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

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment

Affiliations
  • 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.

Keyword

Fibrosing mediastinitis; Pulmonary hypertension; Steroid treatment

MeSH Terms

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

Figure

  • Figure 1 Initial Chest PA : Bulging and marginal irregularity of aorta & aortic arch

  • 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

  • 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.

  • Figure 4 Microscopically, the lesion showed dense fibrosis with some inflammatory cells(H&E, × 100)


Reference

1. Goodwin RA, Nickell JA, des Perez RM. Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis. Medicine. 1972. 51:227–246.
2. Ernest NA, Bacos JM, Macher AM, Marsh HB, Savage DD, Fulmer JD, et al. Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Am J Med. 1977. 63:634–643.
3. Kittredge RD, Nash AD. The many facets of sclerosing fibrosis. Am J Roentgenol Radium Ther Nucl Med. 1974. 122:288–298.
4. Sherrick AD, Brown LR, Harms GF, Myers JL. The radiographic findings of fibrosing mediastinitis. Chest. 1994. 106:484–489.
5. Levine MR, Kaye L, Mair S, Bates J. Multifocal fibrosclerosis: report of a case of bilateral idiopathic sclerosing pseudotumor and retroperitoneal fibrosis. Arch Ophthalmol. 1993. 111:841–843.
6. Kim KH, Kim HC, Chung MP, Kim HJ, Lee KS, Han JH, et al. Two cases of fibrosing mediastinitis caused by tuberculosis. Tuberc Respir Dis. 1997. 44:1146–1157.
7. Park KS, Jee HO, Park YK, Kim KH. Superior vena caval syndrome: report of a case. Korean J Thorac Cardiovasc Surg. 1979. 12:140–150.
8. Ko WO, Kim GH, Kim YS, Kim SW, Park SK, Lee DP, et al. A case of chronic sclerosing mediastinitis. Tuberc Respir Dis. 1995. 42:231–237.
9. Sim JJ, In GH, Kim HG, Kang GH, Yu SH, Chul S, et al. Idiopathic fibrosing mediastinitis causing extensive fibrotic veno-occlusion with minimal mediastinal involvement. Tuberc Respir Dis. 2002. 52:278–282.
10. Berry DF, Buccigrossi D, Peabody J, Peterson KL, Moser KM. Pulmonary vascular occlusion and fibrosing mediastinitis. Chest. 1986. 89:296–301.
11. Loyd JE, Tillman BF, Atkinson JB, des Prez RM. Mediastinal fibrosis complicating histoplasmosis. Medicine. 1988. 67:295–310.
12. Weinstein JB, Aronberg DJ, Sagel SS. CT of fibrosing mediastinitis: findings and their utility. AJR Am J Roentgenol. 1983. 141:247–251.
13. Light AM. Idiopathic fibrosis of mediastinum: a discussion of three cases and review of the literature. J Clin Pathol. 1978. 31:78–88.
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