Autoimmune polyglandular syndromes are rare disorders characterized by failure of several endocrine glands, as well as non-endocrine organs, associated ... APS type III shows normal adrenal function [4] and. APS type IV is a combination of ...
Gynecological Endocrinology, 2012; Early Online: 1–3 © 2012 Informa UK, Ltd. ISSN 0951-3590 print/ISSN 1473-0766 online DOI: 10.3109/09513590.2012.692222
Case Report
utoimmune polyglandular syndrome: an unusual presentation with A empty sella, premature ovarian failure, and Hashimoto’s thyroiditis associated with thyroid cancer Carolina Garcia Soares Leães, Maria Carolina Rios, Juliana Pereira Passaglia, Julia Fernanda Pereira-Lima & Miriam da Costa Oliveira
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Department of Neuroendocrinology, Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA), Porto Alegre, Brazil
Autoimmune polyglandular syndrome (APS) is a rare endocrine disorder of autoimmune etiology characterized by failure of at least two endocrine glands. APS types I and II are the most frequent of the four types of APS so far described, with a prevalence of 1/9000 and 1/20 000 inhabitants, respectively [1,2]. APS type I has been detected more commonly in children and adolescents. It is defined by a persistent fungal infection, hypoparathyroidism and adrenal insufficiency, and may be associated with other autoimmune diseases. APS type II affects young adults and is characterized by adrenal insufficiency, autoimmune thyroid disease and/or type 1 diabetes mellitus (DM1). Autoimmune oophoritis (AO) is more rarely detected in APS type II than in type I [3]. APS type III shows normal adrenal function [4] and APS type IV is a combination of autoimmune disorders that do not fall into the previously mentioned conditions. APS may be associated with other non-endocrine autoimmune diseases. Although patients with Hashimoto’s thyroiditis (HT), a possible component of APS, are 30% more likely to have differentiated thyroid carcinoma [5], there is no report of increased prevalence of thyroid cancer in patients with different presentations of APS. We report a case of APS type II, with clinical multiple gland involvement, associated with thyroid cancer and an empty sella.
irregular menstrual cycles, single delivery without complications at age 21 and amenorrhea at age 26. At that time, the patient was diagnosed with primary hypothyroidism and levothyroxine therapy was started. At age 37 years, she underwent total thyroidectomy in a foreign service, after detection of a 2 cm nodule in the thyroid during levothyroxine treatment. Multicentric papillary carcinoma was diagnosed, with cervical lymph node metastasis. Positive anti-thyroperoxidase antibodies (442 U/mL) and negative anti-thyroglobulin antibodies were detected. Complementary radioiodine therapy was performed (100 mCi) with endogenous thyroid-stimulating hormone (TSH) stimulation and post-therapeutic 131-I scan revealed a cervical area of radioiodine uptake. During follow-up, the patient reported headache and dizziness and magnetic resonance imaging (MRI) of the brain was performed. MRI scan showed an empty sella and the patient was then referred to our neuroendocrinology service. The patient denied other comorbidities and use of medications other than levothyroxine (150 mcg/day). She had a positive family history for thyroid cancer and type 2 diabetes mellitus and negative for early menopause or autoimmune diseases. Physical (1.61 m, 62 kg, BMI: 23.9 kg/m2) and neurological examination revealed no other abnormalities. Biochemical tests revealed impaired glucose tolerance (fasting glucose: 103 mg/dL; after 75 g glucose load: 187 mg/dL); mixed dyslipidemia: total cholesterol 264 mg/dL (
