Human Anatomy at Colby

Ari Thomas, Laurel Edington and Danielle Levine

http://youtu.be/DiKDOyG6Olg

Grand_Rounds_Celiac_Disease powerpoint_pdf

Grand Rounds Synopsis

A 42-year-old man presented with a chief complaint of chest and abdominal discomfort that had begun suddenly two days before as a sharp left upper quadrant pain radiating to his back, associated with nausea and early satiety, and that increased in intensity over the next two days. The next day, he experienced substernal chest pressure consistent with his usual angina, but which did not respond to a single sublingual nitroglycerin tablet; it only resolved completely after IV morphine, ketorolac (an NSAID), chewable aspirin, and three more sublingual nitroglycerin tablets. He had experienced no recent abdominal trauma, vomiting, rectal bleeding or black stools.¹

The patient’s past medical history includes hypertension, hyperlipidemia (excess blood lipids), and coronary artery disease (myocardial infarction at 32 years of age, with coronary artery angioplasty with stent placement, and subsequent bypass grafting).^1,2 For more than ten years before presentation (since the coronary-artery bypass surgery), he has experienced chronic diarrhea that has worsened since his cholesterol-lowering medication was increased 6 months ago.¹  During the past six months, he has experienced daily headaches, nocturia (excessive urination at night), feeling warm at night, an unintentional weight loss of 35 lbs, and occasional early satiety.^1,2  Although he has a family history of colon cancer, a colonoscopy performed 4 months prior was unremarkable.¹

The abdominal and chest pain, weight loss, and history of gastrointestinal symptoms suggested an acute chest syndrome, acute abdominal syndrome, inflammatory disorder, or cancer. An acute coronary syndrome was unlikely and an echocardiogram and chest radiography confirmed this. The physical exam ruled out acute chest syndromes, but sensitivity in the upper right quadrant suggested an upper abdominal disorder. Lipase, aminotransferase, and amylase levels were elevated, suggesting pancreatitis, liver injury (from an infection or drug use) or disease, or liver cancer. A CT scan of the abdomen ruled out pancreatitis and colon and small bowel disorders, but showed enlarged jejunal lymph nodes. An exploratory laparotomy was performed and the lymph nodes showed reactive follicular and interfollicular hyperplasia and lipogranulomas, suggesting an inflammatory abdominal disorder. Evidence of lymphoproliferative disorders was absent, ruling out cancer. A small-bowel biopsy showed flattened villi and intraepithelial lymphocytes, which suggested celiac disease. Positive IgA tissue transglutaminas and IgA endomysial antibody tests, the most specific and sensitive tests for celiac disease, were positive and confirmed the final diagnosis.¹

The patient was advised to follow a gluten-free diet with an intramuscular iron supplementation as well as a multivitamin for general vitamin and mineral deficiencies.³ The gluten-free diet includes avoiding foods made out of wheat, rye, barley, oats, and processed foods that may contain wheat flour.⁴

Based on the patient’s symptoms, doctors diagnosed the patient with celiac disease. This disease is an autoimmune disorder that is provoked by intaking various forms of gluten and affects the small bowel. The intestinal symptoms of this disease include abdominal pain, diarrhea, a mild elevation of aminotransferase levels, and an increased risk of pancreatitis. Abdominal pain in the patient may have been due to transient intussusception related to celiac enteropathy.  Celiac disease also has extragastrointestinal system effects such as rashes, arthralgias, neurologic and psychiatric effects, fatigue, and infertility.⁴ Patients also suffer from malabsorption of nutrients which can lead to weight loss, iron-deficiency, and osteoporosis. Patients have an abnormal immune response to the gliadin component of the gluten protein, where type 1 helper T cells cause inflammation in the epithelium and lamina propria of the small intestine, which alters the structures of the intestinal villi and therefore causes malabsorption.³ Celiac disease may also accompany type 1 diabetes, thyroiditis, and hepatitis.¹

Celiac disease is different than a gluten sensitivity.⁵ Although the symptoms are similar, a person with a gluten sensitivity does not have the intestinal damage that a person with celiac disease has. Patients with a gluten sensitivity also do not have the IgA tissue transglutaminase or IgA endomysial antibodies that patients with celiac disease have.⁶ Since blood tests and intestinal biopsies will not diagnose a gluten sensitivity, using a process of exclusion helps to diagnose the sensitivity.⁵ Both disorders are treated by following a strict gluten-free diet.^5,6

References:

1. Ole-Petter Riksfjord Hamnvik, M.D., Fidencio Saldana, M.D., Bruce D. Levy, M.D., and Joseph Loscalzo, M.D., Ph.D. N Engl J Med 2014; 371:1333-1338.

2. Medline Plus: Medical Dictionary. Besthesda, MD: U.S. National Library of Medicine, 2012. (Accessed January 13, 2015 at http://www.nlm.nih.gov/medlineplus/mplusdictionary.html.)

3. Leffler, D. Celiac Disease Diagnosis and Management: A 46-Year-Old Woman With Anemia. Jama 2011; 306:1582–1592.

4. Rubio-Tapia, A., Hill, I. D., Kelly, C. P., Calderwood, A. H., & Murray, J. A. American College of Gastroenterology Clinical Guideline: Diagnosis and Management of Celiac Disease. The American Journal of Gastroenterology 2013, 108:656–677.

5. Non-Celiac Gluten Sensitivity.  Ambler, PA.: National Foundation for Celiac Awareness, 2015. (Accessed January 25, at http://www.celiaccentral.org/non-celiac-gluten-sensitivity/).

6. Gluten Sensitivity.  Woodland Hills, CA.: Celiac Disease Foundation, 2015.  (Accessed January 25, at http://celiac.org/celiac-disease/non-celiac-gluten-sensitivity/).