Aravena Carlos, Almeida Francisco A, Mukhopadhyay Sanjay, Ghosh Subha, Lorenz Robert R, Murthy Sudish C, Mehta Atul C
Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA.
Department of Respiratory Diseases, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
J Thorac Dis. 2020 Mar;12(3):1100-1111. doi: 10.21037/jtd.2019.11.43.
Idiopathic subglottic stenosis (iSGS) is a fibrotic disease of unclear etiology that produces obstruction of the central airway in the anatomic region under the glottis. The diagnosis of this entity is difficult, usually delayed and confounded with other common respiratory diseases. No apparent etiology is identified even after a comprehensive workup that includes a complete history, physical examination, pulmonary function testing, auto-antibodies, imaging studies, and endoscopic procedures. This approach, however, helps to exclude other conditions such as granulomatosis with polyangiitis (GPA). It is also helpful to characterize the lesion and outline management strategies. Therapeutic endoscopic procedures and surgery are the most common management modalities, but medical therapy can also play a significant role in preventing or delaying recurrence. In this review, we describe the morphology, pathophysiology, clinical presentation, evaluation, management, and prognosis of iSGS.
特发性声门下狭窄（iSGS）是一种病因不明的纤维化疾病，可导致声门以下解剖区域的中央气道梗阻。该疾病的诊断困难，通常会延迟，且易与其他常见呼吸系统疾病混淆。即使经过全面检查，包括完整病史、体格检查、肺功能测试、自身抗体检测、影像学检查和内镜检查，仍无法确定明显病因。然而，这种检查方法有助于排除其他疾病，如肉芽肿性多血管炎（GPA）。它也有助于明确病变特征并制定管理策略。治疗性内镜手术是最常见的管理方式，但药物治疗在预防或延缓复发方面也可发挥重要作用。在本综述中，我们描述了iSGS的形态学、病理生理学、临床表现、评估、管理及预后。
