“Strawberry tongue” is a characteristic symptom of Kawasaki Disease, an autoimmune disorder that, if not correctly diagnosed and treated, can result in heart damage in children and possible death.
For KD, a model T (cell)
Kawasaki Disease is a severe childhood illness that can, without treatment, result in damage to coronary arteries and, possibly, premature death. There is currently no diagnostic test for KD and standard treatment – a single infusion of intravenous immunoglobulin (IVIG) – is not without its problems and concerns.
KD, though, is self-limiting. That is, the body produces a type of cell called regulatory T cells or Treg that act to mitigate the inflammatory effects of KD and related damage to coronary arteries.
In a paper published in the journal Autoimmunity, first author Alessandra Franco,MD, PhD, an associate professor in the Department of Pediatrics at UC San Diego School of Medicine, Division of Allergy Immunology and Rheumatology and Rady Children’s Hospital-San Diego, and colleagues elucidate the role of Treg in KD and the mechanism for IVIG treatment.
The researchers have identified the underlying mechanism that explains why IVIG stimulates production of a particular type of Treg that recognizes the heavy constant region of antibodies and reduces inflammation and pediatric vasculitis of coronary arteries.
More broadly, Franco and colleagues say “natural Treg,” which is derived from the thymus during fetal development, has beneficial effects beyond KD. It also helps prevent plaque formation in atherosclerosis, a major form of heart disease.
The findings have significant clinical implications. IVIG is an expensive treatment. It is not available in many parts of the world where KD patients are common. “With current recombinant peptide technology, it should be possible to develop a stable, peptide-based therapeutic for testing as an optimized treatment,” Franco said. “Beyond KD, such a therapeutic may have applications in other vasculopathies, including atherosclerosis.”
You can read Franco’s paper, with co-authors Ranim Touma, Yali Song, Chisato Shimizu, Adriana H. Tremoulet, John T. Kanegaye and Jane C. Burns, all at UC San Diego, here.