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老年发病的成人Still 病的临床特征。

Clinical features of elderly-onset Adult-onset Still's disease.

机构信息

Department of Rheumatology, Faculty of Medicine, Saga University, Saga, Japan.

Department of Rheumatology and Clinical Immunology, Jichi Medical University, Shimotsuke, Japan.

出版信息

Mod Rheumatol. 2021 Jul;31(4):862-868. doi: 10.1080/14397595.2020.1829340. Epub 2020 Oct 13.

Abstract

OBJECTIVES

To clarify the characteristics of patients with elderly-onset Adult-onset Still's disease (AOSD).

METHODS

Patients were classified into elderly-onset (>60 years: 47 patients) and younger-onset (≤60 years: 95 patients) groups according to their age at diagnosis of AOSD. Clinical features, treatments, and prognosis were compared between the elderly-onset and younger-onset groups.

RESULTS

In the elderly-onset group, compared with the younger-onset group, typical skin rashes were less frequent (21.3% vs 58.9%, respectively;  < .0001), whereas pleuritis (27.7% vs 7.4%, respectively;  = .0011) and disseminated intravascular coagulation (19.1% vs 2.1%, respectively;  = .0004) were more frequent, and serum ferritin levels were higher (median 12,700 ng/ml vs 2526 ng/ml, respectively;  < .0001). Overall survival and AOSD-related survival were reduced ( = .0006 and  = .0023, respectively) and drug-free remission was less frequent ( = .0035) in the elderly-onset group compared with the younger-onset group.

CONCLUSIONS

Our results demonstrated that elderly-onset AOSD patients had several characteristics that differed from younger-onset AOSD patients, including less typical skin lesions, more AOSD-related complications, higher ferritin levels, and poorer prognoses.

摘要

目的

阐明老年发病的成人Still 病(AOSD)患者的特征。

方法

根据 AOSD 的发病年龄,将患者分为老年发病组(>60 岁:47 例)和年轻发病组(≤60 岁:95 例)。比较老年发病组和年轻发病组之间的临床特征、治疗和预后。

结果

与年轻发病组相比,老年发病组中典型皮疹较少见(分别为 21.3%和 58.9%;<0.0001),而胸膜炎(分别为 27.7%和 7.4%;=0.0011)和弥散性血管内凝血(分别为 19.1%和 2.1%;=0.0004)更为常见,且血清铁蛋白水平更高(中位数分别为 12700ng/ml 和 2526ng/ml;<0.0001)。与年轻发病组相比,老年发病组的总生存率和 AOSD 相关生存率降低(=0.0006 和=0.0023),无药物缓解率较低(=0.0035)。

结论

我们的结果表明,老年发病的 AOSD 患者与年轻发病的 AOSD 患者存在一些不同的特征,包括不典型皮疹较少、AOSD 相关并发症更多、铁蛋白水平更高、预后更差。

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