According to a publication from Karolinska Institutet, a medical research university near Stockholm, Sweden, new research led by the University suggests that regulatory T cells have an especially important role in regulating gut immune health.
The study followed a number of patients with the rare “immune dysregulation, polyendocrinopathy, enteropathy, X-linked” (or IPEX) sydrome. Individuals with this condition lack regulatory T cells. T cells are one of two types of lymphocyte (the other being B cells), a form of white blood cell that plays an important role in destroying present antigens. Regulatory T cells, as the name implies, control the extent of said immune reaction – usually acting to prevent lymphocytes from attacking healthy tissue.
About IPEX
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked, or IPEX syndrome, is a condition where individuals affected develop multiple autoimmune disorders. Autoimmune disorders are a type of disease where the body’s defense systems mistakenly target healthy tissues as though they were pathogens. Symptoms and prognostic outlook can vary between various autoimmune disorders, but they’re often difficult to treat – many have limited, if any, existing treatment options.
Although the chances of developing an autoimmune disorder are slim (we are a rare disease website, after all), shockingly, almost 25% of people with one autoimmune disorder will develop more over time.
The parts of the body most commonly affected by IPEX-linked autoimmune conditions are the skin, intestines, and endocrine glands. Most people with IPEX will develop an autoimmune disorder of their intestines called autoimmune enteropathy, characterized by immune attacks on healthy cells in the digestive tract. Many will develop eczema-linked dermatitis as well; and type 1 diabetes remains the most prevalent endocrine disorder among affected individuals.
Symptoms of IPEX and its related health conditions typically present in early childhood – even infancy. If left untreated, IPEX is usually fatal within the first year of life.
Regulatory T Cells Linked to Gut Immune Health
Treating IPEX is especially challenging because there are so many ways it can manifest – no two cases are exactly alike.
The Karolinska Institutet’s research hoped to identify the immune system’s preferred cell targets in IPEX patients. By examining the activity of regulatory T cells throughout the body, the Swedish researchers found that intestinal epithelial proteins were being aggressively targeted by immune cells in almost all of their participants.
Because IPEX shows an especially strong propensity to lead to enteropathy, understanding the condition’s course of action in the gut is of particular concern to scientists. Enteropathy is also one of the most dangerous complications of IPEX – frequently, deaths linked to the condition are the result of diarrhea or malnutrition.
By identifying which proteins in the intestine are most commonly targeted by the immune system, the Karolinska Institutet has taken the first step in developing any new treatment – identifying a target. Only once researchers know which cells are being affected, and how, can they begin to formulate therapies that act on said cells. The research is an intriguing addition to our collective understanding of IPEX syndrome, and will doubtlessly spur new research in turn.
Why is it important for scientists to understand how a disease works? What are scientists looking for when identifying clinical targets? Patient Worthy wants to hear from you!
