ISSN: 2373-6372GHOA

Gastroenterology & Hepatology: Open Access
Case Report
Volume 4 Issue 2 - 2016
Addisonian Crisis in a Patient with Schmidt’s Syndrome
Lynda Hoang*, Hugo Rivera, Mehul Dalaut and Sulaiman Sultan
Internal Medicine Residency, Parkview Medical Center, USA
Received: January 26, 2016 | Published: February 12, 2016
*Corresponding author: Lynda Hoang, Internal Medicine Residency, Parkview Medical Center, USA, Email:
Citation: Hoang L, Rivera H, Dalaut M, Sultan S (2016)Addisonian Crisis in a Patient with Schmidt’s Syndrome. Gastroenterol Hepatol Open Access 4(2): 00093. DOI: 10.15406/ghoa.2016.04.00093

Introduction

Schmidt’s syndrome, also known as polyglandular syndrome type II, is a rare disease with potential life threatening consequences. It is characterized by multiple immune endocrinopathies, including Addison’s disease with thyroid disease and/or type I diabetes [1-3]. We describe a case of Addisonian crisis in a patient with known Addison’s disease. Interestingly, she also has type 1 diabetes mellitus and Graves’ disease but was never previously diagnosed with Schmidt’s syndrome.

Case

EPI: Exocrine Pancreatic Insufficiency; CCK: Cholecystokinin; HIV: Human Immunodeficiency Virus; FE-1: Fecal Elastase-1; PERT: Pancreatic Enzyme Replacement Therapy; PPI: Protompump Inhibitors; CF: Cystic Fibrosis; CFTR: Cystic Fibrosis Transmembrane Conductance Regulator; IBD: Inflammatory Bowel Disease; CD: Chron’s Disease; UC; Ulcerative Colitis; AIDS: Acquired Immunodeficiency Syndrome; CT: Computed Tomography; MRCP: Magnetic Resonance Cholangiopancreatography; S-MRCP: Secretin-Enhanced Magnetic Resonance Cholangiopancreatography; EUS: Endoscopic Ultrasound; FNA: Fine Needle Aspiration; ERCP: Endoscopic Retrograde Cholangiopancreatography; Fcht: Fecal Chymotrypsin; PLT: Pancreolauryl Test.

Discussion

Schmidt’s syndrome is a compendium of polyendocrine autoimmune diseases (Addison’s disease, autoimmune thyroiditis/Graves’ disease, type I diabetes) and can be associated with other non-endocrine autoimmune disorders, such as myasthenia gravis, Sjogren’s syndrome, and rheumatoid arthritis [3]. It is autosomal dominant with variable penetrance and usually affects middle-aged females (female-to-male ratio 3:1) [1]. The diagnosis of this condition can be challenging due to its rarity (1.4 - 4.5 cases/100,000 population), atypical presentation, and variance in clinical symptoms depending on the type and severity of gland involved. Thus, a high index of suspicion is warranted for early diagnosis and appropriate hormonal therapy [1].

References

  1. Gupta AN, Nagri SK (2012) Schmidt's syndrome - Case report. Australas Med J 5(6): 292-295.
  2. Bhullar S, Seifeldin R, Hemady N (2014) A non-classical presentation of Schmidt’s syndrome: a rare and lethal disease. J Endocrinol Metab 9(1): 79-82.
  3. Neufeld M, Maclaren NK, Blizzard RM (1980) Two types of autoimmune Addison’s disease associated with different polyglandular autoimmune (PGA) syndrome. Pediatr Ann 9(4): 154-162.
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