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A simple, yet promising treatment for pulmonary fibrosis

A group of researchers at the Institute for Biomedical Research of Barcelona is working on a pioneering treatment for idiopathic pulmonary fibrosis based on an innovative hypothesis: the transplant of type II pneumocytes, the cells that allow the lung to exchange gases

STAFF | February 11th, 2011


Currently, the only treatment that can be offered to patients with idiopathic pulmonary fibrosis (IPF) is a lung transplant because there are no effective drug treatments. But this situation could change if an experimental study that involves transplanting a cell type key to lungs’ ability to exchange gases proves to be successful.

"When we came up with the idea for this treatment, our first thought was that someone would have already tried it, it seemed so simple," said one of the researchers at the Institute of Biomedical Research of Barcelona of Spain’s National Research Council (IIBB-CSIC). This promising study that can be tracked from the science website Recerca en Acció (Research in Action).

To understand what pulmonary fibrosis is, one must first know how the lungs work and their structure. A simplified model is to imagine our lungs as two bags that fill with air when we inhale. The oxygen in the inhaled air is absorbed into the blood through the lung walls while the CO2 the body produces is expelled.

This exchange of gases between the blood and the lungs requires the maximum possible surface area and for the wall that separates the blood vessels from the air to be very thin. To achieve the maximum surface area, the lungs are comprised of small air sacs, called alveoli, which look similar to bunches of grapes, resulting in millions of small bags inside a larger one.

The study is based on a new, yet very simple idea: restoring the level of type II pneumocytes in the lungsThe lung wall is made thin by very flat and specialized cells, type I pneumocytes. Since these cells are highly specialized and cannot reproduce, the researchers decided to work with type II pneumocytes.

There are many other types of cells in the lungs apart from pneumocytes. There are some cells, called fibroblasts, which despite existing in small numbers are very important in the case of pulmonary fibrosis. These cells greatly increase their numbers in the event of an injury to repair the lungs, but they leave the lung wall thick with scar tissue that does not allow for gas exchange.

Occasionally, and for reasons still unknown, fibroblasts spread throughout the lung, making the lung walls thicker and transforming the lungs into more rigid and fibrous structures, hence the name pulmonary fibrosis.

While most attempts at treating the disease have focused on fibroblasts or helping the lung function despite their increased presence, the IIBB study is based on a new, yet very simple idea: restoring the level of type II pneumocytes in the lungs

The hypothesis put forward by the IIBB researchers is that type II pneumocytes will become type I pneumocytes, which will then retake their place on the lung wall and, thus, stop fibroblasts from proliferating. The lungs would not recover their initial state, but, if successful, the treatment would stop the disease from spreading throughout the lungs.
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