Wisłowska Małgorzata
Rheumatology Clinic, National Institute of Geriatrics, Rheumatology and Rehabilitation, 1 Spartanska Street, 02-637 Warsaw, Poland.
Biomedicines. 2024 Sep 10;12(9):2067. doi: 10.3390/biomedicines12092067.
Adult-onset Still's disease (AOSD) is a rare chronic autoinflammatory condition characterized by a spiking fever, arthritis, a rash, hepatosplenomegaly, lymphadenopathy, leucocytosis, and hyperferritinemia. It is sometimes accompanied by life-threatening complications like macrophage activation syndrome/hemophagocytic lymphohistiocytosis (MAS/HLH). Treatment options for AOSD include glucocorticoids (GCs), immunosuppressive drugs, biological medications, and Janus kinase (JAK) inhibitors. The features that differentiate MAS/HLH from AOSD are: in MAS/HLH, a different type of fever, which is persistent, a sharp decrease in the number of leukocytes and thrombocytes, a further increase in the level of transaminases and ferritin, significant hepatosplenomegaly, lymphadenopathy, symptoms of the central nervous system (CNS), disseminated intravascular coagulation (DIC) and hemophagocytosis in the bone marrow. This study aimed to evaluate the course of AOSD, which results in MAS/HLD.
Nine AOSD patients, four of whom developed MAS/HLH, were treated at the Rheumatology Clinic in the Central Clinical Hospital of the Ministry of Interior Affairs from 1 January 2015 to 15 March 2020 and at the Rheumatology Clinic in the National Institute of Geriatric, Rheumatology and Rehabilitation from 1 September 2021 to 1 March 2024. Medical history, clinical data, demographic data, laboratory data, imaging data, Hscore, and treatment data were collected.
All the patients with MAS and an Hscore above 150 recovered.
MAS/HLH requires rapid diagnosis as well as treatment with methylprednisolone pulses, cyclosporine A, and etoposide. When comparing patients who developed MAS/HLH with those who did not, possible risk factors were identified: the presence of pregnancy (two cases) and an aggressive course of AOSD. The Hscore is a useful tool for identifying patients with MAS/HLH.
成人斯蒂尔病（AOSD）是一种罕见的慢性自身炎症性疾病，其特征为高热、关节炎、皮疹、肝脾肿大、淋巴结病、白细胞增多和高铁蛋白血症。它有时会伴有危及生命的并发症，如巨噬细胞活化综合征/噬血细胞性淋巴组织细胞增生症（MAS/HLH）。AOSD的治疗选择包括糖皮质激素（GCs）、免疫抑制药物、生物制剂和Janus激酶（JAK）抑制剂。MAS/HLH与AOSD的区别特征在于：在MAS/HLH中，发热类型不同，为持续性发热，白细胞和血小板数量急剧减少，转氨酶和铁蛋白水平进一步升高，显著肝脾肿大、淋巴结病、中枢神经系统（CNS）症状、弥散性血管内凝血（DIC）以及骨髓噬血细胞现象。本研究旨在评估导致MAS/HLD的AOSD病程。
2015年1月1日至2020年3月15日在内政部中央临床医院风湿病诊所以及2021年9月1日至2024年3月1日在国家老年病、风湿病和康复研究所风湿病诊所对9例AOSD患者进行了治疗，其中4例发展为MAS/HLH。收集了病史、临床数据、人口统计学数据、实验室数据、影像学数据、Hscore和治疗数据。
所有Hscore高于150的MAS患者均康复。
MAS/HLH需要快速诊断以及用甲泼尼龙冲击、环孢素A和依托泊苷进行治疗。在比较发生MAS/HLH的患者与未发生者时，确定了可能的危险因素：妊娠（2例）和AOSD的侵袭性病程。Hscore是识别MAS/HLH患者的有用工具。
