scholarly journals Achalasia in a Patient with Polyglandular Autoimmune Syndrome Type II

2015 ◽  
Vol 9 (2) ◽  
pp. 160-164 ◽  
Author(s):  
Bashar S. Amr ◽  
Chaitanya Mamillapalli
Keyword(s):  
Autoimmune Thyroid Disease ◽  
Syndrome Type ◽  
Type Ii ◽  
Rare Disorders ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Thyroid ◽  
Autoimmune Syndrome ◽  
Esophageal Sphincter ◽  
Polyglandular Autoimmune Syndrome Type

Achalasia is a rare disease characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter to relax. The etiology of this disease remains unknown. Polyglandular autoimmune syndrome type II is a well-identified disease characterized by the occurrence of autoimmune Addison's disease in combination with autoimmune thyroid disease and/or type 1 diabetes mellitus. We report a case that suggests autoimmunity and immunogenicity as a probable contributing factor for association of these two rare disorders.

scholarly journals A rare simultaneous manifestation of polyglandular autoimmune syndrome type II

2020 ◽  
Vol 2020 ◽  
Author(s):  
Michael Dick ◽  
Michael Croxson
Keyword(s):  
Coeliac Disease ◽  
Autoimmune Thyroid Disease ◽  
Type I Diabetes ◽  
Type I ◽  
Syndrome Type ◽  
Type Ii ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Thyroid ◽  
Autoimmune Syndrome ◽  
Polyglandular Autoimmune Syndrome Type

Summary Polyglandular autoimmune syndrome type II is a rare condition defined by the presence of autoimmune primary adrenal insufficiency along with autoimmune thyroid disease and/or type-I diabetes. Onset of these conditions will usually be separated by several years, though in rare instances it can occur simultaneously. This syndrome can also be associated with various non-endocrine autoimmune diseases, such as vitiligo and alopecia. Coeliac disease is less commonly associated with polyglandular autoimmune syndrome type II and is more commonly associated with polyglandular autoimmune syndrome type III. Here we describe an interesting case of a young male presenting with simultaneous manifestation of Addison’s disease and Graves, with coincident asymptomatic coeliac disease, as a rare manifestation of polyglandular autoimmune syndrome type II. Learning points: Polyglandular autoimmune syndrome type II is rare, has female predominance, and peak onset in the third and fourth decades of life. Onset of Addison’s disease will usually precede or follow onset of type-I diabetes or autoimmune thyroid disease by several years in this syndrome. Simultaneous onset can occur, as in this case. Coeliac disease is uncommonly associated with this syndrome. Coeliac disease is more commonly associated with polyglandular autoimmune syndrome type III. Coeliac disease should be screened for in patients with associated autoimmune conditions, such as type-I diabetes or autoimmune thyroid disease.

scholarly journals Primary Amenorrhea Associated with Hyperprolactinemia in Polyglandular Autoimmune Syndrome Type II: A Case Report

2018 ◽  
Vol 40 (07) ◽  
pp. 425-429
Author(s):  
Luiza Cottas ◽  
Maria Borges ◽  
Lívia Oliveira ◽  
Ana Resende ◽  
Meire Ataíde ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Primary Hypothyroidism ◽  
Primary Amenorrhea ◽  
Syndrome Type ◽  
Type Ii ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Thyroid ◽  
Addison Disease ◽  
Autoimmune Syndrome ◽  
Polyglandular Autoimmune Syndrome Type

AbstractPolyglandular autoimmune syndrome type II (PGA-II) is a rare immunoendocrinopathy syndrome characterized by the occurrence of autoimmune Addison disease along with diabetes mellitus type 1 and/or autoimmune thyroid disease. Here, we report the case of a 23-year-old female with PGA-II who was followed up at the dermatology and endocrinology clinics of the Universidade Federal do Triângulo Mineiro, located in the state of Minas Gerais, Brazil. First, the patient presented diffuse skin hyperpigmentation, vitiligo; and in sequence, due to vomiting, appetite and weight loss, hypoglycemia, amenorrhea, and galactorrhea, the patient was then diagnosed with PGA-II. The patient also presented intense hyperprolactinemia due to primary hypothyroidism. The late diagnosis of PGA-II is frequent because the disorder is uncommon and has non-specific clinical manifestations. This report emphasizes the significance of a timely diagnosis and appropriate treatment to reduce morbidity and mortality associated with these diseases, especially Addison disease. The present study reports a rare case of a patient with PGA-II with primary amenorrhea associated with hyperprolactinemia.

