Purpose: In a substantial proportion of patients with thymoma, many different types of paraneoplastic syndromes are observed. The association between thymoma and autoimmune liver diseases, however, has been found in very few cases. Methods: We report the case of a 31-year-old man affected by autoimmune hepatitis associated with myasthenia gravis and thymoma, successfully treated with extended thymectomy. Results: The patient is free from neoplastic and hepatic disease 4 years after surgery. Eighteen months after thymectomy, an exacerbation of hepatitis was successfully treated with steroids. Conclusions: To the authors' knowledge, only 7 cases of myasthenia gravis associated with thymoma and autoimmune hepatitis have been reported in the English-language literature. The exact role of thymoma in immune-mediated hepatitis is unclear. It seems likely that thymoma-associated T-cell abnormalities, due to the presence of thymoma, may have a role in the development of this rare clinical triad of autoimmune hepatitis, thymoma and myasthenia gravis.

Autoimmune hepatitis : an uncommon presentation of thymoma / P. Mendogni, L. Rosso, D. Tosi, A. Palleschi, I. Righi, F. Minonzio, N. Fusco, M. Nosotti. - In: TUMORI. - ISSN 0300-8916. - 102(2016 Nov), pp. S40-S42.

Autoimmune hepatitis : an uncommon presentation of thymoma

L. Rosso;A. Palleschi;FUSCO, NICOLA;M. Nosotti
2016-11

Abstract

Purpose: In a substantial proportion of patients with thymoma, many different types of paraneoplastic syndromes are observed. The association between thymoma and autoimmune liver diseases, however, has been found in very few cases. Methods: We report the case of a 31-year-old man affected by autoimmune hepatitis associated with myasthenia gravis and thymoma, successfully treated with extended thymectomy. Results: The patient is free from neoplastic and hepatic disease 4 years after surgery. Eighteen months after thymectomy, an exacerbation of hepatitis was successfully treated with steroids. Conclusions: To the authors' knowledge, only 7 cases of myasthenia gravis associated with thymoma and autoimmune hepatitis have been reported in the English-language literature. The exact role of thymoma in immune-mediated hepatitis is unclear. It seems likely that thymoma-associated T-cell abnormalities, due to the presence of thymoma, may have a role in the development of this rare clinical triad of autoimmune hepatitis, thymoma and myasthenia gravis.
autoimmune; hepatitis; paraneoplastic syndrome; thymoma
Settore MED/21 - Chirurgia Toracica
12-apr-2016
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/400497
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