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Year : 2017  |  Volume : 5  |  Issue : 1  |  Page : 71-73

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

Department of Endocrinology, King Fahd Armed Forces Hospital, Jeddah, 21159, Saudi Arabia

Date of Web Publication16-Nov-2016

Correspondence Address:
Patan Murthuza Khan
P.O. Box 9862, King Fahd Armed Forces Hospital, Jeddah 21159
Saudi Arabia
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DOI: 10.4103/1658-631X.194251

PMID: 30787757

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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}.

  Abstract in Arabic 

ملخص البحث:
يعرض الباحثون حالة مريضة تبلغ من العمر 25 سنة، تعاني من التهاب مناعي في الغدة الدرقية أحضرت إلى المستشفى في حالة ذهولية ، اظهر تحليل السكر في الدم انخفاضا قدره (36.3ing/dl) مما استدعى إعطاءها محلول سكر وريديا. أظهر تقييم حالتها وجود حساسية القمح ونقص في الصفائح ، إلى جانب معاناتها من التهاب الغدة الدرقية المناعي. تسمى هذه الحالة بمتلازمة متعددة الغدد المناعي، كما اظهر تقييمها فيما بعد أنها تعاني من العقم ومتلازمة السمع الفارغ وقصور في الغدد النخامية وانخفاض في سكر الدم، ومتلازمة متعددة الغدة المناعـــي (PAS III).

Keywords: Empty sella syndrome, hypopituitarism, polyglandular autoimmune syndrome III

How to cite this article:
Bokhari SA, Khan PM, Al Jabri K, Galal M. Polyglandular autoimmune syndrome iii with hypoglycemia and association with empty sella and hypopituitarism. Saudi J Med Med Sci 2017;5:71-3

How to cite this URL:
Bokhari SA, Khan PM, Al Jabri K, Galal M. Polyglandular autoimmune syndrome iii with hypoglycemia and association with empty sella and hypopituitarism. Saudi J Med Med Sci [serial online] 2017 [cited 2021 Mar 8];5:71-3. Available from: https://www.sjmms.net/text.asp?2017/5/1/71/194251

  Introduction Top

Polyglandular autoimmune syndromes (PASs) are rare disorders characterized by failure of several endocrine and nonendocrine organs associated with immune-mediated destruction. Autoimmune polyglandular syndrome III is a constellation of autoimmune thyroiditis, with another organ-specific autoimmune disease, which cannot be classified as PAS I or PAS II. We are reporting the case of a 25-year-old female with PAS III with a rare association with empty sella turcica and hypopituitarism presenting with severe hypoglycemia. We will discuss the possible association and pathogenesis of empty sella in the setting of PAS III.

  Case Report Top

A 25-year-old Saudi female with a known case of autoimmune thyroiditis (Hashimoto's thyroiditis) presented to the emergency room in stuporous condition. For the past 7 years, she had been treated at a different hospital and prescribed irregular thyroxine replacement to regulate her menstrual cycles. The patient presented with fever, sore throat, vomiting, abdominal pain and confusion of 2 days duration.

A blood sugar test by glucocheck revealed a blood sugar level of 1.7 mmols/l (30.6 mg/dl). The patient was resuscitated with intravenous glucose and her cognitive function promptly improved. Previously, she had experienced similar episodes of hypoglycemia following flu-like symptoms. The patient had a history of nausea, occasional vomiting, abdominal pain and weight loss of 20 kg over the past 2 years. There was no history of headache visual disturbance, galactorrhea, polyuria and polydipsia. The patient had a 6-year-old daughter and had been unsuccessful in conceiving since her daughter's birth. Following delivery, she had no history of postpartum hemorrhage but had experienced irregular menstrual cycles. There was no family history of autoimmune disease. Upon physical examination, the pulse was 102/min and the blood pressure was 100/64 mmHg. There was no hypo- or hyperpigmentation of the skin, hyperpigmentation of the buccal mucosa. The rest of physical examination revealed no abnormalities. The radiological imagings were normal; MRI of the pituitary fossa showed a partially empty sella with asymmetry of the residual pituitary gland [Figure 1].
Figure 1: (a) Coronal section of pituitary magnetic resonance imaging showing empty sella with no obvious pituitary gland tissue. (b) High signal T2 sagittal section of same pituitary showing empty sella.

