Parathyroid Endothelium

A New Look at the Pathophysiology of Idiopathic Hypoparathyroidism
  • Maria Luisa Brandi
  • Andrea Fattorossi


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


Idiopathic Pulmonary Fibrosis Parathyroid Gland Pernicious Anemia Human Skin Fibroblast Parathyroid Tissue 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Doniach, D., and Bottazzo, G. F., 1981, Polyendocrine autoimmunity, in: Clinical Immunology Update (E. C. Franklin, ed.), Elsevier, Amsterdam, p. 95.Google Scholar
  2. 2.
    Craig, J. M., Schiff, L. H., and Boone, J. E., 1955, Chronic moniliasis associated with Addison’s disease, Am. J. Dis. Child. 89:669–684.Google Scholar
  3. 3.
    Blizzard, R. M., Chee, D., and Davis, W., 1966, The incidence of parathyroid and other antibodies in the sera of patients with idiopathic hypoparathyroidism, Clin. Exp. Immunol. 1:119–128.PubMedGoogle Scholar
  4. 4.
    Lupulescu, A., Potorac, E., Pop, A., Heitmanck, S., Merculiev, E., Chisiu, N., Oprisan, R., and Neacsu, C., 1968, Experimental investigations on immunology of the parathyroid gland, Immunology 14:475–482.PubMedGoogle Scholar
  5. 5.
    Posillico, J. T., Wortsman, J., Srikanta, S., Eisenbarth, G. S., Mallette, L. E., and Brown, E. M., 1986, Parathyroid cell surface autoantibodies that inhibit parathyroid hormone secretion from dispersed human parathyroid cells, J. Bone Mineral Res. 1:475–483.CrossRefGoogle Scholar
  6. 6.
    Brandi, M. L., Aurbach, D. G., Fattorossi, A., Fattorossi, A., Quarto, R., Marx, S. J., and Fitzpatrick, L. A., 1966, Antibodies cytotoxic to bovine parathyroid cells in autoimmune hypoparathyroidism, Proc. Natl. Acad. Sci. USA 83:8366–8369.CrossRefGoogle Scholar
  7. 7.
    Irvine, W. J., and Scarth, L., 1969, Antibody to the oxyphil cells of the human parathyroid in idiopathic hypoparathyroidism, Clin. Exp. Immunol. 4:505–510.PubMedGoogle Scholar
  8. 8.
    Betterle, C., Caretto, A., Zeviani, M., Pedini, B., and Salvianti, C., 1985, Demonstration and characterization of anti-human mitochondria autoantibodies in idiopathic hypoparathyroidism and in other conditions, Clin. Exp. Immunol. 62:353–360.PubMedGoogle Scholar
  9. 9.
    Quarto, R., Brandi, M. L., Fitzpatrick, L. A., Fattorossi, A., and Aurbach, G. D., 1986, Antibodies to parathyroid cells in autoimmune hypoparathyroidism, in: Current Advances in Skeletogenesis (S. Hurwitz and J. Sela, eds.), Heiliger, Jerusalem, p. 230.Google Scholar
  10. 10.
    Sakaguchi, K., Santora, A., Zimering, M., Curcio, F., Aurbach, G. D., and Brandi, M. L., 1987, Functional epithelial cell line cloned from rat parathyroid glands, Proc. Natl. Acad. Sci. USA 84:3269–3273.PubMedCrossRefGoogle Scholar
  11. 11.
    Brandi, M. L., Ornberg, R. L., Sakaguchi, K., Curcio, F., Fattorossi, A., Lelkes, P.I., Matsui, T., Zimering, M., and Aurbach, G. D., 1990, Establishment and characterization of a clonal line of parathyroid endothelial cells, FASEB J. 4:3152–3158.PubMedGoogle Scholar
  12. 12.
    Brandi, M. L., Fitzpatrick, L. A., Coon, H. G., and Aurbach, G. D., 1986, Bovine parathyroid cells: Cultures maintained for more than 140 population doublings, Proc. Natl. Acad. Sci. USA 83:1709–1713.PubMedCrossRefGoogle Scholar
  13. 13.
    Banerjee, D. K., Ornberg, R. L., Youdim, M. B. H., Heldman, E., and Pollard, H. B., 1985, Endothelial cells from bovine adrenal medulla develop capillary-like growth patterns in culture, Proc. Natl. Acad. Sci. USA 82:4702–4706.PubMedCrossRefGoogle Scholar
  14. 14.
    Voyta, J. C., Via, D. P., Butterfield, C. E., and Zetter, B. R., 1984, Identification and isolation of endothelial cells based on their increased uptake of acetylated-low density lipoprotein, J. Cell Biol. 99:2034–2042.PubMedCrossRefGoogle Scholar
  15. 15.
    Fattorossi, A., Aurbach, G. D., Sakaguchi, K., Cama, A., Marx, S. J., Streeten, E. A., Fitzpatrick, L. A., and Brandi, M. L., 1988, Anti-endothelial cell antibodies: Detection and characterization in sera from patients with autoimmune hypoparathyroidism, Proc. Natl. Acad. Sci. USA 85:4015–4019.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1992

Authors and Affiliations

  • Maria Luisa Brandi
    • 1
  • Andrea Fattorossi
    • 2
  1. 1.Department of PhysiopathologyUniversity of Florence Medical SchoolFlorenceItaly
  2. 2.Immunology Unit, Medical DepartmentDASRSRomeItaly

Personalised recommendations