Tuberc Respir Dis.  2006 Jul;61(1):74-79.

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment

• Jung BY
• Eo SJ
• Park ES
• Kim YT
• Cho JS
• Oh MH
• Seo GH
• Na JO
• Lee SD
• Uh ST
• Kim YH
• Park CS

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.

Keyword

Fibrosing mediastinitis; Pulmonary hypertension; Steroid treatment

MeSH Terms

Biopsy
Echocardiography
Fibrosis
Hypertension, Pulmonary*
Mediastinitis*
Mediastinum
Pulmonary Artery
Thoracotomy
Tomography, X-Ray Computed

Figure

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  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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