支气管色素沉着纤维化的临床表现、诊断与治疗

Clinical Manifestations, Diagnosis and Treatment of Bronchial Anthracofibrosis

摘要: 支气管色素沉着纤维化(Bronchial Anthracofibrosis, BAF)是一种少见且特征明显的气道疾病，其支气管黏膜色素沉着与周围组织纤维化，造成支气管狭窄、阻塞，严重影响患者呼吸功能与生活质量。BAF临床表现多样但缺乏特异性，常见症状有咳嗽、咳痰、呼吸困难、咯血等，多为慢性反复病程。好发于中老年人，女性发病率稍高，有长期吸烟、职业粉尘暴露及肺部疾病史者患病风险更高。支气管镜检查是确诊BAF的关键手段，而病理、影像学(如胸部X线、MSCT)、痰液、肺功能等检查虽能提供辅助信息，但均不具备确诊决定性价值，且目前整体诊断标准尚未统一。目前BAF在发病机制、诊断标准、治疗方案均存在问题，未来需深入研究发病机制，完善诊断标准，优化治疗方案，探索新疗法并加强长期随访。对BAF深入研究，有助于提升呼吸系统疾病诊疗水平，改善患者预后。

Abstract: Bronchial Anthracofibrosis (BAF) is a rare and distinctive airway disease with bronchial mucosal pigmentation and fibrosis of the surrounding tissues, resulting in bronchial stenosis and obstruction, which seriously affects the respiratory function and quality of life of patients. The clinical manifestations of BAF are varied, but lack of specificity, and the common symptoms include cough, sputum, dyspnea, hemoptysis, etc. It is often chronic and recurrent. Common symptoms include cough, sputum, dyspnoea, haemoptysis, etc. It is a chronic recurrent disease. BAF is prevalent in middle-aged and elderly people, with a slightly higher incidence in women, and the risk is higher in people with a history of long-term smoking, occupational dust exposure, and lung disease. Bronchoscopy is the key tool to confirm the diagnosis of BAF, while pathology, imaging (Chest X-ray, MSCT), sputum, pulmonary function and other tests can provide auxiliary information, but do not have the decisive value of confirming the diagnosis and the overall diagnostic criteria have not yet been unified. Currently, there are problems in the pathogenesis, diagnostic criteria, and treatment options of BAF, and in the future, in-depth study of the pathogenesis, improvement of diagnostic criteria, optimisation of treatment options, exploration of new therapies, and enhancement of long-term follow-up are needed. In-depth research on BAF can help to improve the diagnosis and treatment of respiratory diseases and improve the prognosis of patients.