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Hypothalamitis: A Novel Autoimmune Endocrine Disease. A Literature Review and Case Report
Uğur Türe, Annamaria De Bellis, Mehmet Volkan Harput, Giuseppe Bellastella, Melih Topcuoglu, Cumhur Kaan Yaltirik, Paolo Cirillo, Rima Nur Yola, Aydın Sav, Fahrettin Kelestimur
The Journal of Clinical Endocrinology & Metabolism, Volume 106, Issue 2, February 2021, Pages e415–e429, [Ссылки доступны только зарегистрированным пользователям ]
Published: 26 October 2020 Article history

The relationship between the endocrine system and autoimmunity has been recognized for a long time and one of the best examples of autoimmune endocrine disease is autoimmune hypophysitis. A better understanding of autoimmune mechanisms and radiological, biochemical, and immunological developments has given rise to the definition of new autoimmune disorders including autoimmunity-related hypothalamic–pituitary disorders. However, whether hypothalamitis may occur as a distinct entity is still a matter of debate.

Evidence Acquisition
Here we describe a 35-year-old woman with growing suprasellar mass, partial empty sella, central diabetes insipidus, hypopituitarism, and hyperprolactinemia.

Evidence Synthesis
Histopathologic examination of surgically removed suprasellar mass revealed lymphocytic infiltrate suggestive of an autoimmune disease with hypothalamic involvement. The presence of antihypothalamus antibodies to arginine vasopressin (AVP)-secreting cells (AVPcAb) at high titers and the absence of antipituitary antibodies suggested the diagnosis of isolated hypothalamitis. Some similar conditions have sometimes been reported in the literature but the simultaneous double finding of lymphocytic infiltrate and the presence of AVPcAb so far has never been reported.

Conclusions
We think that the hypothalamitis can be considered a new isolated autoimmune disease affecting the hypothalamus while the lymphocytic infundibuloneurohypophysitis can be a consequence of hypothalamitis with subsequent autoimmune involvement of the pituitary. To our knowledge this is the first observation of autoimmune hypothalamic involvement with central diabetes insipidus, partial empty sella, antihypothalamic antibodies and hypopituitarism.