigo



New Cystic Fibrosis Medication Might Aid in the Treatment of Pneumonia

Share on facebook
Share on twitter
Share on linkedin
Share on whatsapp

According to a study published in Science Daily, pathogen-caused pneumonia, such as SARS-CoV-2 and pneumococcus, can be lethal. Fluid buildup in the airways can cause acute respiratory distress syndrome. The molecular pathways that lead to fluid buildup in the lungs have been discovered by researchers at Charité — Universitätsmedizin Berlin.

Consequently, they discovered the foundations for a novel therapy method: They speculated that a medicine already used to treat cystic fibrosis might also be effective in treating pneumonia, independent of the germs responsible for the condition, based on the hopeful findings of their lab research.   

The most common cause of restrictive lung disease is pneumonia. A medical disease known as pulmonary edema develops when the air sacs in the lungs fill up with fluid rather than air. Patients are having difficulty breathing owing to a shortage of oxygen. The symptoms were linked to acute respiratory distress syndrome.

“Even with the greatest medical technology and care, more than 40% of ARDS patients die in the ICU. The issue is that traditional medicines, such as antibiotics, antivirals, and immune-modifying medications, seldom produce significant enough improvement to justify continued usage.” Professor Dr. Wolfgang Kuebler, head of Charité’s Institute of Physiology and the study’s primary investigator, believes so.

“As a result, our methodology differed significantly from the literature. Rather than focusing on getting rid of the infection, we aimed to improve the natural defenses of the pulmonary vascular system.” This makes perfect sense, given that pulmonary edema induces lung swelling. The lungs’ veins dilate, allowing blood to flow into the surrounding tissue and fill the alveoli.  

But what exactly causes this reaction? What molecular processes exist beneath the surface in this case? Professor Kuebler headed a group of Charité researchers that set out to answer these concerns. The investigations employed lung cell, lung tissue, and lung organ cultures.

This study focused on the chloride channel CFTR, which is primarily present in the mucosal cells that line our airways. As a result, our mucus remains thin and drains more easily. Researchers have validated what has long been suspected: CFTR is expressed by cells lining pulmonary blood arteries. However, its expression is dramatically decreased in pneumonia.  

Researchers used an inhibitor to block the channel. They manipulated the number of chloride ions in the cells to explore what happens at the molecular level in the absence of CFTR in the pulmonary arteries.

They later employed immunofluorescence imaging, a type of diagnostic imaging: The study’s lead author, Dr. Lasti Erfinanda of the Institute of Physiology, noted that the researchers observed a metabolic cascade begun by CFTR inhibition that eventually led to permeable pulmonary blood vessels. “CFTR plays a significant role in the development of pulmonary edema.”  

The study discovered that in the absence of CFTR, cells would accumulate chlorine because chloride would no longer be evacuated. Excess chloride causes a chain reaction, resulting in an unregulated calcium influx into the cells via a calcium channel. According to Professor Kuebler, “the increasing calcium concentration then induces the vascular cells to contract.”   


“As a result, fluid might escape from the blood vessels via the interstices that develop between the cells. The pulmonary vascular barrier is strongly reliant on chloride channels working properly.”  

The study team altered their emphasis in answer to a concern about how to reduce or prevent the loss of chloride channels in the pulmonary arteries caused by pneumonia. For this aim, the scientists utilized a CFTR modulator, a medication previously used to treat cystic fibrosis. Because of a genetic defect, the CFTR chloride channel in the mucosal cells lining the airways is malfunctioning in people with cystic fibrosis.

According to Dr. Erfinanda, ivacaftor aids mucus clearance by increasing the chance of chloride channel opening. We suspected it might help the cells lining the pulmonary capillaries, so we undertook studies to find out.  

The chloride channels were more stable after ivacaftor treatment, with much less degradation than expected from the lungs’ inflammatory processes. Animal studies revealed that ivacaftor therapy increased survival rates, decreased lung damage, and resulted in considerably less severe symptoms and a much better overall condition when compared to placebo.

The experiment went so successfully that Professor Kuebler and his colleagues were taken aback. “We anticipate our findings will pave the way for clinical trials of CFTR modulators in persons with pneumonia. Even in the context of unknown diseases, this intriguing pathogen-independent treatment has the potential to help a large number of patients and prevent pneumonia from turning fatal.”

One Response

Leave a Reply


Free CME credits

Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.

Digital Certificate PDF

On course completion, you will receive a full-sized presentation quality digital certificate.

medtigo Simulation

A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.

medtigo Points

medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
  • Registration with medtigo = 10 points
  • 1 visit to medtigo’s website = 1 point
  • Interacting with medtigo posts (through comments/clinical cases etc.) = 5 points
  • Attempting a game = 1 point
  • Community Forum post/reply = 5 points

    *Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.

All Your Certificates in One Place

When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.

Our Certificate Courses