成人Still病
Adult-onset Still disease
定义 英文原文(暂无中文)
A rare inflammatory multisystem disorder characterized clinically by four cardinal signs: fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash.
别名
Wissler-Fanconi综合征
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 成年期、老年期
- 患病率
- 1-9 / 1 000 000(France)
临床表型 59
极常见 99–80%14
- 关节疼痛 HP:0002829
- 关节痛/关节炎 HP:0005059
- 关节炎 HP:0001369
- C-反应蛋白水平升高 HP:0011227
- 红细胞沉降速度升高 HP:0003565
- 红斑 HP:0010783
- 疲乏 HP:0012378
- 发热 HP:0001945
- 血清铁蛋白升高 HP:0003281
- 白细胞增多症 HP:0001974
- 关节肿胀 HP:0001386
- 中性粒细胞增多症 HP:0011897
- 瘙痒 HP:0000989
- 皮疹 HP:0000988
常见 79–30%18
- 腹痛 HP:0002027
- 贫血 HP:0001903
- 虚弱 HP:0025406
- 碱性磷酸酶升高 HP:0003155
- 循环肝转氨酶水平升高 HP:0002910
- 全身性淋巴结肿大 HP:0008940
- 肝脏肿大 HP:0002240
- 科布纳现象 HP:6000933
- 淋巴结肿大 HP:0002716
- 斑丘疹样皮疹 HP:0040186
- 肌痛 HP:0003326
- 咽痛 HP:0033050
- 咽炎 HP:0025439
- 胸腔积液 HP:0002202
- 胸膜炎 HP:0002102
- 脾肿大 HP:0001744
- 一过性肺浸润 HP:0005828
- 体重减轻 HP:0001824
偶见 29–5%18
- 脂代谢异常 HP:0003119
- 厌食症 HP:0002039
- 抗核抗体阳性 HP:0003493
- 骨髓细胞减少 HP:0005528
- 软骨破坏 HP:0100773
- 循环醛缩酶浓度升高 HP:0012544
- 脑病 HP:0001298
- 噬血细胞作用 HP:0012156
- 肝炎 HP:0012115
- 间质性肺炎 HP:0006515
- 淋巴结炎 HP:0002840
- 心肌炎 HP:0012819
- 恶心 HP:0002018
- 胰腺炎 HP:0001733
- 心包炎 HP:0001701
- 蛋白尿 HP:0000093
- 呼吸窘迫 HP:0002098
- 类风湿因子阳性 HP:0002923
罕见 <4–1%6
- 淀粉样变 HP:0011034
- 结膜炎 HP:0000509
- 弥漫性血管内凝血 HP:0005521
- 巩膜外层炎 HP:0100534
- 非感染性脑膜炎 HP:0033430
- 葡萄膜炎 HP:0000554
排除 0%3
- 淋巴瘤 HP:0002665
- 脓毒症 HP:0100806
- 重症EB病毒感染 HP:0031693
近两年的全球研究 107L2
2024/08 起在 Europe PMC 检索所得,按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务,尚未引入。
- 2026-08Clinical Features and Outcome Measures Across Still Disease (Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still Disease) Cohorts Worldwide: A Systematic Literature Review
- 2026-07Acute Adult-Onset Still Disease Presenting as Fever of Unknown Origin and Severe Myalgia in a Resource-Limited Tertiary Hospital in Brazil
- 2026-07Examination of HLA-DRB1*15-linked Candidate Antigens in Still Disease With and Without Lung Disease and Drug-Associated Immune Reactions
- 2026-06综述病例报告开放获取Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review
- 2026-06开放获取Malignancy rates in children, young people and adults with JIA: an observational study using CPRD Aurum
- 2026-05Autoinflammatory syndromes in chronic urticaria are rare but clinically distinct: A retrospective cohort study
- 2026-05病例报告开放获取Severe Hyperferritinemia in Metabolic Dysfunction-Associated Steatotic Liver Disease With Normal Transferrin Saturation and Polyclonal Hypergammaglobulinemia Mimicking Iron Overload
- 2026-04病例报告开放获取Autoimmune Progesterone Dermatitis: A Case Report and Review of Literature
- 2026-04病例报告开放获取Severe Treatment-Refractory Macrophage Activation Syndrome: Hemophagocytic Lymphohistiocytosis Presenting With Neurologic Crisis in Systemic Lupus Erythematosus
- 2026-04开放获取The Role of Extracorporeal Membrane Oxygenation in Cardiopulmonary Dysfunction Due to Rheumatologic Diseases: A Systematic Review
