Article
Immunology
Po-Ku Chen, Joung-Liang Lan, Po-Hao Huang, Jye-Lin Hsu, Ching-Kun Chang, Ni Tien, Hui-Ju Lin, Der-Yuan Chen
Summary: This study found that AOSD patients and severe COVID-19 patients have significantly higher levels of galectins and cytokines compared to healthy controls. Active AOSD patients showed elevated IFN-gamma and IL-17A compared to healthy controls. IL-18 is a key discriminator between active AOSD and severe COVID-19.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Ji-Won Kim, Mi-Hyun Ahn, Ju-Yang Jung, Chang-Hee Suh, Hyoun-Ah Kim
Summary: Neutrophils are crucial immune cells that defend against pathogens through mechanisms such as phagocytosis and formation of neutrophil extracellular traps (NETs). While NETs can kill pathogens, they may also cause toxic effects in hosts and trigger autoimmune responses in susceptible individuals, contributing to various inflammatory autoimmune diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Rheumatology
Jinchao Jia, Luyu Yang, Zhujun Cao, Mengyan Wang, Yuning Ma, Xiong Ma, Qiaoyan Liu, Jialin Teng, Hui Shi, Honglei Liu, Xiaobing Cheng, Junna Ye, Yutong Su, Yue Sun, Huihui Chi, Tingting Liu, Zhihong Wang, Liyan Wan, Chengde Yang, Qiongyi Hu
Summary: The study identified Lipocalin-2 (LCN2) as a potential biomarker for liver damage in adult-onset Still's disease (AOSD). LCN2 levels were found to be significantly elevated in AOSD patients compared to healthy controls, and were closely correlated with inflammatory markers and liver dysfunction. This suggests that LCN2 could be a useful tool for identifying AOSD patients with systemic inflammation, particularly those with liver involvement.
Article
Immunology
Yuning Ma, Mengyan Wang, Jinchao Jia, Jianfen Meng, Jialin Teng, Dehao Zhu, Hui Shi, Yue Sun, Yutong Su, Honglei Liu, Xiaobing Cheng, Junna Ye, Huihui Chi, Tingting Liu, Xia Chen, Liyan Wan, Zhuochao Zhou, Fan Wang, Dongyi He, Chengde Yang, Qiongyi Hu
Summary: This study found that type I interferons play important roles in the pathogenesis of adult-onset Still's disease through their promotion of neutrophil extracellular trap formation, characterized by enhanced release of oxidized mitochondrial DNA.
JOURNAL OF AUTOIMMUNITY
(2022)
Review
Medicine, General & Internal
Stephane Mitrovic, Bruno Fautrel
Summary: Adult-onset Still's disease (AOSD) is a complex systemic autoinflammatory disorder with diverse clinical manifestations and unpredictable courses. Clinical phenotypes can be classified based on symptom evolution or dominant clinical evolution, but these classifications are mainly based on case series rather than robust epidemiological studies.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Luana Andreea Macovei, Alexandra Burlui, Ioana Bratoiu, Ciprian Rezus, Anca Cardoneanu, Patricia Richter, Andreea Szalontay, Elena Rezus
Summary: Adult-onset Still's disease is a systemic inflammatory disorder with unknown etiology, characterized by symptoms such as high fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis. Treatment options include NSAIDs, corticosteroids, csDMARDs, and biological therapy targeting various inflammatory cytokines.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Jinchao Jia, Mengyan Wang, Jianfen Meng, Yuning Ma, Yang Wang, Naijun Miao, Jialin Teng, Dehao Zhu, Hui Shi, Yue Sun, Honglei Liu, Xiaobing Cheng, Yutong Su, Junna Ye, Huihui Chi, Tingting Liu, Zhuochao Zhou, Liyan Wan, Xia Chen, Fan Wang, Hao Zhang, Jingjing Ben, Jing Wang, Chengde Yang, Qiongyi Hu
Summary: In this study, researchers found that high ferritin levels not only indicate hyperinflammation, but also trigger neutrophil leukocyte activation and extracellular trap formation. They demonstrated that ferritin exposure increases Msr1 expression on neutrophil leukocytes and acts as a ligand to trigger the NET formation pathway. Targeting NETs or Msr1 could benefit patients with hyperferritinemic syndrome.
NATURE COMMUNICATIONS
(2022)
Review
Rheumatology
Yuning Ma, Jianfen Meng, Jinchao Jia, Mengyan Wang, Jialin Teng, Dehao Zhu, Chengde Yang, Qiongyi Hu
Summary: Adult-onset Still's disease is a rare auto-inflammatory disorder characterized by high fever, skin rash, polyarthritis, and other symptoms. Treatment options have expanded beyond traditional medications to include anti-cytokine biologic agents.
