Polyglandular Autoimmune Syndrome Type III Complicated By Autoimmune Hepatitis, a Case Report

Presentation Number: MON 295
Date of Presentation: April 3rd, 2017

Pamela Ohri*1, Manmeet Kaur2 and Summaya Latif3
1UCONN Health, Farmington, CT, 2Hospital Of Central Connecticut, Southington, CT, 3University of Connecticut Health Center, Farmington, CT

Abstract

Introduction:

Polyglandular Autoimmune Syndrome (PGA) Type III is a disorder characterized by the presence of autoimmune thyroid disease in combination with another autoimmune illness, excluding primary adrenal insufficiency. We describe a rare case of PGA Type III complicated by Autoimmune Hepatitis.

Clinical Case:

A 51 year-old female with a past medical history of hypothyroidism secondary to autoimmune thyroiditis presented with severe, cramping epigastric and abdominal pain accompanied by bloating and diarrhea. Electrolyte panel and complete blood count were within normal reference range. She underwent gastroenterologic workup with EGD and colonoscopy. Colonoscopy was within normal limits. EGD revealed multiple small subcentimeter polyps of the gastric fundus. Histopathologic examination confirmed grade 1 gastric carcinoid tumor (Ki67<1%), with intestinal metaplasia and absence of parietal cells.

Laboratory evaluation revealed a significantly elevated gastrin level of 1663 pg/mL (N <100) and elevated anti-parietal cell antibody at 81 Units (N <20), which confirmed type 1 gastric carcinoid. She was also found to have anti-TPO level >394 IU/mL (N <35), suggesting polyglandular autoimmune syndrome type III. Further workup revealed chromogranin A level 26 ng/mL (N <15) and pancreatic polypeptide level >1600 pg/mL (N <291), but was otherwise largely within normal limits. She continued to complain of bloating, abdominal pain and flushing, thus evaluation for the presence of liver metastasis was pursued. Octreotide scintography showed normal physiologic uptake, with no evidence of somatostatin rich tumor. Helical CT of the chest, abdomen and pelvis revealed no evidence of metastatic disease, but did show heterogeneous appearing liver. Transaminase levels were elevated and indicative of hepatocellular damage with AST 239 u/L (N 14-36), ALT 173 u/L (N 9-52). Viral hepatitis panel was negative, as well as ANA, but she was found to have positive anti-smooth muscle (Anti-SM) antibodies, suggesting autoimmune hepatitis.

Conclusion:

The patient presented with an extremely rare combination of three different autoimmune disorders, and is only the second known case of PGA Type III complicated by autoimmune hepatitis to date. Though the specific incidence of PGA Type III is unknown, it is a rare disorder. This case highlights the importance of early detection and proper screening for additional autoimmune disease processes in order to prevent future complications and end organ failure.

 

Nothing to Disclose: PO, MK, SL