- 2026-03开放获取Sex-based differences in penicillin allergy labeling among adults in a Canadian ambulatory urgent medicine clinic: a cross-sectional study
- 2026-03病例报告开放获取Case Report: Flagellate eruption of adult-onset still disease in an Afro-Caribbean woman: highlighting atypical cutaneous disease in richly pigmented skin
- 2026-03开放获取Proceedings of the 2<sup>nd</sup> Symposium "Autoimmune Diseases -Clinical Unmet Needs in Systemic Autoimmune Diseases Guide Clinical, Translational, and Basic Research"
- 2026-03开放获取Reliability and Construct Validity of the Physician's Global Assessment of Lung Disease in Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease
- 2026-03综述开放获取Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective
- 2026-02A Multifaceted Interplay Among Hemophagocytosis, Interleukin-18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases
- 2026-02A Case of Refractory Adult-Onset Still Disease With Macrophage Activation Syndrome and Pulmonary Alveolar Proteinosis
- 2026-02开放获取Concurrent Idiopathic Multicentric Castleman Disease with TAFRO and Sjögren's Syndrome in a Young Patient: A Challenging Diagnostic Overlap
- 2026-02Visualization analysis of the use of traditional Chinese medicine in the diagnosis and treatment of rare diseases in mainland China based on CiteSpace
- 2026-02系统综述Clinical Features and Outcome Measures Across Still Disease (Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still Disease) Cohorts Worldwide: A Systematic Literature Review
在中国开展的临床试验 3L2
按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实,并与主治医生商量。
登记为可入组 1
- 招募中NCT07191444A Study of Firsekibart Versus Anakinra in Adult-Onset Still's Disease中国研究中心 1 个:Shanghai
其他状态的试验(2 项)
- 状态未知NCT03651193Multi-center Registration Study of Adult Onset Still's Disease in Chinese Population中国研究中心 1 个:Hunan
- 进行中·不再招募NCT07151157Therapeutic Value of Sirolimus in Refractory Adult-Onset Still's Disease中国研究中心 1 个:Shanghai
中国境外的在招试验 7L2
这些试验在中国没有研究中心,通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处:看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生,或据此进一步查该药是否已在境外获批、是否有拓展性用药(expanded access)通道。
美国3法国2瑞士1加拿大1德国1意大利1荷兰1西班牙1土耳其1比利时1
CT.gov 报告命中 7 项,此处取回并展示最近的 6 项。
- 招募中NCT07517250A Study on the Use of Canakinumab Among Familial Mediterranean Fever and Still's Disease Patients瑞士
- 招募中NCT07203001A Phase II Trial to Evaluate the Clinical Efficacy, Safety and Tolerability of MAS825 in Pediatric and Adult Participants With Still's Disease加拿大、法国、德国、意大利、荷兰、西班牙、土耳其、美国
- 招募中NCT04402086Rheumatology Patient Registry and Biorepository美国
- 招募中NCT03510442Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still's Disease, and Related Conditions美国
- 招募中NCT05927454Acostill ( RaDiCo Cohort) (RaDiCo Acostill)法国
- 招募中NCT02143986Glycosylated Ferritin in Macrophagic Activation Syndromes比利时
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
- 中文病名:Orphanet 中文包,冻结于 2020-06-01
- 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)