Załęska Monika, Błasińska-Przerwa Katarzyna, Oniszh Karina, Roszkowska-Śliż Barbara, Roszkowski-Śliż Kazimierz
Oddział XI Chorób Płuc, IGiCHP, ul. Płocka 26, 01–138, Warszawa, Poland.
Pneumonol Alergol Pol. 2013;81(3):273-80.
Fibrosing mediastinitis is a rare disease, mostly associated with previous granulomatous diseases, such as histoplasmosis, tuberculosis, or sarcoidosis. We present a case of 42-year-old woman with sarcoidosis, who had developed fibrosing mediastinitis and pulmonary hypertension. Contrast-enhanced computer tomography showed abnormal, bilateral, solid tissues surrounding the hila and mediastinum. Magnetic resonance imaging showed: abnormal, intermediate signal tissue in the mediastinum, surrounding hila, narrowing both lower lobe arteries and both lobe bronchi, left upper lobe atelectasis, and contrast enhancement of mediastinal infiltration. The patient was treated with steroids for 14 months (initial dose of prednisone was 1 mg/kg/day, then tapered) with clinical and radiological improvement. We reviewed the literature concerning fibrosing mediastinitis. The various causes of that disorder, the radiological manifestation, and possible treatment modalities are discussed. The causes of pulmonary hypertension in our patient are another aspect of the article. It could be associated with both the fibrosing mediastinitis and the sarcoidosis.
纤维性纵隔炎是一种罕见疾病,大多与既往肉芽肿性疾病相关,如组织胞浆菌病、结核病或结节病。我们报告一例42岁结节病女性患者,该患者并发了纤维性纵隔炎和肺动脉高压。增强计算机断层扫描显示双侧肺门和纵隔周围有异常实性组织。磁共振成像显示:纵隔、肺门周围有异常中等信号组织,双下叶动脉和双叶支气管变窄,左上叶肺不张,纵隔浸润有对比增强。该患者接受了14个月的类固醇治疗(泼尼松初始剂量为1mg/kg/天,随后逐渐减量),临床和影像学均有改善。我们查阅了有关纤维性纵隔炎的文献。讨论了该疾病的各种病因、影像学表现及可能的治疗方式。我们患者肺动脉高压的病因是本文的另一个方面。它可能与纤维性纵隔炎和结节病均有关。