Adrenocortical Crisis Triggered by Levothyroxine in an Unrecognized Autoimmune Polyglandular Syndrome Type-2: A Case Report with Review of the Literature

Background: Autoimmune polyglandular syndrometype-2 (APS-2) is an uncommon endocrine disorder of Addison's disease with an autoimmune thyroid disorder and/or type 1 diabetes mellitus. The diagnosis is more challenging when a patient presents with nonspecific neuropsychiatric features with hypothyroidism in the setting of unrecognized Addison's disease.

Case report: We report a case of subclinical autoimmune hypothyroidism presented with nonspecific neuropsychiatric symptoms precipitated by stress. Despite levothyroxine treatment, her symptoms deteriorated and she was admitted with persistent vomiting and hypovolemic shock. Clinical features and laboratory parameters were suggestive of underlying adrenocortical insufficiency. Pre-existing autoimmune hypothyroidism combined with Addison's disease confirmed the diagnosis of unrecognized APS-2. She remarkably improved and her thyroid function tests also normalized with the treatment of corticosteroids only.

Review of the literature: We identified only five published case reports of our title by searching the database. Neufeld and Betterle have reported their data of APS-2 and concluded that a full-blown clinical picture of two or more components of the syndrome is like the tip of the iceberg.

Conclusion: The patients of one major component of APS-2 should be screened for other components of the disease to pick up latent cases. Addison's disease should be ruled out in patients of hypothyroidism who are intolerant to levothyroxine.

Keywords: Autoimmune polyglandular syndrome type-2; addison'scrisis; adrenocortical insufficiency; autoimmune thyroiditis; hypothyroidism; levothyroxine; schmidt's syndrome.