Tang Nan, Tao Tao, Bao Xiao-Li
Department of Respiratory and Critical Care Medicine, Chongqing University Fuling Hospital, Chongqing, China.
AME Case Rep. 2024 Mar 27;8:49. doi: 10.21037/acr-23-160. eCollection 2024.
Fibrosing mediastinitis (FM) secondary to atypical sarcoidosis (atypical presentation of sarcoidosis) is rarely reported at home and abroad. Its clinical manifestations represent a lack of specificity, and the initial diagnosis is frequently difficult. In particular, this case has multiple pulmonary nodules with mediastinal lymph node enlargement and bilateral pleural effusion, and pulmonary fibrosis still exists after treatment, which is inconsistent with any clinical stage of pulmonary sarcoidosis, further increasing the diagnostic difficulty. We retrospectively analyzed the clinical data of a case of FM secondary to atypical sarcoidosis diagnosed by endobronchial ultrasound-guided cautery-assisted transbronchial mediastinal cryobiopsy (EBUS-CA-TBMCB) in Chongqing University Fuling Hospital, to improve clinicians' attention to FM and understand that EBUS-CA-TBMCB remains an effective way of etiological diagnosis.
A 70-year-old man was hospitalized with cough and dyspnea for two months. After admission, through chest computed tomography (CT), ultrasound guided bilateral lung biopsy, left parietal pleural biopsy, and EBUS-CA-TBMCB, the final diagnosis was atypical sarcoidosis secondary FM. After taking glucocorticoid orally, the patient's condition improved significantly, and was discharged from the hospital. We continued following up outside the hospital, and the patient's condition was further improved.
The diagnosis of FM is mainly based on typical imaging manifestations. When the contrast-enhanced chest CT finds localized or diffuse soft tissue density shadows around the mediastinum and pulmonary hilum with an irregular shape, with or without calcification, particular attention should be paid to exclude FM. EBUS-CA-TBMCB, as an improved minimally invasive method, can obtain enough tissue samples for pathological diagnosis, which may be the effective biopsy method for the etiology of FM to avoid missed diagnosis and misdiagnosis in the future.
非典型结节病（结节病的非典型表现）继发的纤维性纵隔炎（FM）在国内外鲜有报道。其临床表现缺乏特异性，初始诊断常常困难。特别是本病例存在多发肺结节、纵隔淋巴结肿大及双侧胸腔积液，治疗后仍有肺纤维化，不符合肺结节病的任何临床分期，进一步增加了诊断难度。我们回顾性分析了重庆大学附属涪陵医院1例经支气管内超声引导电灼辅助经支气管纵隔冷冻活检（EBUS-CA-TBMCB）诊断为非典型结节病继发FM的临床资料，以提高临床医生对FM的重视，并认识到EBUS-CA-TBMCB仍是病因诊断的有效方法。
一名70岁男性因咳嗽、呼吸困难2个月入院。入院后，通过胸部计算机断层扫描（CT）、超声引导下双侧肺活检、左顶叶胸膜活检及EBUS-CA-TBMCB，最终诊断为非典型结节病继发FM。口服糖皮质激素后，患者病情明显改善，出院。我们继续在院外随访，患者病情进一步好转。
FM的诊断主要基于典型的影像学表现。当胸部增强CT发现纵隔和肺门周围有局部或弥漫性软组织密度影，形态不规则，有无钙化时，应特别注意排除FM。EBUS-CA-TBMCB作为一种改良的微创方法，能够获取足够的组织样本进行病理诊断，可能是避免未来FM病因漏诊和误诊的有效活检方法。
