Autoimmune Polyglandular Syndrome II in a Patient with Takotsubo's Cardiomyopathy

Presentation Number: SAT-0755
Date of Presentation: June 21st, 2014

Karla Cristina Borromeo Detoya*1, Daniela Ciltea2, Dylan Timberlake3 and Nairmeen A Haller4
1Akron General Medical Center, Cleveland Clinic Affiliate, Akron, OH, 2Akron Gen Prtnrs Physician Grp, Akron, OH, 3Northeast Ohio Medical University, 4Akron General Med Ctr, Akron, OH


Introduction: Autoimmune Polyglandular Syndrome (APS) is a rare condition involving multiple endocrinopathies and associated autoimmune disorders. It is preceded by adrenal failure in almost 50% of cases.  Takotsubo Cardiomyopathy (TCM) is a disorder with a poorly understood pathogenesis, leading to transient dysfunction of the middle segments/apex of the left ventricle and subsequent apical systolic dilatation.  We report a case of APS type II in a 65-year-old female with recent history of TCM. 

Clinical Case:  A 65-year-old female diagnosed with TCM based on cardiac catheterization consulted seven months later for progressive and chronic fatigue, hair loss, nail problems, insomnia, eczema and multiple hypopigmented patches.  She was being maintained on hormone replacement therapy for hypothyroidism (TSH at 33.3 uIU/mL, n 0.358- 3.74 uIU/mL; free T4 at 0.37 ng/dL, n 0.82-1.77 ng/dL).  Workup revealed thyroid peroxidase antibodies (51 IU/mL, n <35 IU/mL) present and was unremarkable for other autoimmune antibodies (anti-thyroglobulin, anti-tissue transglutaminase, anti-cardiolipin, anti-phosphatidylserine, anti-glutamic acid decarboxylase, anti-islet cell, and anti-21 alpha hydroxylase antibodies). ACTH (17 pg/mL, n 6-50 pg/mL) and DHEAS (75 ug/dL, n <146 ug/dL) were both normal. Despite the sensitivity of anti-21 alpha hydroxylase antibody testing, negative results have been documented in diagnosed cases of Addison’s disease. Thus, confirmatory cosyntropin stimulation test was performed and result suggested primary adrenal insufficiency (baseline cortisol at 1.7 ug/dL, 30 minute cortisol at 11.0 ug/dL, 60 minute cortisol at 13.6 ug/dL; n baseline cortisol at least 5 ug/dL, increase in 30 minute cortisol at least 7 ug/dL, 60 minute cortisol at least 18 ug/dL). Autoimmune Polyglandular Syndrome was  suspected based on symptomatology, laboratory findings of  hypothyroidism, primary adrenal insufficiency and recent clinical findings consistent with dermatomycosis.  The patient was then started on hydrocortisone, with good response to treatment.

Conclusion: This is a case of TCM with hypothyroidism,presenting with clinical and laboratory findings consistent with APS II.  Three other cases of APS with concomitant TCM have been reported.  While TCM is currently of unclear etiology, if related to APS it may be an early sign of the latter disease, as seen in this case.  One plausible theory argues that TCM may be triggered by potentiated catecholamine release in APS II due to ACTH stimulation.  Other authors cited possible hormonal preconditioning combined with the patient being in distress as factors contributing to the coexistence of these two diseases.  This case report supports the possibility that APS II and TCM may share common pathogenetic pathways which may include autoimmunity, hormonal factors, and genetic factors.  Further studies are recommended to establish correlation.


Nothing to Disclose: KCBD, DC, DT, NAH