Polyglandular syndrome (Schmidt syndrome) is associated with adrenocortical deficiency and which of the following conditions?
Ref: Harrison's Principles of Internal Medicine, IHed; Harrison's Online > Chapter 351. Disorders Affecting Multiple Endocrine Systems.Explanation:Immunologic Syndromes Affecting Multiple Endocrine OrgansWhen immune dysfunction affects two or more endocrine glands and other nonendocrine immune disorders are present, the polyglandular autoimmune (PGA) syndromes should be considered.The PGA syndromes are classified as two main types:Type I syndrome starts in childhood and is characterized by mucocutaneous candidiasis. hypoparathyroidism, and adrenal insufficiencyType II, or Schmidt syndrome is more likely to present in adults and most commonly includes adrenal insufficiency, thyroiditis, or type / diabetes mellitus & Graves diseaseFeatures of Polyglandular Autoimmune (PGA) SyndromesPGA I syndromePGA II syndrome (Schmidt syndrome)EpidemiologyAutosomal recessivePolvaenic inheritanceMutations in APECED geneHLA-DR3 and HLA-DR4 associatedChildhood onsetAdult onsetEqual male: female ratioFemale predominanceDisease Associations Mucocutaneous candidiasisAdrenal insufficiencyHypoparathyroidismHypothyroidismAdrenal insufficiencyGraves' diseaseHypogonadismAlopeciaType 1 diabetes HypoqonadnsmHypothyroidismHypophysitisDental enamel hypoplasiaMyasthenia gravisMalabsorptionVitiligoChronic active hepatitisAlopeciaVitiligoPernicious anemiaPernicious anemiaCeNac diseaseAPECED - Autoimmune polyendocrinopathy- candidiasis-ectodermal dystrophy.
Graves disease
Hypoparathyroidism
Hashimoto's thyroiditis
Islet cell adenoma
Miscellaneous (Endocrinology)