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成人Still 病患者以复发性胸腔和心包积液为表现的不常见表现。

An Unusual Presentation of Adult-Onset Still's Disease in a Patient with Recurrent Pleural and Pericardial Effusions.

机构信息

Department of Medical Education, Saint Michael's Medical Centre, New York Medical College, Newark, NJ, United States.

Department of Medical Education, Saint Michael's Medical Centre, New York Medical College, Newark, NJ, United States.

出版信息

Am J Med Sci. 2021 May;361(5):655-658. doi: 10.1016/j.amjms.2020.06.024. Epub 2020 Jun 27.

Abstract

Adult-Onset Still's Disease (AOSD) usually presents with a salmon-colored skin rash and arthralgias. However, it can also be present with pleural and pericardial effusions. These effusions are often misdiagnosed as having an infectious etiology because AOSD usually present with fever, leukocytosis, elevated inflammatory markers, procalcitonin and CRP. There is usually a delay in giving steroids until the exclusion of all infectious etiologies, including extensive workups. Herein, we present a case report of AOSD in a patient with recurrent pleural and pericardial effusions, with no skin rashes or joint pain. Patient initially presented with fever, pleural and pericardial effusions, which was then treated as pneumonia with parapneumonic effusions. Patient returned for the second time with shortness of breath, productive cough, and fever, with no resolutions of pleural and pericardial effusions. Patient was found to have an extremely high ferritin levels, whereby a diagnosis of AOSD was made after excluding infection, malignancy and other rheumatological disorders based on the Yamaguchi criteria. AOSD is a rare disease with unusual presentation and diagnosis is often delayed. This case aimed to raise awareness among physicians of the multifaceted presentation of AOSD.

摘要

成人Still 病(AOSD)通常表现为鲑鱼色皮疹和关节痛。然而,它也可能伴有胸腔和心包积液。这些积液常常被误诊为具有感染性病因,因为 AOSD 通常表现为发热、白细胞增多、炎症标志物升高、降钙素原和 CRP 升高。在排除所有感染性病因(包括广泛的检查)之前,通常会延迟给予类固醇。在此,我们报告了一例 AOSD 患者的病例,该患者反复出现胸腔和心包积液,但无皮疹或关节痛。患者最初表现为发热、胸腔和心包积液,随后被误诊为肺炎合并类肺炎性胸腔积液。患者第二次因呼吸困难、咳痰和发热返回,胸腔和心包积液仍未消退。患者的铁蛋白水平极高,根据 Yamaguchi 标准,在排除感染、恶性肿瘤和其他风湿病后,诊断为 AOSD。AOSD 是一种罕见疾病,其表现形式不典型,诊断常常延迟。本病例旨在提高医生对 AOSD 多方面表现的认识。

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