scholarly journals Polyglandular Autoimmune Syndrome Type II in a Patient Presenting With Hypotension After Acute Illness

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A151-A152
Author(s):  
Samantha Sokoloff ◽  
Rachana Mundada ◽  
Barbara Simon
Keyword(s):  
Septic Shock ◽  
Adrenal Insufficiency ◽  
Severe Illness ◽  
Adrenal Crisis ◽  
Syndrome Type ◽  
Type Ii ◽  
Polyglandular Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
Polyglandular Autoimmune Syndrome Type

Abstract Introduction: Polyglandular Autoimmune Syndrome Type II (PAS-2) is a rare disorder characterized by two or more endocrine diseases (primary adrenal insufficiency, autoimmune thyroid disease, type 1 diabetes). Of these, the most common is autoimmune thyropathy, followed by Type 1 Diabetes (1). These endocrinopathies rarely have concurrent onset. This case reports a 22-year-old male, recently recovered from severe pneumonia, who presented to the Emergency Department in acute adrenal crisis and was diagnosed with PAS-2. Case: A 22-year-old male with past medical history of celiac disease presented with abdominal discomfort, nausea, vomiting, fatigue and dizziness for 1–2 weeks. Review of systems included a 20-pound weight loss over several months. Recent history included a hospitalization at another facility three weeks prior for pneumonia and septic shock requiring admission to the ICU and vasopressor treatment. He was not discharged on any medications. He was afebrile with heart rate of 105/min, blood pressure 78/48 mm Hg and BMI of 17.2. Physical exam revealed dry mucous membranes and mild diffuse abdominal tenderness. Skin was warm and dry without hyperpigmentation. Laboratory values included sodium 123 (135 - 146 mmol/L), potassium 6.8 (3.3 - 4.8 mmol/L), glucose 47 (70 - 100 mg/dL), TSH 37.67 (0.3 - 5.00 uIU/mL), and FT4 0.7 (0.7 - 1.7 ng/dL). He was started on fluids and intravenous hydrocortisone. Cortisol and ACTH levels drawn prior to the initiation of steroids resulted at 0.6 (6–20 mcg/dl) and 977 (9 - 46 pg/mL) respectively. Additional labs included: aldosterone < 1 ng/dL, 21 hydroxylase antibody positive, TPO antibody > 1000 (0 - 100 Units) and GAD-65 antibody > 47 IU/mL (<5 IU/mL). Levothyroxine was initiated after hydrocortisone. Blood glucose was elevated during hospitalization, peaking at 227 mg/dL. He was discharged on prednisone, fludrocortisone and levothyroxine. At 2 week follow up, he reported overall improvement in health and was pleased with a weight gain of 12 lbs. Blood glucose remained mildly elevated (123 - 143 mg/dL). Conclusion: The patient had pneumonia and septic shock septic at an outside hospital three weeks prior to presentation. There was no record of steroid administration or suspicion of adrenal insufficiency. We postulate that his severe illness contributed to significant depletion of his adrenal reserve, and therefore he presented to our facility a short time later in overt adrenal crisis. Adrenal crisis is unusual to be the first presentation of PAS-2. It is important to have a high index of suspicion for adrenal insufficiency and PAS-2 in patients presenting with severe illness or hypotension who have known autoimmune disorders. Reference: 1. Kahaly, G.J., Frommer, L. Polyglandular autoimmune syndromes. J Endocrinol Invest. 2018; 41: 91–98.

Sign in / Sign up

Export Citation Format

Share Document