A new pathogenesis of Still’s disease, a rare intractable disease, revealed

A study on the pathogenesis of ‘Still’s disease’, one of the very unfamiliar diseases to us, has been published.
Still’s disease, one of the rare and intractable autoimmune diseases, is called ‘Still’s disease (systemic juvenile idiopathic arthritis)’ before the age of 16 depending on the age at which it appears, and later patients are called ‘adult type Still’s disease’.
It is a rare disease with an incidence of 0.16-0.4 per 100,000 people. It causes inflammation in various parts of the body, such as fever, skin rash, joint and muscle pain, sore throat, liver enlargement, spleen enlargement, and lymph node increase of unknown cause, which drastically reduces the quality of life. open The pathogenesis is still unknown, and infection, immune abnormalities, and genetic factors are suspected.
Therefore, it is difficult to clearly distinguish diseases with fever, such as infections, malignant tumors, and other autoimmune diseases, and it is difficult to diagnose because there is no separate diagnostic method. However, there is a characteristic that the level of ferritin, an indicator showing the storage state of iron (Fe) in the blood, is significantly higher than normal.

In the midst of this, the team led by Professor Hyuna Kim and Jiwon Kim of the Department of Rheumatology at Ajou University Hospital confirmed that the pathogenesis of Still’s disease is related to ‘neutrophils’ and ‘NET’ through a review of various previously published papers, and confirmed the potential as biomarkers for diagnosis. presented and noted.
Neutrophils, which account for 40-70% of white blood cells in the human body, play an important role in removing pathogens that invade our body. It can cause autoimmune diseases and auto-inflammatory diseases in people with this high level.
In the process of viral hepatitis, bacterial hepatitis, or environmental triggers causing inflammation, excessive activation of neutrophils and NETs results in autoinflammation, which eventually causes immune cells that protect our body from bacteria or viruses to attack our body and cause inflammation. He explained that it appears that the disease, Still’s disease, appears to be developing.
Professor Kim Hyun-ah said, “The pathogenesis of Still’s disease is still not clear. However, this study confirms that neutrophils and NETs are related to the pathogenesis and is expected to help in the discovery of biomarkers for the development of new treatments and prognosis. do,” he said.
This study was published in the SCI journal ‘International Journal of Molecular Sciences’, ‘An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease’. Update on the pathogenic role of neutrophils).
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