6533b85dfe1ef96bd12be7ed

RESEARCH PRODUCT

The Autoimmune Regulator AIRE in Thymoma Biology: Autoimmunity and Beyond

Berthold SchalkePärt PetersonWilfred NixAlexander MarxPeter HohenbergerPhilipp StröbelHans-stefan HofmannKarsten WiebeHans HoffmannPhilipp A. SchnabelNick WillcoxJoachim PfannschmidtRalf Gold

subject

Pulmonary and Respiratory MedicineThymomaThymomamedicine.medical_treatmentAutoimmunitymedicine.disease_causeWorld healthAutoimmune DiseasesAutoimmunityAntigenAIREhemic and lymphatic diseasesAPS-1HumansMedicineMyasthenia gravisbusiness.industryInsulinThymus NeoplasmsAutoimmune regulatormedicine.diseaseMyasthenia gravisOncologyImmunologybusinessAPECEDTranscription Factors

description

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.

https://doi.org/10.1097/jto.0b013e3181f1f63f