Download citation file:
Celiac disease, also known as gluten-sensitive enteropathy (as well as two older and less preferred terms, nontropical sprue and celiac sprue) is a multisystem disorder estimated to affect approximately 1% of Americans (1). It results from an inappropriate T-cell–mediated immune response to ingested gluten that causes inflammatory injury to the small intestine in genetically predisposed persons. Damage to the proximal small intestinal mucosa results in the malabsorption of nutrients. The average age of diagnosis is in the fifth decade of life but only an estimated 10% to 15% of persons with celiac disease in the United States have been diagnosed (2). The prevalence of celiac disease in the United States seems to have increased 4- to 5-fold over the past 3 to 4 decades (3). Disease manifestations are protean, and gastrointestinal symptoms are not always present. Virtually every body system can be affected, with dermatologic, hematologic, neurologic, musculoskeletal, endocrine, reproductive, and digestive systems most commonly involved. Moreover, celiac disease is associated with a variety of autoimmune conditions whose clinical course may be affected by the diagnosis and treatment of celiac disease. Although patients respond well to treatment with a gluten-free diet, unrecognized or untreated celiac disease is associated with both increased mortality (3, 4) and risk for intestinal lymphoma (5).
Dermatitis herpetiformis. This disorder is an intensely pruritic papulovesicular rash affecting extensor surfaces, such as the shoulders (top), elbows, knees, back, and buttocks (bottom). Although all patients with dermatitis herpetiformis have the intestinal lesions of celiac disease, few have gastro-intestinal symptoms. Immunofluorescent detection of IgA deposits at the dermal–epidermal junction in a perilesional biopsy of a fresh skin lesion is sufficient for diagnosis and precludes the need for intestinal biopsies.
Endoscopic appearance of celiac disease. Such features include scalloping or notching of the folds, as shown. Fissuring or cracking of the flat intervening mucosa between the folds can also be seen. These endoscopic features are helpful for targeting biopsy sites, but their absence does not rule out the diagnosis; as such, intestinal biopsies should be obtained during upper endoscopy for evaluation of potential celiac disease.
Histologic appearance of celiac disease. Characteristic features of the intestinal mucosa in celiac disease include inflammation and varying degrees of villous atrophy. Inflammation comprises lymphocytes, plasma cells, macrophages, and other chronic inflammatory cells in the lamina propria. It also includes intraepithelial lymphocytes, which are more prominent toward the tips of the villi. The biopsy depicted here shows partial villous atrophy with characteristic inflammatory changes.
Please read the other comments before posting. Contributors must reveal any conflict
Comments are moderated and will appear on the site at the discretion of The American
College of Physicians editorial staff. Please be sure your email address is
updated in your account, otherwise the American College of Physicians will not be
able to contact you about your comment.
* = Required Field
Disclosure of Any Conflicts of Interest*
(applies to the past 5 years and foreseeable future) Indicate any potential conflicts
of interest of each author below, including specific financial interests and relationships
and affiliations relevant to the subject matter or materials discussed in the manuscript
(eg, employment/affiliation, grants or funding, consultancies, honoraria, speakers
bureau, stock ownership or options, expert testimony, royalties, donation of medical
equipment, or patents filed, received, or pending). If all authors have none, check
"No potential conflicts or relevant financial interests" in the box below. Please
also indicate any funding received in support of this work. The information will
be posted with your response.
The In the Clinic® slide sets are owned and copyrighted by the American College
of Physicians (ACP). All text, graphics, trademarks, and other intellectual property
incorporated into the slide sets remain the sole and exclusive property of the ACP.
The slide sets may be used only by the person who downloads or purchases them and
only for the purpose of presenting them during not-for-profit educational activities.
Users may incorporate the entire slide set or selected individual slides into their
own teaching presentations but may not alter the content of the slides in any way
or remove the ACP copyright notice. Users may make print copies for use as hand-outs
for the audience the user is personally addressing but may not otherwise reproduce
or distribute the slides by any means or media, including but not limited to sending
them as e-mail attachments, posting them on Internet or Intranet sites, publishing
them in meeting proceedings, or making them available for sale or distribution in
any unauthorized form, without the express written permission of the ACP. Unauthorized
use of the In the Clinic slide sets will constitute copyright infringement.
to gain full access to the content and tools.
Learn more about subscription options