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以肉芽肿性多血管炎为病因的隐匿性鼻窦综合征:一例报告

Granulomatosis With Polyangiitis as an Etiology of Silent Sinus Syndrome: A Case Report.

作者信息

Kramer Nicholas, Manthei Brandon, Speier Luke, Bigcas Jo-Lawrence M, Manthei Scott

机构信息

College of Osteopathic Medicine, Touro University Nevada, Henderson, USA.

Department of Otolaryngology-Head and Neck Surgery, Kerkorian School of Medicine at the University of Nevada Las Vegas, Las Vegas, USA.

出版信息

Cureus. 2024 May 31;16(5):e61442. doi: 10.7759/cureus.61442. eCollection 2024 May.

Abstract

Silent sinus syndrome (SSS) is a rare condition characterized by the collapse of the maxillary sinus and the sinking of the eye socket (enophthalmos). Only around 100 cases of SSS have been reported so far. The underlying cause of this condition is the chronic obstruction of the osteomeatal complex, which leads to sinus contraction. In this case, we present a novel finding linking SSS with granulomatosis with polyangiitis (GPA). The patient described is a 39-year-old male who was diagnosed with SSS after a prolonged period of sinus pressure, headaches, epistaxis, and generalized congestion. Additionally, the patient reported a significant autoimmune history, including a previous occurrence of ANCA-mediated glomerulonephritis. Surgical intervention revealed the presence of significant granulation tissue, while histopathological examination identified areas of necrosis, vasculitis, and multinucleated giant cells consistent with GPA. This finding was further supported by the detection of positive blood c-ANCA. This case is particularly noteworthy as it is the first reported instance of GPA causing SSS. It serves as an excellent example to illustrate the underlying pathophysiology of SSS.

摘要

寂静性窦综合征(SSS)是一种罕见病症,其特征为上颌窦塌陷和眼球内陷。迄今为止,仅报告了约100例SSS病例。该病症的根本原因是骨窦复合体的慢性阻塞,这会导致鼻窦收缩。在此病例中,我们呈现了一个将SSS与肉芽肿性多血管炎(GPA)相关联的新发现。所描述的患者为一名39岁男性,在经历了长期的鼻窦压痛、头痛、鼻出血和全身性充血后被诊断为SSS。此外,该患者有显著的自身免疫病史,包括既往发生的抗中性粒细胞胞浆抗体(ANCA)介导的肾小球肾炎。手术干预发现存在大量肉芽组织,而组织病理学检查确定了与GPA一致的坏死、血管炎和多核巨细胞区域。血液中抗蛋白酶3型抗中性粒细胞胞浆抗体(c-ANCA)呈阳性的检测结果进一步支持了这一发现。该病例尤为值得注意,因为它是首例报告的由GPA导致SSS的病例。它是阐释SSS潜在病理生理学的一个绝佳实例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0083/11214768/43d44cb5d28f/cureus-0016-00000061442-i01.jpg

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