Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment.

Jung, Bo Yong; Eo, Sung Jun; Park, Eun Seo; Kim, Young Tong; Cho, Jae Sung; Oh, Mi Hye; Seo, Gi Hyun; Na, Joo Ock; Lee, Sang Do; Uh, Soo Taek; Kim, Yong Hoon; Park, Choon Sik

doi:2006.61.1.74

Tuberc Respir Dis > Volume 61(1); 2006 > Article

Case Report

Tuberculosis and Respiratory Diseases 2006;61(1):74-79.
DOI: https://doi.org/10.4046/trd.2006.61.1.74 Published online July 1, 2006.

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment.

Bo Yong Jung, Sung Jun Eo, Eun Seo Park, Young Tong Kim, Jae Sung Cho, Mi Hye Oh, Gi Hyun Seo, Joo Ock Na, Sang Do Lee, Soo Taek Uh, Yong Hoon Kim, Choon Sik Park

¹Department of Internal Medicine, University of Soonchunhyang College of Medicine, Korea. juokna@schch.co.kr
²Department of Radiology, University of Soonchunhyang College of Medicine, Korea.
³Department of Diagnostic Pathology, University of Soonchunhyang College of Medicine, Korea.
⁴Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Abstract

Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid therapy has been suggested for individuals with progressive symptoms, bu there is littlet data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to a compression of a main pulmonary artery by fibrosing mediastinitis which was confirmed by a biopsy with a thoracotomy. The chest CT scan and 2D echocardiography performed before and after a trial of steroid therapy demonstrated improvement after steroid therapy.

Key Words: Fibrosing mediastinitis, Pulmonary hypertension, Steroid treatment