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Autoimmune Polyendocrine Syndrome Type 2: Pathophysiology, Natural History, and Clinical Manifestations

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Immunoendocrinology: Scientific and Clinical Aspects

Part of the book series: Contemporary Endocrinology ((COE))

Summary

Autoimmune Polyendocrine Syndrome Type 2 (APS-2) is the association of multiple organ-specific autoimmune diseases including but not limited to type 1 diabetes, Addison’s disease, and autoimmune thyroid disease. Additional autoimmune diseases are associated with APS-2, in particular celiac disease. The diseases share a common pathophysiology characterized by T-cell mediated destruction of the target organ. The component diseases of APS-2 have overlapping genetic risk factors. The natural history is characterized by a long preclinical phase, identifiable by the presence of organ-specific autoantibodies and normal gland function. Over time, destruction of the gland progresses and clinical disease develops. Treatment addresses the underlying hormonal abnormalities. Subjects with one autoimmune disease are at risk for the development of additional autoimmune diseases and therefore careful clinical and laboratory screening is required for follow-up.

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Acknowledgments

Dr. Barker is supported by the Juvenile Diabetes Research Foundation (JDRF) grant number 11-2005-15.

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  1. Pediatric Endocrinology, The Children’s Hospital, Aurora, CO, USA

    Jennifer M. Barker

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  1. Health Sciences Center, Children's Hospital, University of Colorado, East 9th Avenue 4200, Denver, 80262, Colorado, USA

    George S. Eisenbarth

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Barker, J.M. (2011). Autoimmune Polyendocrine Syndrome Type 2: Pathophysiology, Natural History, and Clinical Manifestations. In: Eisenbarth, G. (eds) Immunoendocrinology: Scientific and Clinical Aspects. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-478-4_9

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  • DOI: https://doi.org/10.1007/978-1-60327-478-4_9

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