成人史迪爾氏症在長期追蹤下的演變

The progression of adult onset Still’s disease during long-term follow-up

目的：為研究成人史迪爾氏症患者於追蹤期間的疾病演變，及討論成人史迪爾氏症與其他發炎性疾病的關係。方法：本研究回溯性搜尋台大醫院自1998年至2020年間的成人史迪爾氏症患者，並記錄病患的臨床特性及後續於追蹤期間獲得的其他相關診斷。結果：112名成人史迪爾氏症患者，追蹤時間中位數為32.9個月，其中5名患者被診斷為惡性腫瘤，包含一位淋巴癌、一位血癌、一位腦腫瘤、一位口腔上皮癌、及一位甲狀腺乳突上皮癌，而診斷的時間距離初次成人史迪爾氏症發病1.5個月至20.5個月不等。另有2名患者被診斷為類風濕性關節炎但缺乏特異性抗體。並無患者被診斷出任何潛隱性感染性疾病。結論：大部分的成人史迪爾氏症患者並不會演變為其他惡性疾病或風濕性疾病，在我們的病人族群中，僅有4.1%的病患演變為惡性疾病，且均發生於成人史迪爾氏症診斷後的2年內。儘管如此，成人史迪爾氏症仍可能是惡性腫瘤的前驅症狀，因此建議在診斷成人史迪爾氏症之後，維持至少2年的追蹤期，並注意是否有惡性疾病的徵兆。

Objective: To analyze the emergence of inflammatory diseases found during the follow-up period in patients diagnosed as adult onset Still’s disease (AOSD) at first, and to discuss whether the latter associated diseases were attributable to or associated with AOSD. Methods: We conducted a retrospective, case-series study of patients diagnosed as AOSD in NTUH registry from 1998 to 2020. Clinical characteristics and other diagnoses made during the follow-up period were recorded. Results: Of the 112 patients enrolled with a median follow-up of 32.9 months, five patients were diagnosed as malignancies, including one lymphoma, one leukemia, one brain tumor, one oral squamous cell carcinoma, and one thyroid papillary carcinoma after 1.5 to 20.5 months following the diagnosis of AOSD. Two patients were diagnosed as rheumatoid arthritis with the absence of specific antibodies. No occult infections considered attributable to AOSD were observed. Conclusions: Most of the patients with AOSD did not develop other rheumatic diseases or malignancies during the follow-up periods. The proportion of malignancy-associated AOSD was also low (4.1%), and all malignancies occurred within the first two years after AOSD. However, AOSD might still be a preceding event of hematologic malignancy or a solid tumor. Two years of follow-up with an awareness of malignancies was suggested for patients diagnosed as AOSD in the first place.