Zaibi Haifa, Fessi Rana, Dhahri Besma, Ben Amar Jihen, Aouina Hichem
Faculty of Medicine of Tunis, University of Tunis El Manar, Tunisia, Charles Nicole Pulmonology Department, Tunis, Tunisia.
Tanaffos. 2019 Mar;18(3):272-275.
Tracheobronchopathia osteochondroplastica is a rare idiopathic disease of the trachea and the main bronchi, characterized by multiple submucosal osteocartilaginous nodules. Although the etiology of tracheobronchopathia osteochondroplastica remains unknown, several theories have been proposed. We report a case of a 47-year-old non-smoker woman with wheezing dyspnea over the past two years, which was treated as asthma without improvement. Investigations, including chest computed tomography scan, fiberoptic bronchoscopy, and endobronchial biopsy, indicated tracheobronchial amyloid light-chain (AL) amyloidosis. Thirteen years later, she was admitted for cough and wheezing. The bronchoscopic examination demonstrated nodular lesions distributed along the cartilaginous rings of the lower portion of the trachea and the main bronchi. Endobronchial biopsy confirmed tracheobronchopathia osteochondroplastica. We found tracheobronchopathia osteochondroplastica to be the end stage of amyloidosis.
骨软骨化生型气管支气管病是一种罕见的气管和主支气管特发性疾病，其特征为多个黏膜下骨软骨结节。尽管骨软骨化生型气管支气管病的病因尚不清楚，但已有多种理论提出。我们报告一例47岁不吸烟女性，在过去两年中出现喘息性呼吸困难，曾按哮喘治疗但无改善。包括胸部计算机断层扫描、纤维支气管镜检查和支气管内活检在内的检查显示为气管支气管轻链（AL）淀粉样变性。13年后，她因咳嗽和喘息入院。支气管镜检查显示结节性病变沿气管下部和主支气管的软骨环分布。支气管内活检确诊为骨软骨化生型气管支气管病。我们发现骨软骨化生型气管支气管病是淀粉样变性的终末期。
