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. 1995 Mar;50(3):280–283. doi: 10.1136/thx.50.3.280

Sclerosing mediastinitis: a report on 18 cases.

T M Mole 1, J Glover 1, M N Sheppard 1
PMCID: PMC1021193  PMID: 7660343

Abstract

BACKGROUND--Sclerosing mediastinitis is a rare condition which causes dense fibrosis of the mediastinum. Few large studies have been reported to date. The clinical and pathological features of cases have been studied in a specialist referral centre in the UK. METHODS--The pathological files of the Royal Brompton Hospital were examined and 18 cases of sclerosing mediastinitis were identified between 1970 and 1993. The clinical notes were obtained and the pathological specimens analysed. RESULTS--There were 12 men and six women of age range 9-64 years. Twelve patients presented with shortness of breath, six had haemoptysis, three had hoarseness, four had pleuritic chest pain, three general weakness, two had dysphagia, and one was asymptomatic. Nine patients had a previous history of pulmonary tuberculosis. Two had auto-immune disease--one rheumatoid arthritis and the other systemic lupus erythematosus. There were three cases of previous malignancy--two undifferentiated carcinoma of the lung and the other Hodgkin's disease. Serological tests revealed only one positive reaction to Histoplasma. The erythrocyte sedimentation rate and serum immunoglobulins were raised in nine patients. Diagnosis was usually by thoracotomy with biopsy. All cases had fibrosis and chronic inflammation with no active granulomas. No infective organisms or positive cultures were obtained in any case. Ten of the 18 cases are alive up to 15 years after diagnosis, with only two deaths and six lost to follow up. CONCLUSIONS--Sclerosing mediastinitis is a slowly progressive condition associated with previous tuberculosis, mediastinal malignancy, and autoimmune disease. The outlook is excellent for those cases without underlying malignancy.

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Selected References

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  1. Bloomberg T. J., Dow C. J. Contemporary mediastinal tuberculosis. Thorax. 1980 May;35(5):392–396. doi: 10.1136/thx.35.5.392. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Comings D. E., Skubi K. B., Van Eyes J., Motulsky A. G. Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med. 1967 May;66(5):884–892. doi: 10.7326/0003-4819-66-5-884. [DOI] [PubMed] [Google Scholar]
  3. Dines D. E., Payne W. S., Bernatz P. E., Pairolero P. C. Mediastinal granuloma and fibrosing mediastinitis. Chest. 1979 Mar;75(3):320–324. doi: 10.1378/chest.75.3.320. [DOI] [PubMed] [Google Scholar]
  4. Goodwin R. A., Nickell J. A., Des Prez R. M. Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis. Medicine (Baltimore) 1972 May;51(3):227–246. doi: 10.1097/00005792-197205000-00008. [DOI] [PubMed] [Google Scholar]
  5. Graham J. R., Suby H. I., LeCompte P. R., Sadowsky N. L. Fibrotic disorders associated with methysergide therapy for headache. N Engl J Med. 1966 Feb 17;274(7):359–368. doi: 10.1056/NEJM196602172740701. [DOI] [PubMed] [Google Scholar]
  6. KUNKEL W. M., Jr, CLAGETT O. T., McDONALD J. R. Mediastinal granulomas. J Thorac Surg. 1954 Jun;27(6):565–574. [PubMed] [Google Scholar]
  7. Mitchell I. M., Saunders N. R., Maher O., Lennox S. C., Walker D. R. Surgical treatment of idiopathic mediastinal fibrosis: report of five cases. Thorax. 1986 Mar;41(3):210–214. doi: 10.1136/thx.41.3.210. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Schowengerdt C. G., Suyemoto R., Main F. B. Granulomatous and fibrous mediastinitis. A review and analysis of 180 cases. J Thorac Cardiovasc Surg. 1969 Mar;57(3):365–379. [PubMed] [Google Scholar]
  9. Strimlan C. V., Dines D. E., Payne W. S. Mediastinal granuloma. Mayo Clin Proc. 1975 Dec;50(12):702–705. [PubMed] [Google Scholar]
  10. Urschel H. C., Jr, Razzuk M. A., Netto G. J., Disiere J., Chung S. Y. Sclerosing mediastinitis: improved management with histoplasmosis titer and ketoconazole. Ann Thorac Surg. 1990 Aug;50(2):215–221. doi: 10.1016/0003-4975(90)90737-q. [DOI] [PubMed] [Google Scholar]

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