CELIAC DISEASE Diagnostic Image Compendium Presented by Gutsy Overu BIOL 218 Human Anatomy INTRODUCTION The purpose of this compendium is to explain what celiac disease is and to illustrate normal vs. pathological intestines and villi Celiac disease is an inflammatory condition of the small intestine, induced by gluten Gluten is the term for the storage proteins of wheat. The alcohol-soluble fraction, called gliadin, has been most studied, but most of all gluten proteins are likely to be toxic in celiac disease Celiac disease is very common, one of the most common inherited diseases that physicians can encounter. Originally considered to be a rare disease of childhood, it is now recognized predominantly as a disease of adults Overall, it is considered to occur in about 1 per 250 individuals. Most recent figures are 1 per 133 in USA, 1 per 122 in Northern Ireland, 1 per 99 Finnish school children and 1 per 100 in western England, which indicate it occurs in about 0.5 to 1% of those of European descent There are other factors apart from gluten that can contribute to the development of celiac disease. Some of the factors are the timing of the first ingestion of gluten in childhood and the amount of gluten, whether breast feeding occurs and whether other members of the family have celiac disease. Smoking also influences the onset of celiac disease. WHAT IS CELIAC DISEASE? What is celiac disease? Celiac disease is an inflammatory condition of the small intestine, induced by gluten. It has diverse clinical manifestations that resemble a multi-systemic disorder rather than a primary intestinal disease. Originally considered a rare disease of childhood, celiac disease is, however, now recognized as a common condition that may be diagnosed at any age. Other terms for celiac disease include gluten sensitive enteropathy and non-tropical sprue. http://3.bp.blogspot.com/_PC3aIMjVWm8/SRVtnmHBGII/AA AAAAAAAso/EC-dMr7gTcs/s400/villi.jpg SYMPTOMS Clinical Presentation The clinical manifestations of celiac disease vary markedly by the age of the patient, the duration and extent of disease, and the presence of extraintestinal pathology (TABLE 2).1 Depending on the features at the time of presentation and taking into account the histological and immunologic abnormalities at the time of diagnosis, celiac disease can be divided into the following three clinical forms: classic (typical), atypical, and silent (asymptomatic).1 Significance of celiac disease Symptomatic celiac disease is associated with a considerable amount of illness and morbidity due to chronic gastrointestinal symptoms and malabsorption of nutrients, weight loss, metabolic bone disease, anemia and general debility. Celiac disease results in an increased risk of the development of various malignancies including lymphoma at any site, not only the small intestine. http://www.uspharmacist.com/CMSImagesContent/20 08/12/USP0812%20Celiac%20T2.jpg VILLI DAMAGE This figure illustrated the progression of destruction to the intestinal villi by gluten. When a person with celiac disease consumes foods or uses produces that contain gluten, his or her immune system reacts by damaging the small intestine and the minute, fingerlike protrusions that line the small intestine, called villi, are damaged or destroyed. Normally villi permit nutrients from food to be absorbed into the bloodstream. If the villi are not healthy or have been destroyed, a person become malnourished regardless of the quantity of food eaten. This villi damage can also lead to lactose intolerance. http://www.my-health-and-wellnesscoach.com/images/villi.JPG ENDOSCOPY Endoscopic and biopsy findings in patients with and without celiac disease. (A) Highdefinition endoscopic photo of normal small intestine. The villi are clearly visible with no evidence of atrophy or scalloping of the folds. (B) Biopsy specimen of normal small intestine (hematoxylin-eosin; original magnification, × 100). (C) PillCam image of small intestine in a patient with celiac disease, showing scalloping of the mucosal folds (arrows) characteristic of a