Polyglandular Disorders

Polyglandular syndromes are disorders in which there is dysfunction and pathology of more than one endocrine gland. These may be either neoplastic syndromes, involving proliferation of various tissues and often a component of hyperfunction of endocrine glands, or they may be autoimmune syndromes in which immune destruction of tissues may result in loss of function. In general, these are genetic syndromes in which key growth regulatory genes (neoplastic disorders) or immune regulatory genes (autoimmune disorders) are mutated. Patients presenting with only one feature of these syndromes should be screened for the other features to ensure that all manifestations are treated and to enable screening of other family members or genetic counseling of the patient.