Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Sclerosing Mediastinitis

  • Luka BrcicEmail author
Reference work entry


Fibrosing mediastinitis; Idiopathic mediastinal fibrosis


Sclerosing mediastinitis, like granulomatous mediastinitis, is a part of the spectrum of fibrosing mediastinitis, with one important distinction, and, that is, that sclerosing mediastinitis is usually an idiopathic reaction to some autoimmune disorders, radiation, drugs (methysergide), and even foreign bodies if left in the mediastinum. Pronounced fibrosis is probably caused by aberrant response to inflammatory stimulus resulting in fibroproliferative reaction. It occurs in patients with obvious disturbances of immunological system, presenting as systemic lupus erythematosus, rheumatoid arthritis, Raynaud’s phenomenon, and also with idiopathic diseases like Riedel’s struma, sclerosing cholangitis, and retroperitoneal fibrosis. Goodwin et al. back in 1972 suggested that sclerosing mediastinitis results from a delayed hypersensitivity reaction to some antigen, which still today represents probably the best...

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References and Further Reading

  1. Athanassiadi, K. A. (2009). Infections of the mediastinum. Thoracic Surgery Clinics, 19, 37–45.CrossRefPubMedGoogle Scholar
  2. Divatia, M., Kim, S. A., & Ro, J. Y. (2012). IgG4-related sclerosing disease, an emerging entity: A review of a multi-system disease. Yonsei Medical Journal, 53, 15–34.CrossRefPubMedGoogle Scholar
  3. Flieder, D. B., Suster, S., & Moran, C. A. (1999). Idiopathic fibroinflammatory (fibrosing/sclerosing) lesions of the mediastinum: A study of 30 cases with emphasis on morphologic heterogeneity. Modern Pathology, 12, 257–264.PubMedGoogle Scholar
  4. Goodwin, R. A., Nickell, J. A., & Des Prez, R. M. (1972). Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis. Medicine (Baltimore), 51, 227–246.CrossRefGoogle Scholar
  5. Loyd, J. E., Tillman, B. F., Atkinson, J. B., & Des Prez, R. M. (1988). Mediastinal fibrosis complicating histoplasmosis. Medicine (Baltimore), 67, 295–310.CrossRefGoogle Scholar
  6. Peikert, T., Colby, T. V., Midthun, D. E., Pairolero, P. C., Edell, E. S., Schroeder, D. R., & Specks, U. (2011). Fibrosing mediastinitis: Clinical presentation, therapeutic outcomes, and adaptive immune response. Medicine (Baltimore), 90, 412–423.CrossRefGoogle Scholar
  7. Peikert, T., Shrestha, .B, Aubry, M.C., Colby, T.V., Ryu, J.H., Sekiguchi, H., Smyrk, T.C., Specks, U., Yi, E.S. (2012). Histopathologic overlap between fibrosing mediastinitis and IgG4-related disease. International Journal of Rheumatology, 2012, article ID 207056, 7 pages.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Institute of PathologyMedical University of GrazGrazAustria