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The patient was diagnosed with autoimmune thyroiditis, autoimmune thrombocytopenia, celiac disease (PAS III) and partial empty sella syndrome with hypopituitarism. Corticosteroids were initiated, followed by adjustment by adjustment of the Thyroxine. A gluten-free diet was advised by the dietician. The patient's symptoms improved and blood sugars were maintained within the normal range. Her thrombocytopenia responded quickly with steroid treatment [Figure 2].
Figure 2: The graph showing chronic thrombocytopenia and brisk response to corticosteroids.

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  Discussion Top

PAS is defined as multiple endocrine gland insufficiencies accompanied by autoimmune disease of endocrine and nonendocrine systems. Schmidt described a case of non-tuberculous adrenal gland dysfunction with thyroiditis in 1926. Neufeld defined PAS I, PAS II and PAS III and its subtypes in 1980.[1] PAS III is a combination of autoimmune thyroiditis with other organ-specific autoimmune disease. In this case, a 25-year-old Saudi female had suffered from autoimmune thyroiditis with poorly controlled thyroid function for 7 years. Patient had a history of celiac disease. The laboratory findings revealed iron deficiency anemia, low total protein, low albumin and positive anti-transglutaminase immunoglobulin A and immunoglobulin G. Confirmation with jejunal biopsy could not be done since the patient refused to have an endoscopy.

