Scientists have discovered what triggers a deadly lung disease

Researchers from Rutgers University have made an important discovery in understanding idiopathic pulmonary fibrosis (IPF) - a deadly disease that claims the lives of 80% of patients within ten years. Scientists have established that networks of incorrectly positioned immune cells play a key role in the development of the disease. This revolutionary discovery could pave the way for creating effective treatments for a disease that currently has virtually no cure.

The research results were published in the authoritative scientific journal European Respiratory Journal (ERS).

IPF is characterized by progressive scarring of lung tissue, which critically impairs breathing and leads to chronic oxygen deficiency. Modern medications have only minimal impact on the progression of the disease. Lung transplantation, although an option for some patients, is associated with high risk - half of operated patients do not survive to the five-year mark after transplantation.

In the new study, scientists applied innovative spatial mapping methods to comparatively analyze healthy lung tissues and samples obtained from patients with IPF. Researchers made an astonishing discovery: the lungs of patients are literally permeated with plasma cells - specialized immune cells that normally localize in bone marrow and are responsible for antibody production.

"The most surprising thing about our study is that absolutely all fibrotic areas of the lungs of patients with IPF are literally dotted with plasma cells," emphasized the lead author of the study, Qi Yang, a professor of pediatrics at the Robert Wood Johnson Medical School at Rutgers University. - "In healthy lungs, such cells are practically absent, but in patients with IPF, their concentration reaches colossal values."

The scientific team identified previously unknown cellular networks controlling this abnormal immune process. Researchers discovered new types of vascular wall cells that secrete specific signaling proteins, as well as unique fibroblasts that produce a protein that attracts plasma cells to affected areas of the lungs.

"This type of fibroblast has not been described in scientific literature until now," noted Reynold Panettieri, director of the Rutgers Institute for Translational Medicine and Science and co-author of the study. "It is known that fibroblasts cause the formation of scar tissue in the skin, lungs, and brain, but the discovered variety of cells appears to be characteristic exclusively of lung tissue."

After identifying the abnormal accumulation of plasma cells in the lung tissue of patients, the research team conducted a series of experiments on laboratory mice. The results showed that blocking certain signaling pathways significantly reduces the accumulation of plasma cells and significantly slows down the scarring process.

Particularly encouraging is the fact that drugs that affect plasma cells already exist and are used in clinical practice. Medications used for the treatment of multiple myeloma (a malignant neoplasm of plasma cells) could potentially be repurposed for the treatment of IPF.

This discovery gives hope to patients with IPF, for whom there are currently virtually no effective treatments. The disease predominantly affects men over 60 years of age, and most patients pass away within five years after diagnosis.

In the near future, scientists plan to determine whether the discovered plasma cells produce antibodies that attack healthy tissues, as well as to investigate in detail the mechanisms of formation of abnormal fibroblasts and vascular wall cells.