In the latest Clinical Problem-Solving article, a 42-year-old man with a history of coronary artery disease presented to the emergency department with left-upper-quadrant abdominal pain that radiated to his back and along the subcostal margin. He also reported substernal chest pressure similar to his usual angina.
Elevated lipase and amylase levels can be indicative of pancreatic inflammation, but elevations are also seen with other intraabdominal conditions, such as cholecystitis, bowel obstruction, or celiac disease. Certain drugs, such as opiates and cholinergic agents, can also spuriously raise lipase and amylase levels.
•What is the epidemiology and typical presentation of celiac disease?
Celiac disease is an autoimmune disorder that affects the small bowel and that is triggered by ingested gluten from barley, rye, and wheat. The disease has both intestinal and extraintestinal clinical manifestations. The intestinal symptoms occur in 40 to 50% of adults, a prevalence that is less than that in children, and include abdominal pain, diarrhea, and other nonspecific abdominal symptoms; a mild elevation in aminotransferase levels is reported in 15 to 20% of patients with celiac disease. Celiac disease is associated with an increase by a factor of three in the risk of pancreatitis.
•What are the extraintestinal manifestations of celiac disease?
Celiac disease is also manifested outside the gastrointestinal tract. Rashes (e.g., dermatitis herpetiformis), arthralgias, neurologic and psychiatric symptoms, fatigue, and infertility can be presenting manifestations. Patients can also present with sequelae of malabsorption, including weight loss, iron-deficiency anemia, and osteoporosis or osteomalacia due to calcium and vitamin D malabsorption. Celiac disease can be associated with other autoimmune conditions, such as type 1 diabetes, autoimmune thyroiditis, and hepatitis. Some retrospective studies, but not others, have shown an increased risk of incident ischemic heart disease, a finding that has been postulated to be associated with chronic inflammation.
Morning Report Questions
Q: What is the epidemiology and prevalence of celiac disease?
A: The prevalence of celiac disease in screening studies is 0.5 to 1%; the disease is seen in all populations for which gluten is part of the diet, although the prevalence varies depending on the population studied. The HLA class II genes HLA-DQ2 or, much less commonly, HLA-DQ8 are expressed in the majority of patients with celiac disease. Although men and women have a similar prevalence of celiac disease in population-based screening studies, the disease is diagnosed more frequently in women than in men.
Q: How may the diagnosis of celiac disease made?
A: The diagnosis of celiac disease is usually made on the basis of serologic screening, followed by a confirmatory small-bowel biopsy. The serologic test of choice is the IgA anti-tissue transglutaminase antibody assay, which is highly standardized, specific (94%), and sensitive (97%). Measurement of IgG anti-tissue transglutaminase antibodies or deamidated gliadin peptide IgG antibodies can be performed in persons who are IgA-deficient. IgA antiendomysial antibodies are highly specific, but testing is expensive and operator-dependent. Measurement of antigliadin antibodies is no longer recommended for diagnosis owing to low diagnostic accuracy. Positive serologic testing in adults should be followed by a small-bowel biopsy to assess the severity of the small-bowel involvement and to ensure that the serologic test results are not falsely positive. Findings on biopsy range from near-normal villous architecture with prominent intraepithelial lymphocytosis to complete villous atrophy.