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Jung, Eo, Park, Kim, Cho, Oh, Seo, Na, Lee, Uh, Kim, and Park: Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment
Case Report
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2006; 61(1): 74-79.

Published online: 31 July 2006

DOI: https://doi.org/10.4046/trd.2006.61.1.74

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment

Bo Yong Jung, M.D., Sung Jun Eo, M.D., Eun Seo Park, M.D., Young Tong Kim, M.D.1, Jae Sung Cho, M.D., Mi Hye Oh, M.D.2, Gi Hyun Seo, M.D., Joo Ock Na, M.D., Sang Do Lee, M.D.3, Soo Taek Uh, M.D., Yong Hoon Kim, M.D., Choon Sik Park

Department of Internal Medicine, University of Soonchunhyang College of Medicine, Korea.

1Department of Radiology, University of Soonchunhyang College of Medicine, Korea.

2Department of Diagnostic Pathology, University of Soonchunhyang College of Medicine, Korea.

3Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Address for correspondence: Joo Ock Na, M.D. Address: 23-20, Bong Myeong-Dong, Cheonan Hospital, Soonchunhyang University Collage of Medicine, Cheonan, 330-721, KOREA. Phone: +82-41-570-3666, Fax: +82-41-574-5762, juokna@schch.co.kr

Received 18 April 2006       Accepted 16 June 2006

Copyright © 2006 The Korean Academy of Tuberculosis and Respiratory Diseases

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.

Keywords: Fibrosing mediastinitis, Pulmonary hypertension, Steroid treatment

Figures and Tables

Figure 1
Initial Chest PA : Bulging and marginal irregularity of aorta & aortic arch
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Figure 2
Panel A&B: Initial Chest CT: diffuse soft tissue lesion in mediastinum, pulmonary artery, ascending aorta and aortic arch. wall margin of aortic arch and pulmonary artery is irregular. Panel C&D: After 3 month Steroid therapy, Marked decreased soft tissue in mediastinum, pulmonary artery, ascending aorta and aortic arch on follow-up chest CT
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Figure 3
Panel A: Initial lung perfusion scan: large perfusion defect on both upper lobe. Panel B: After 53 months Steroid therapy, Marked decreased large perfusion defect on both upper lobe.
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Figure 4
Microscopically, the lesion showed dense fibrosis with some inflammatory cells(H&E, × 100)
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References

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ORCID iDs

Choon Sik Park
https://orcid.org/http://orcid.org/0000-0003-2977-0255

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