She has asymptomatic chronic mild thrombocytopenia. On exclusion of other causes of chronic thrombocytopenia, such as drugs, autoimmune diseases, viral hepatitis and idiopathic thrombocytopenia a diagnosis of autoimmune thrombocytopenia was made. Thrombocytopenia secondary to celiac disease is rarely described and was unlikely as it responded to steroids. With the combination of autoimmune thyroiditis, celiac disease and idiopathic thrombocytopenia, we diagnosed her with PAS III.[2] Even though autoimmune thrombocytopenia is an uncommon association with PAS, its association with PAS II and PAS III has been reported in several cases in the literature.[3],[4],[5],[6],[7] The association of celiac disease with PAS is also reported in few case reports.[8],[9],[10] It is quite interesting to note that the present case with PAS III is associated with empty sella and hypopituitarism, manifested by severe secondary adrenal insufficiency presenting with hypoglycemia, hyperprolactinemia and hypogonadism. Very few cases of PAS associated with empty sella have been reported in the literature.[10],[11],[12],[13] 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. Although some studies claim deficiencies of one or more pituitary hormones the evidence for such deficiencies is not convincing in primary empty sella.[14] This patient had poorly controlled autoimmune thyroiditis for several years. The resultant thyrotrophic hyperplasia might have damaged the anterior pituitary gland leading to hypopituitarism and subsequently empty sella. This scenario is extremely rare and only four cases have been reported in the literature.[15] The other speculation could be the empty sella with hypopituitarism might have been the late consequence of autoimmune hypophysitis, which is a known association of PAS.[16] There are several case reports where the empty sella is described as the final outcome of lymphocytic hypophysitis.[12],[17],[18] This interesting case demonstrates PAS III with a rare association of empty sella syndrome and hypopituitarism.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Neufeld M, Bizzard RM. Polyglandular autoimmune disease. In: Pinchera A, Doniach D, Fenzi DF, Baschieri L, editors. Autoimmune Aspects of Endocrine Disorders. London, UK: Academic Press; 1980. p. 357-65.  Back to cited text no. 1
Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med 2004;350:2068-79.  Back to cited text no. 2
Candrina R, Giustina A. Development of type II autoimmune polyglandular syndrome in a patient with idiopathic thrombocytopenic purpura. Isr J Med Sci 1988;24:57-8.  Back to cited text no. 3
Dove AF, Patterson KG, Aronstam A. Iidiopathic thrombocytopenic purpura and autoimmune thyroid disease. Practitioner 1977;218:550-2.  Back to cited text no. 4
Volpe JA, Johnston GS. Coexistent hypothyroidism and idiopathic thrombocytopenic purpura. Mil Med 1970;135:1146-8.  Back to cited text no. 5
Nonko O, Juncot T, Yuko Y, Toshiaki K, Masturin N, Tooru Y, et al. Autoimmune polyglandular syndrome type III associated with slowly progressive type I DM, chronic thyroiditis, pernicious anemia, and idiopathic thrombocytopenia purpura. A case report. J Jpn Diabet Soc 2006;49:723-9.  Back to cited text no. 6
Ch'ng CL, Jones MK, Kingham JG. Celiac disease and autoimmune thyroid disease. Clin Med Res 2007;5:184-92.  Back to cited text no. 7
Kumar V, Rajadhyaksha M, Wortsman J. Celiac disease-associated autoimmune endocrinopathies. Clin Diagn Lab Immunol 2001;8:678-85.  Back to cited text no. 8
Miskiewicz P, Kepczynska-Nyk A, Bednarczuk T. Coeliac disease in endocrine diseases of autoimmune origin. Endokrynol Pol 2012;63:240-9.  Back to cited text no. 9
Kim JS, Lee MD, Kim HS, Chung CH. A case of polyglandular autoimmune syndrome type II associated with empty sella.;J Korean Soc Endocrinol; 1998:13:295-300  Back to cited text no. 10
De Rosa G, Della Casa S, Corsello SM, Cecchini L, Callà C. Autoimmune polyglandular syndrome, primary empty sella, and acute lymphocytic leukaemia. Clin Endocrinol (Oxf) 1987;27:535-43.  Back to cited text no. 11
Novoa Mogollon FJ, Carrillo Dominguez A, Puigdevall Gallego V, López del Val T, Mendoza Betancor B, Betancor Leon P. Type II autoimmune polyglandular syndrome associated with empty sella turcica. An Med Interna 1989;6:89-91.  Back to cited text no. 12
Leães CG, Rios MC, Passaglia JP, Pereira-Lima JF, Oliveira Mda C. Autoimmune polyglandular syndrome: An unusual presentation with empty sella, premature ovarian failure, and Hashimoto's thyroiditis associated with thyroid cancer. Gynecol Endocrinol 2012;28:999-1001.  Back to cited text no. 13
De Marinis L, Bonadonna S, Bianchi A, Maira G, Giustina A. Primary empty sella. J Clin Endocrinol Metab 2005;90:5471-7.  Back to cited text no. 14
Dutta D, Maisnam I, Ghosh S, Mukhopadhyay P, Mukhopadhyay S, Chowdhury S. Panhypopituitarism with empty sella a sequel of pituitary hyperplasia due to chronic primary hypothyroidism. Indian J Endocrinol Metab 2012;16 Suppl 2:S282-4.  Back to cited text no. 15
Gao H, Gu YY, Qiu MC. Autoimmune hypophysitis may eventually become empty sella. Neuro Endocrinol Lett 2013;34:102-6.  Back to cited text no. 16
Karaca Z, Tanriverdi F, Unluhizarci K, Kelestimur F, Donmez H. Empty sella may be the final outcome in lymphocytic hypophysitis. Endocr Res 2009;34:10-7.  Back to cited text no. 17
Klein J, Fehm HL. Unusual presentation of hypophysitis preceding an empty sella in a 75-year-old woman. Neuro Endocrinol Lett 2005;26:757-8.  Back to cited text no. 18


  [Figure 1], [Figure 2]


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