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三阴性干燥综合征与复发性气胸:自身免疫性疾病的罕见表现

Triple-Negative Sjogren's Syndrome and Recurrent Pneumothorax: An Uncommon Presentation of Autoimmune Disease.

作者信息

Imad Ziryab, Abdalla Yassin A, Hamza Salih B, Abubakr Mohammed E, Elneel Fathelrahman H, Mustafa Fatima M, Abdalgadir Sami A, William Jimmy

机构信息

Rheumatology, Haj Elsafi Teaching Hospital, Khartoum, SDN.

Internal Medicine, University of Bahri, Khartoum, SDN.

出版信息

Cureus. 2022 Jul 7;14(7):e26636. doi: 10.7759/cureus.26636. eCollection 2022 Jul.

DOI:10.7759/cureus.26636
PMID:35949775
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9356595/
Abstract

Sjogren's syndrome (SS) is a chronic exocrinopathy caused by lymphocytic infiltration and is associated with numerous manifestations and morbidities. We discuss a case of a 60-year-old female who presented to the Acute Medical Assessment Unit complaining of progressive shortness of breath for one month, not associated with chest pain or lower limb swelling. She also reported joint pain involving both wrists and proximal interphalangeal (PIP) joints, oral dryness, hair loss, and numerous tongue ulcerations. Blood workup revealed triple-negative SS, negative rheumatoid factor, anti-SSA and anti-SSB, a high erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer of 640. A diagnosis of SS was made. Nevertheless, her CT chest showed massive left-sided pneumothorax; subsequently, a chest tube was urgently inserted. The chest tube was removed two days later with complete resolution on chest X-ray (CXR). However, one week later, she presented with a recurrent pneumothorax that persisted and required surgical intervention that led to complete recovery afterward. Pneumothorax is an extremely rare but potentially unfavorable complication related to SS, with only two cases reported in the literature so far and usually associated with underlying lung pathology.

摘要

干燥综合征(SS)是一种由淋巴细胞浸润引起的慢性外分泌腺病,与多种临床表现和疾病相关。我们讨论一例60岁女性病例,该患者因进行性气短1个月就诊于急性医学评估单元,气短与胸痛或下肢肿胀无关。她还报告了双腕关节和近端指间关节(PIP)疼痛、口干、脱发以及多处舌溃疡。血液检查显示为三阴性SS,类风湿因子、抗SSA和抗SSB均为阴性,红细胞沉降率(ESR)升高,抗核抗体(ANA)滴度为640。确诊为SS。然而,她的胸部CT显示左侧大量气胸;随后紧急插入胸腔引流管。两天后拔除胸腔引流管,胸部X线(CXR)显示气胸完全消失。然而,一周后,她再次出现气胸且持续存在,需要手术干预,术后完全康复。气胸是与SS相关的一种极其罕见但可能不利的并发症,迄今为止文献中仅报道了两例,且通常与潜在的肺部病变有关。

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Combined seronegativity in Sjögren's syndrome.干燥综合征中的联合血清阴性
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