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• W2416400314 abstract "The onset and development of autoimmune diseases in humans is based on several factors which include both a strong genetic background as well as environmental factors. The latter act on the genetically predisposed host triggering the immune mechanism that produces the immunopathological diseases known as organ specific or non-organ specific ones1. A large bulk of evidences2-4 indicate that major histocompatibility complex genes play a pivotal role; these determinants act in association with several non major histocompatibility complex genes mostly involved in antigen processing and presentation and in the process of cell-tocell cooperation. Nevertheless, although such a large documentation suggests that autoimmune diseases have strong genetic bases, the precise definition of the genes involved in such a process is still lacking. Clinical cases characterized by a multiorgan involvement raise intriguing questions about the fine mechanism involved in the tolerance to self components and about the abnormalities that allow for autoimmune reactivity. The paper by Ghiringhelli et al.5 published in the present issue of Annali describes the clinical observations on a case of autoimmune disease involving different organs. The patient was affected by autoimmune thyroiditis, myasthenia gravis, polymyositis and alopecia and presented with this clinical picture following thymus removal because of a thymoma. The different organs (thyroid, muscle and skin) involved in the autoimmune pathology during a longtime interval, might lead to interpret in a unified manner all the conditions as a complex association of multiorgan autoimmunity, rather than as distinct situations. It may be hypothesized that the removal of the thymus compromised, in a patient already prone to autoimmunity, the capacity to avoid self reactivity. This observation acquires great importance in the light of the recent discovery of a gene located on chromosome 21 that can go through several and different mutation processes that correlate with a rare autosomal recessively inherited disease: autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) also known as autoimmune polyglandular syndrome (APS) type I. Endocrine APS is generally divided into two major sub" @default.
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• W2416400314 date "2003-05-13" @default.
• W2416400314 modified "2023-09-23" @default.
• W2416400314 title "Multiorgan autoimmune diseases: a single root for different leaves?" @default.
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