支气管壁弥漫增厚伴多发结节样隆起

Diffuse thickening of bronchial walls with multiple nodular mucosal protrusions

本文报道1例58岁女性患者,因“鼻塞、咽痛、咳嗽6个月,憋气5个月”就诊。病史中有口干1年。体格检查发现牙齿片状脱落,腮腺、颌下腺及颈部淋巴结肿大,肺内啰音。腮腺造影显示分支导管不显影,末梢导管呈云雾状,排空迟缓。唇腺活检病理可见灶性淋巴浸润。胸部CT提示气管及双侧支气管管壁弥漫增厚,气管镜下气管、双侧支气管黏膜广泛结节。支气管镜活检可见支气管黏膜周围淋巴细胞浸润。患者被诊断为干燥综合征,并接受糖皮质激素治疗。泼尼松起始剂量为30 mg/d,逐渐减量。经糖皮质激素治疗后临床症状缓解,淋巴结、双侧颌下腺、腮腺肿大消退,CT提示支气管增厚改善,气管镜下黏膜多发结节样隆起消退,最终诊断为干燥综合征大气道受累。干燥综合征气道受累中,小气道受累如细支气管炎支气管扩张症很常见,但大气道受累,特别是气道内多发结节病例罕见。本例患者通过临床特征、影像学、支气管镜及病理特征,结合治疗反应确定了诊断。

A 58-year-old woman presented with a six-month history of nasal congestion,sore throat and cough,and a five-month history of dyspnea.She had a history of xerostomia for one year.On examination,the bilateral submandibular gland and parotid glands were enlarged.Parotid and anterior cervical lymph nodes were palpable.There were rales in both lungs.The rest of the physical examination was unremarkable.Sialographic analysis showed normal caliber in the main duct,stenosis in secondary ducts,and dilation in the proximal ducts.Minor salivary gland biopsy demonstrated periductal lymphocytic infiltration.Chest computed tomography(CT)showed diffuse thickening of the tracheal and bilateral bronchial walls.Bronchoscopy revealed macroscopic multiple nodules mainly in the trachea and bilateral main bronchus.Endobronchial biopsy showed lymphocytic infiltration in the bronchial submucosa.She was diagnosed with Sjögren′s syndrome and treated with glucocorticoids.The dose of prednisone was started at 30 mg/d and tapered gradually.Following treatment,the patient′s clinical condition improved dramatically,with shrinkage of the enlarged lymph nodes,bilateral submandibular and parotid glands.A repeated chest CT scan revealed improvement of the tracheal and bilateral bronchial thickening.Multiple nodules in the airway regressed,as evidenced by repeated bronchoscopic examination.The final diagnosis was a large-airway disease associated with Sjögren′s syndrome.Among airway diseases in Sjögren′s syndrome,peripheral airway diseases including bronchiolitis and bronchiectasis are common;however,central airway lesions in Sjögren′s syndrome,especially with macroscopic nodules,are rare.In this case,we demonstrated tracheal and endobronchial nodules in Sjögren′s syndrome as determined by clinical features,CT scan,bronchoscopy,and response to therapy.