Polyglandular autoimmune syndrome type 3 with pituitary involvement in a Latin American patient

Abstract

Introduction: polyglandular autoimmune syndrome (PAS) is defined as the coexistence of at least two types of autoimmune endocrine diseases. Polyglandular autoimmune syndrome type 3 comprises autoimmune thyroid diseases and other autoimmune diseases not including Addison’s disease. Up until now, there was only one case report of PAS type 3 in Asia, coupled with isolated reduction of gonadotropin releasing hormone (GnRH) caused by pituitary disease.

Case presentation: we present the case of a woman in her 20s with a one-year history of Hashimoto disease and type 1 diabetes, who presented with controlled hypothyroidism and hyperglycemia, but experiencing secondary amenorrhea. Following the GnRH stimulation test, she was diagnosed with secondary amenorrhea attributed to possible autoimmune hypothalamitis and PAS type 3. Our patient’s HLA profile reported the DQB1*0201 allele and DRB1*0301 and *0803, DQ2 and DR8 gene sites.

Discussion: this is the first report in Latin American literature of PAS type 3 associated with pituitary involvement along with the other two diagnostic elements. A GnRH stimulation test is recommended for patients with PAS coupled with secondary amenorrhea which does not improve despite thyroid disease treatment. This case is anecdotal, with the description of this disease being relevant to our region