结核后纵隔纤维化致肺动脉高压1例并文献复习

Pulmonary hypertension complicating mediastinal fibrosis secondary to tuberculosis:A case report and literature review

回顾性分析中南大学湘雅二医院呼吸与危重症医学科收治的1例结核后纵隔纤维性致肺动脉高压患者的临床资料并进行相关文献复习。患者为汉族女性,55岁,于2013年8月因“活动后气促7个月”入院。入院检查发现红细胞沉降率增快,结核感染T细胞斑点试验阳性,双肺多发多形病变,双肺门及纵隔内多发淋巴结肿大并部分钙化,双肺多发支气管变窄,双侧胸膜增厚,胸腔镜检查发现左侧胸膜腔内少许粘连带。考虑可能存在结核感染,采用抗结核治疗4个月,患者的气促症状无改善。2016年再次入院检查,肺动脉CT造影提示双肺门区软组织密度影,内见钙化灶,病变压迫肺动脉及肺静脉,肺动脉主干增宽,右室增大,左肺静脉回流障碍,左侧胸腔积液。最终诊断为纵隔纤维化(结核感染所致)、继发肺动脉高压。纵隔纤维化是一种罕见的引起肺动脉高压的纵隔良性疾病,其临床表现缺乏特异性,早期诊断往往比较困难。当接诊原因不明的肺动脉高压患者时应特别注意排除纵隔纤维化,尽早行胸部增强CT检查,避免漏诊和误诊。

The clinical data for a patient with pulmonary hypertension complicating mediastinal fibrosis secondary to tuberculosis admitted to the Second Xiangya Hospital,Central South University has been retrospectively analyzed,and the relevant literature has been reviewed.A 55-year-old Han Chinese woman initially presented with increasing shortness of breath on exertion for 7 months was admitted to our hospital in August 2013.Admission examination revealed an increased erythrocyte sedimentation rate,positive in T-SPOT TB test,multiform lesions in both lungs,the enlarged lymph nodes with calcification in mediastinum and bilateral hilar,the narrowed bilateral main bronchial lumen,and the thickened bilateral pleural,as well as adhesion band in the left pleural cavity under the thoracoscope.These results indicated that mycobacterium tuberculosis infection may be present.After 4 months of anti-tuberculosis treatment,there is no improvement in symptom of the patient.In 2016,the patient was admitted to our hospital again because of shortness of breath after exercise.The computed tomography pulmonary angiography revealed illdefined soft tissue density lesions with calcification on bilateral hilar and mediastinum,encasing the major mediastinal vascular structures.The lesions compressed and wrapped the pulmonary artery and vein branches,resulting in their lumens narrowed severely.Moreover,the main pulmonary artery trunk was widened and the right ventricular was enlarged significantly.The patient was finally diagnosed as mediastinal fibrosis with the potential pathogenic mechanisms being tuberculosis,secondary pulmonary hypertension.Mediastinal fibrosis is an uncommon,benign and progressive condition characterized by an invasive proliferation of the fibrous tissue within the mediastinum,which can lead to pulmonary hypertension.Due to lack of specific clinical manifestations,diagnosis is difficult in the early stage.Physicians should pay attention to mediastinal fibrosis when accepting patients with unexplained pulmonary hypertension.Contrast-enhanced CT should be performed as early as possible to avoid mis-diagnosis or missed diagnosis.