Idiopathic hypoparathyroidism (IHP) is a metabolic disorder characterized by deficient secretion of parathyroid hormone (PTH) and consequent hypocalcemia. Although most cases are sporadic, familial occurrence of IHP has been reported as well, with no preference for age or sex. The rarity and heterogeneity of the disease gave rise to different classifications of the various forms of IHP. This is also the explanation given for the relative paucity of information on the mechanisms that underlie PTH deficiency. Autoimmune mechanisms have been suggested to be pathogenetically instrumental in IHP, even though the disease seems to be a very rare condition in comparison with other autoimmune endocrine failures. An autoimmune basis for IHP is supported by several clinical, histological, serological, and experimental observations: (1) IHP has been described in association with Addison’s disease, primary hypothyroidism, type I diabetes, malabsorption syndromes, primary hypogonadism, vitiligo, pernicious anemia, and alopecia. All these entities are believed to be of autoimmune origin or associated with autoimmune phenomena. The presence of two of the triad of Addison’s disease, hypoparathyroidism, and chronic mucocutaneous candidiasis is recognized as an autosomal recessive trait, the type I polyglandular autoimmune syndrome (Type I PGA).1 (2) Histological examination in IHP has shown atrophy of the parathyroid glands with lymphocytic infiltration.2 (3) Antiparathyroid antibodies occurred in 38% of pediatric patients with IHP using an indirect immunofluorescence assay.3 (4) Inoculation of homologous parathyroid tissue induced isoimmune hypoparathyroidism.4 (5) Antibodies directed against cell surface antigens inhibited PTH secretion in IHP.5
KeywordsIdiopathic Pulmonary Fibrosis Parathyroid Gland Pernicious Anemia Human Skin Fibroblast Parathyroid Tissue
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