关键词: extra-articular manifestations primary sjogren’s syndrome recurrent pneumothorax ss-a & ss-b triple negative ss extra-articular manifestations primary sjogren’s syndrome recurrent pneumothorax ss-a & ss-b triple negative ss

来  源:   DOI:10.7759/cureus.26636   PDF(Pubmed)

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病,并伴有多种表现和疾病。我们讨论了一例60岁的女性,她向急性医学评估组提出抱怨进行性呼吸急促一个月,不伴有胸痛或下肢肿胀。她还报告了涉及手腕和近端指间(PIP)关节的关节疼痛,口腔干燥,脱发,和许多舌头溃疡。血液检查显示SS为三阴性,类风湿因子阴性,抗SSA和抗SSB,高红细胞沉降率(ESR),抗核抗体(ANA)滴度为640。对SS进行了诊断。然而,她的胸部CT显示左侧大量气胸;随后,紧急插入胸管。两天后取出胸管,胸部X射线(CXR)完全消失。然而,一周后,她出现了持续的复发性气胸,需要手术干预,随后导致完全恢复。气胸是与SS相关的极为罕见但潜在不利的并发症,到目前为止,文献中仅报道了2例,通常与潜在的肺部病理有关。
公众号