malabsorption pattern. There is also evidence of villous atrophy compared with normal. (D) Biopsy specimen of small intestine in a patient with celiac disease (hematoxylin-eosin; original magnification, × 100). Note the loss of villous architecture. http://www.aafp.org/afp/20071215/af p20071215p1795-u3.jpg MUCOSAL BIOPSY Small-intestinal mucosal biopsy. (A and B) Small-intestinal mucosal biopsy viewed through a dissecting microscope. The normal biopsy (A) shows numerous surface villi, whereas a biopsy from an individual with CD and total villous atrophy shows, in place of the villi, numerous surface openings to underlying crypts and surface ridges (B). (C) H&E-stained section of a normal small-intestinal mucosal biopsy. Original magnification, ×400. (D) H&E-stained section of a small-intestinal mucosal biopsy from an individual with CD and total villous atrophy. Original magnification, ×400. All panels reprinted with from Gastroenterology (1) with permission from the American Gastroenterological Association. http://www.jci.org/articles/view/30253/files/JCI0730253.f1/medium BIOPSY The diagnosis of celiac disease requires finding characteristic changes in a small intestinal biopsy, together with clinical and/or histologic improvement on a gluten-free diet .Biopsy is recommended to establish the diagnosis because the blood tests only suggest the presence of the disease. In addition, the disease is life long. Diagnosis requires the patient to adhere to the diet throughout their life. While it may not easy to control a small child’s diet, as they grow older they need to understand the disease, believe they do have the disease and treat it themselves. Documentation of the disease by biopsy reinforces the diagnosis. Studies have shown adherence to the diet is greatest in those who underwent biopsy http://depts.washington.edu/drrpt/2 003/stories/researchpics/villi.jpg TISSUE STAIN Figure 1. Microscope views of tissues taken from the small intestine by biopsy. At left are normal villi (fingerlike projections extending from the surface). Figure 2. At right, in tissue from a celiac patient, the villi have flattened and disappeared. Figure 1. Figure. 2 http://z.about.com/d/celiac disease/1/0/0/-/-//VilliAtrophy.jpg WHERE DO WE FIND GLUTEN? http://www.isletsofhope.com/pic/ZZPic%2 0celiac-sprue-foods-to-avoid.jpg CONCLUSIONS Adherence to a gluten-free diet remains the mainstay of therapy for celiac disease. Although a gluten-free diet seems simple theoretically, the diet can be difficult for some patients to adhere to. The most common reasons for lack of response are poor compliance and inadvertent gluten ingestion. All patients with celiac disease should be reevaluated periodically. The evaluation should include assessment of growth, assessment of GI and other symptoms associated with celiac disease, and the individual patient's understanding of and compliance with the gluten free-diet. The most useful website for correct and through information pertaining to this disease: http://www.uspharmacist.com/CMSImagesContent/2008/12/USP0812%20Celiac%2 0T2.jpg A follow up compendium that would add to the understanding of celiac disease would be research on various other autoimmune diseases. REFERENCES Slide 3- Diagram of intestinal villi Slide 4- Symptoms chart http://depts.washington.edu/drrpt/2003/stories/researchpics/villi.jpg Slide 9- Histological slide of villi http://www.jci.org/articles/view/30253/files/JCI0730253.f1/medium Slide 8- Biopsy of intestinal villi http://www.aafp.org/afp/20071215/afp20071215p1795-u3.jpg Slide 7- Small intestine mucosal biopsy http://www.my-health-and-wellness-coach.com/images/villi.JPG Slide- 6- Endoscopy http://www.uspharmacist.com/CMSImagesContent/2008/12/USP0812%20Celiac%20T2.jpg Slide 5- Villi Damage http://3.bp.blogspot.com/_PC3aIMjVWm8/SRVtnmHBGII/AAAAAAAAAso/EC-dMr7gTcs/s400/villi.jpg http://z.about.com/d/celiacdisease/1/0/0/-/-/-/VilliAtrophy.jpg Slide 10- Where is Gluten? http://www.isletsofhope.com/pic/ZZPic%20celiac-sprue-foods-to-avoid.jpg
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