Autoimmune polyglandular syndrome: an unusual presentation with empty sella, premature ovarian failure, and Hashimoto’s thyroiditis associated with thyroid cancer

Leães, Carolina García Soares; Rios, Maria Carolina; Passaglia, Juliana; Pereira-Lima, Júlia Fernanda Semmelmann; Oliveira, Miriam da Costa

doi:10.3109/09513590.2012.692222

Cited by 4 publications

(7 citation statements)

References 16 publications

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“…Cases of papillary thyroid cancer in patients with APS including thyroiditis may prove interesting with this view, representing disease models with potential clues for scientific research. Besides the patient described by Karavanaki et al (1) and the case we described, we found in the literature another report suggesting the association between papillary thyroid cancer and APS type II (10) . Moreover, it is also worth pointing out that patients with APS type I/II have a nonsignificant increased mortality rate for thyroid cancer, approximately two-fold that expected in healthy subjects (11) .…”

Section: To the Editorsupporting

confidence: 68%

Papillary thyroid cancer and autoimmune polyglandular syndrome

Mussa

Matarazzo

Corrias

2015

Journal of Pediatric Endocrinology and Metabolism

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With great interest, we read the article by Karavanaki et al. (1) , who reported on a patient with autoimmune polyglandular syndrome (APS) type II and papillary thyroid carcinoma and highlighted the controversial relationship between thyroid autoimmunity and cancer. We take this opportunity to comment on this debated issue and to describe an overlapping case we have diagnosed and followed recently.In 2005, a 12-year-old boy was admitted at our institution for acute abdominal pain and dehydration; the presence of hyponatremia, hypoglycemia, and hyperkalemia prompted the suspicion of acute mineralocorticoid and glucocorticoid deficiency. Diagnostic tests revealed elevated adrenocorticotropic hormone concentrations ( > 1200 pg/mL) and undetectable serum cortisol levels, confirming a primary adrenal insufficiency of autoimmune origin (serum anti-21-hydroxylase antibody positive titer). Further investigations showed also the coexistence of euthyroid Hashimoto ' s thyroiditis; the boy had positive anti-thyroperoxidase ( > 126 U/mL, normal < 40) and anti-human thyroglobulin (888 U/mL, normal < 35) antibody titer and a mildly enlarged thyroid with decreased echogenicity and inhomogeneous ultrasound pattern. The diagnosis was consistent with APS type II; the patient was prescribed replacement therapy with oral hydrocortisone and fludrocortisone acetate and followed up quarterly. Of note, the recent patient history was positive for an episode of pharyngitis and one of severe gastroenteritis. Results of the laboratory assays were consistent with a previous streptococcal infection (anti-streptolysin O titer > 500 U/mL) and a recent hepatitis A infection (IgM index 3.14, positive > 1). A few months after the diagnosis of APS type II, the thyroid function switched to hypothyroidism with serum thyroid-stimulating hormone (TSH) level of 11.8 mU/L (0.4 -4.4 mU/L) and fT4 level of 9.6 pg/mL (9.8 -16.6 pg/mL); levothyroxine treatment was started. The follow-up of this case lasted until the actual age of 20 years; antibodies against transglutaminase, glutamic acid decarboxylase, tyrosine phosphatase-like molecule, gastric parietal cells, and cholinergic receptor were checked yearly and were persistently negative. Results of the twice-yearly clinical evaluation; levels of vitamin B, folate, calcium, phosphorus, parathormone, and glycemia; platelet counts; and liver enzyme dosages have always been normal, and the boy underwent regular growth and pubertal development. At 16 years of age, his annual thyroid ultrasound, which was also part of the follow-up, revealed a hypoechoic nodule at the right thyroid lobe with a maximum diameter of 15 mm, increased intravascular blood flow, and irregular margins. Fine-needle biopsy cytology was consistent with papillary thyroid carcinoma, and the boy underwent total thyroidectomy and central compartment lymph node dissection. Histology confirmed the cytological diagnosis, and the boy was submitted to radioactive iodine treatment. Currently, serum thyroglobulin levels are undetectable and there is...

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Section: To the Editorsupporting

confidence: 68%

Papillary thyroid cancer and autoimmune polyglandular syndrome

Mussa

Matarazzo

Corrias

2015

Journal of Pediatric Endocrinology and Metabolism

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“…In all cases in which APS II syndrome was associated with PTC, Hashimoto's thyroiditis was present. Interestingly, this is the third patient of Southern European origin reported (one from Greece and one from Italy already reported); one other patient comes from Brazil [2][3][4].…”

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confidence: 67%

“…It has been proposed that women with Hashimoto's thyroiditis are 30% more likely to have coexisting PTC compared with healthy controls [6]. As has been pointed out [4], patients with APS II syndrome and PTC may represent a disease model; under this rationale, it is noteworthy that PTC was diagnosed several years after APS diagnosis in all patients [2][3][4], implying a possible causal effect of autoimmunity.…”

mentioning

confidence: 95%

“…Having read the recent article by Ehlers and Schott [1] on the immunological link between Hashimoto's thyroiditis and papillary thyroid cancer (PTC), we would like to provide further insights into this association by presenting the fourth case of autoimmune polyglandular syndrome (APS) II associated with PTC, but the first in which APS syndrome presented with a combination of adrenal insufficiency, autoimmune thyroiditis, and premature ovarian insufficiency (POI) [2][3][4].…”

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confidence: 99%

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Hashimoto's thyroiditis and papillary thyroid cancer: polyglandular hints

Mintziori

Adamidou

Kita

2015

Trends in Endocrinology & Metabolism

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“…Very few cases of PAS associated with empty sella have been reported in the literature. [10111213] The pathogenesis of empty sella and hypopituitarism is debatable in this case with PAS III. The primary empty sella which is not an uncommon condition, may be considered as a coincidental finding with PAS III.…”

Section: Discussionmentioning

confidence: 94%

Polyglandular autoimmune syndrome iii with hypoglycemia and association with empty sella and hypopituitarism

Bokhari

Khan

Jabri

et al. 2017

Saudi J Med Med Sci

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A 25-year-old Saudi female with a known case of autoimmune thyroiditis presented to the Emergency Room in stuporous condition. A blood test revealed a blood sugar level of 1.7 mmols/l (30.6 mg/dl). The patient was resuscitated with intravenous glucose. Further evaluations of the patient revealed celiac disease and idiopathic thrombocytopenia with preexisting autoimmune thyroiditis (polyglandular autoimmune syndrome III [PAS III]). The severe hypoglycemia, coupled with 6 years of infertility evaluation, revealed a rare association of empty sella syndrome with hypopituitarism {PAS II}.

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Autoimmune polyglandular syndrome: an unusual presentation with empty sella, premature ovarian failure, and Hashimoto’s thyroiditis associated with thyroid cancer

Cited by 4 publications

References 16 publications

Papillary thyroid cancer and autoimmune polyglandular syndrome

Papillary thyroid cancer and autoimmune polyglandular syndrome

Hashimoto's thyroiditis and papillary thyroid cancer: polyglandular hints

Polyglandular autoimmune syndrome iii with hypoglycemia and association with empty sella and hypopituitarism

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