Review
Medicine, General & Internal
Stylianos Tomaras, Carl Christoph Goetzke, Tilmann Kallinich, Eugen Feist
Summary: Adult-onset Still's disease is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash, and elevated ferritin levels. Immune dysregulation plays a central role in the disease, involving pathogenic involvement of both arms of the immune system. Extensive research has led to a better understanding of AoSD and revolutionary advances in treatment.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Rheumatology
Karima Daghor-Abbaci, Nadia Ait Hamadouche, Chafia Dahou Makhloufi, Farida Mechid, Fifi Otmani, Mohamed Makrelouf, Amel Otmane, Nourredine Smail, Malika Boucelma, Fatma Zohra Aissat, Salima Lefkir-Teffiani, Bilel Bengana, Nadia Boukheris, Amar Tebaibia, Baya Taharbouchet, Soraya Ayoub, Brahim Benziane, Nadia Oumnia, Chafika Haouichet, Fella Hanni, Nazim Laraba, Djennete Hakem, Nacera Benfenatki, Abdelkrim Berrah
Summary: This study aimed to develop a new diagnostic algorithm for adult-onset Still's disease (AOSD). By conducting a nationwide case-control study, six markers associated with AOSD diagnosis were identified and a diagnostic algorithm was developed to assist physicians in the diagnosis.
CLINICAL RHEUMATOLOGY
(2023)
Article
Medicine, General & Internal
Khetam Ghannam, Jan Zernicke, Claudia Kedor, Joachim Listing, Gerd-R. Burmester, Dirk Foell, Eugen Feist
Summary: In a randomized controlled trial, the IL-1 beta inhibitor canakinumab showed significant effects on the cytokine profile of patients with Adult-onset Still's disease, with a clear association with clinical response. Concentrations of MRP 8/14, S100A12, IL-6 and IL-18 decreased significantly in the canakinumab group after 4 weeks of treatment. Comparing responders with non-responders to canakinumab, a decrease in MRP 8/14, IL-1RA, IL-18, and IL-6 was observed in responders at week 4, highlighting the impact of canakinumab on the cytokine levels in AOSD patients.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Immunology
Jianfen Meng, Yuning Ma, Jinchao Jia, Mengyan Wang, Jialin Teng, Hui Shi, Honglei Liu, Yutong Su, Junna Ye, Yue Sun, Xiaobing Cheng, Huihui Chi, Tingting Liu, Dehao Zhu, Zhuochao Zhou, Liyan Wan, Zhihong Wang, Fan Wang, Xin Qiao, Xia Chen, Hao Zhang, Zihan Tang, Chengde Yang, Qiongyi Hu
Summary: This study compared the cytokine and ferritin levels between severe COVID-19 and active AOSD patients, revealing significant differences in IL-6, IL-10, and ferritin levels, laying the groundwork for developing therapeutic strategies targeting the hyperinflammatory process in COVID-19.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Nicolas Poursac, Itsaso Odriozola, Marie-Elise Truchetet
Summary: Adult-onset Still's disease is a complex autoinflammatory disease with a multifactorial etiology. This review provides a detailed overview of the different complementary tests used in diagnosing and managing the disease, including their value in positive diagnosis, differential diagnosis, and screening for complications. A structured algorithm is proposed for clinical practice, and potential new complementary examinations are discussed.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Rheumatology
Mengzhu Zhao, Di Wu, Min Shen
Summary: A total of 187 patients with adult-onset Still's disease (AOSD) were studied, revealing a 7.5% prevalence of neurological involvement. Common symptoms included fever, rash, liver dysfunction, arthralgia/arthritis, and lymphadenopathy. Treatment strategies mainly consisted of high-dose corticosteroids and immunosuppressive agents, with a 100% clinical remission rate.
Article
Rheumatology
Helen L. Leavis, Paul L. A. van Daele, Catharina Mulders-Manders, Renee Michels, Abraham Rutgers, Elizabeth Legger, Marc Bijl, Elisabeth A. Hak, Wai-Kwan Lam-Tse, Femke Bonte-Mineur, Peter Fretter, Anna Simon, Pieter van Paassen, Marlies C. van der Goes, Marcel Flendrie, Ward Vercoutere, Antoine W. T. van Lieshout, Arjen Leek, Sebastiaan J. Vastert, Sander W. Tas
Summary: This study used a Delphi panel to reach consensus on diagnostic and treatment strategies for adult-onset Still's disease. The panel agreed on recommendations for diagnosis and therapy, including the use of biologics and reduction of glucocorticoid use. The results can serve as a basis for a treatment guideline.
