{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T17:03:15Z","timestamp":1790960595685,"version":"4.1.0"},"reference-count":156,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T00:00:00Z","timestamp":1661126400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>Adult-onset Still\u2019s disease (AOSD) is a rare disease affecting multiple systems and organs with unknown etiology, and the clinical symptoms are usually described as spiking fever, arthritis, evanescent salmon-pink eruptions, lymphadenopathy, splenomegaly, and other manifestations. The laboratory indicators are not specific, often presenting as increased leukocyte counts and neutrophil percentage, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), hyperferritinemia, and increased inflammatory factors. ANA, ENA, and RF are negative. According to those unspecific clinical presentations and laboratory findings, infection, tumor, connective tissue disease, and other diseases must be ruled out before diagnosis. The diagnosis of AOSD is a great challenge for clinicians. The mechanism of AOSD pathogenesis is complicated and still being studied. There is a new opinion that atypical persistent skin eruptions (APSEs) with specific histological manifestations are unique for AOSD, and APSEs might be on a spectrum with classical evanescent eruptions. Studies on APSEs showed that IL-1\u03b2 and IFN-\u03b3 are strongly correlated with the pathogenesis of necrosis keratinocytes in APSEs. IL-1\u03b2 is strongly involved in inflammatory disease when it is abnormal, and plays an important role in the pathogenesis of neutrophil dermatosis. In the early stage of AOSD, skin lesions appear to be evanescent urticaria-like eruptions accompanied by fever, and only neutrophils infiltrate around the blood vessels in the dermis pathologically. As the course of the disease progresses, IL-1\u03b2 is gradually released. Through the stimulation of other inflammatory factors and the influence of unknown factors, IL-1\u03b2 gradually infiltrates into the stratum corneum and finally accumulates around the necrotic keratinocytes of the stratum corneum. However, the detailed mechanism is still unknown. IFN-\u03b3 could play a pro-inflammatory or regulatory role in some disorders. IL-1\u03b2 can enhance the expression of IFN-\u03b3, and IFN-\u03b3 can cause keratinocyte apoptosis by activating the autocrine of caspase. Also, several pieces of evidence indicate that adaptive immunity is also involved in the pathogenesis of AOSD. Increased \u03b1-soluble receptors of IL-2 may suggest T-cell activation and proliferation in AOSD patients. Increased IL-4- and IFN-\u03b3-producing T cells were found in active AOSD and related to disease severity. Frequencies of Treg cells in AOSD were significantly lower and were inversely correlated with disease severity. According to these, more and more researchers have reached a consensus that AOSD is a disease at the crossroads of innate immunity and autoimmunity. In this review, we will provide a comprehensive insight into AOSD, describing research progress and the immunological mechanism contribution to the disease. In the meantime, different treatment options and the efficacy and safety of various biologic agents are also discussed. A further understanding of AOSD requires closer cooperation among doctors from different departments, and this review will provide a new idea for diagnosis and therapeutic options.<\/jats:p>","DOI":"10.3389\/fmed.2022.881431","type":"journal-article","created":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T09:22:47Z","timestamp":1661160167000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":41,"title":["Adult-onset Still\u2019s disease: A disease at the crossroad of innate immunity and autoimmunity"],"prefix":"10.3389","volume":"9","author":[{"given":"Shijia","family":"Rao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lemuel Shui-Lun","family":"Tsang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ming","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Shi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qianjin","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,8,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1136\/ard.30.2.121","article-title":"Still\u2019s disease in the adult.","volume":"30","author":"Bywaters","year":"1971","journal-title":"Ann Rheum Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1038\/s41584-018-0081-x","article-title":"Mechanisms, biomarkers and targets for adult-onset Still\u2019s disease.","volume":"14","author":"Feist","year":"2018","journal-title":"Nat Rev Rheumatol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.jaut.2018.07.018","article-title":"A comprehensive review on adult onset Still\u2019s disease.","volume":"93","author":"Giacomelli","year":"2018","journal-title":"J Autoimmun."},{"key":"B4","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1136\/ard.54.7.587","article-title":"Epidemiology of adult Still\u2019s disease: estimate of the incidence by a retrospective study in west France.","volume":"54","author":"Magadur-Joly","year":"1995","journal-title":"Ann Rheum Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"221","DOI":"10.2188\/jea.7.221","article-title":"Estimated prevalence and incidence of adult Still\u2019s disease: findings by a nationwide epidemiological survey in Japan.","volume":"7","author":"Wakai","year":"1997","journal-title":"J Epidemiol."},{"key":"B6","first-page":"818","article-title":"Epidemiology and outcome of adult-onset Still\u2019s disease in Northwestern Thrace region in Turkey.","volume":"33","author":"Balci","year":"2015","journal-title":"Clin Exp Rheumatol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1080\/03009740510026616","article-title":"Epidemiology and outcome of adult-onset Still\u2019s disease in Northern Norway.","volume":"35","author":"Evensen","year":"2006","journal-title":"Scand J Rheumatol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1399","DOI":"10.1007\/s00296-016-3546-8","article-title":"Epidemiological study of adult-onset Still\u2019s disease using a Japanese administrative database.","volume":"36","author":"Sakata","year":"2016","journal-title":"Rheumatol Int."},{"key":"B9","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1016\/j.semarthrit.2019.04.004","article-title":"Racial\/Ethnic variations in morbidity and mortality in adult onset Still\u2019s disease: an analysis of national dataset.","volume":"49","author":"Mehta","year":"2019","journal-title":"Semin Arthritis Rheum."},{"key":"B10","doi-asserted-by":"publisher","first-page":"250","DOI":"10.26444\/aaem\/132451","article-title":"Adult-onset Still\u2019s disease in Poland - 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