Polyglandular autoimune syndrome Type 2 – case report
Keywords:
Polyglandular autoimmune syndrome type 2, autoimmune thyroiditis, Addison diseaseAbstract
We describe the clinical case of a 32-year-old woman with the
previous diagnosis of thyroiditis, medicated with levothiroxin and
with family history of hipothiroidism (sister).
During the first trimester of pregnancy she begins the clinical
picture of worsening weakness, incapability of weight gain (3Kg
during whole pregnancy), alopecia and hyperpigmentation of
the skin and mucosal surfaces. These symptoms persisted after
delivery and weight loss become worse (about 20%). Hypotension was also noticed by this time. Laboratory tests showed
hyponatremia and hypocloremia, very high plasma ACTH levels
and very low levels of plasma cortisol and plasma aldosterona.
Other hormonal plasma levels were within normal range. The
computerized tomographies of the adrenals and of the pituitary
gland were also normal. The autoantibodies study revealed positive
anti-thyroid antibodies. Diabetes mellitus wasn’t present. After
hydrocortisone was started the clinical and laboratory changes described improved.
We discuss the polyglandular autoimmune syndrome type 2 in
the context of the reported clinical case, reminding the rarity of
presentation of symptoms during pregnancy.
Downloads
References
Baker JR. Autoimmune Endocrine Disease. JAMA 1987;278:1931-1937.
Spinner MW, Blizzard RM, Childs B. Clinical and genetic heterogeneity in idiopathic Addison’s disease and hypoparathyroidism. J Clin Endocrinol Metab 1968;28:795.
Neufeld M, Maclaren NK, Blizzard RM. Two types of autoimmune Addison’s disease associated with different polyglandular autoimmune syndromes. Medicine 1981 Sep; 60(5):355-362.
Chen QY, Kukreja A, Maclaren NK. The autoimmune polyglandular syndromes. In: De Croot LJ, Jameson JL. ed. Endocrinology 4 th edition. Philadelphia: WB Sauders Co 2001:587-599.
Dittmar M, Kahaly GJ. Polyglandular autoimmune syndromes: immunogenetics and long-term follow-up. J Clin Metab 2003; 88(7):2983-2992.
Betterle C, Volpato M, Creggion N, Presott F. Type 2 polyglandular autoimmune disease (Schimdt’s syndrome). J Pediatr Endocrinol Metab 1996 ;9 (1):113-123.
Betterle C, Dal Pra C, Mantero F, Zanchetta R. Autoimmune adrenal insuficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens and their applicability in diagnosis and disease prediction. Endocr Rev 2002;23:327-364.
Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. NEJM 2004;350:2068-2079.
Anderson JR, Goudie RB, Gray KG, Timbury GC. Autoantibodies in Addison’s disease. Lancet 1957;1:1123–1124.
Oelkers W. Adrenal Insufficiency. NEJM 1996;335:1206-1212.
Espinosa GM, Kuttothara A, Iturbide JA, Barronvallejo J. Successful course of pregnacy in a patient with polyglandular Schmidt’s syndrome treated with hormonal replacement therapy. Ginecol Obstet Mex 1998;66:55-56.
Gaither K, Wright R, Appuzzio JJ, Gittens L, Ganesh V. Pregnacy complicates by autoimmune polyglandular syndrome type II: a case report. J Matern Fetal Med 1998;7(3):154-156.
Betterle C et al. Assessment of adrenocortical function and autoantibodies in a baby born to a mother with autoimmune polyglandular syndrome type 2. J Endocrinol Invest. 2004 ;27(7):618-621.
Paiva I, Gomes L, Ribeiro C, Carvalheiro M, Ruas M.Hipofisite autoimune ou linfocítica. Acta Médica Portuguesa 2003; 16:459-464.
Namora J, Tomaz A, Raimundo L, Fernandes C, Ferreira J. Sindrome de Schmidt- a propósito de um caso. Medicina Interna 2004; 11:183-186
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna
Open Access
