Polyglandular syndromes type I, II, III (PDS) is characterized by sequential or simultaneous deficiencies in the function of several endocrine glands that have a common cause. Etiology (cause, set of causes) is most often autoimmune. Polyglandular deficiency syndromes (PDS) involve deficiencies in the function of several endocrine glands, which may occur simultaneously or sequentially. Nonendocrine organs also may be affected. Most cases are autoimmune; triggers are often unknown but may involve viruses or dietary substances. PDS is distinguished by the glands affected. Categorization depends on the combination of deficiencies, which fall within 1of 3 types. Diagnosis requires measurement of hormone levels and autoantibodies against affected endocrine glands. Treatment includes replacement of missing or deficient hormones and sometimes immunosuppressants. Risk factors for the development of autoimmunity include Genetic factors and Environmental triggers. Genetic factors include the AIRE gene mutation, which is causative of type 1, and certain HLA subtypes, which are important in the development of types 2 and 3. Environmental triggers include viral infections, dietary factors, and other as yet unknown exposures.
For type II, see also Schmidt syndrome« Back to Glossary Index