The autoimmune regulator AIRE in thymoma biology: autoimmunity and beyond

J Thorac Oncol. 2010 Oct;5(10 Suppl 4):S266-72. doi: 10.1097/JTO.0b013e3181f1f63f.

Abstract

Thymomas are tumors of thymic epithelial cells. They associate more often than any other human tumors with various autoimmune diseases; myasthenia gravis is the commonest, occurring in 10-50% of thymoma patients, depending on the World Health Organization-defined histologic subtype. Most thymomas generate many polyclonal maturing T lymphocytes but in disorganized microenvironments Failure to induce self-tolerance may be a key factor leading to the export of potentially autoreactive CD4 progeny, thus predisposing to autoimmune diseases. Normally, the master Autoimmune Regulator promotes expression of peripheral tissue-restricted antigens such as insulin by medullary thymic epithelial cells and induction of tolerance to them. The failure of approximately 95% of thymomas to express autoimmune regulator is another feature potentially contributing to autoimmunity.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoimmune Diseases / etiology*
  • Autoimmune Diseases / pathology
  • Autoimmunity*
  • Humans
  • Thymoma / complications
  • Thymoma / immunology*
  • Thymoma / metabolism*
  • Thymus Neoplasms / complications
  • Thymus Neoplasms / immunology*
  • Thymus Neoplasms / metabolism*
  • Transcription Factors / metabolism*

Substances

  • APECED protein
  • Transcription Factors