Biopsy-defined Celiac Disease Research Articles

Colorectal Cancer in Biopsy-defined Celiac Disease Seen over 30 Years: Rare, Even in Elderly Adults

Some malignant disorders, including colorectal cancer (CRC), may be reduced in celiac disease. In this study, 154 older adults, including elderly adults over age 60 years, were seen over 30 years. All patients were biopsy-defined and treated with a gluten-free diet. A single elderly female had an early stage colon cancer in the cecum but a persistent iron deficiency anemia led to later detection of biopsy-defined celiac disease and a gluten-free diet mucosal response. This study indicates that colon cancer is rare in biopsy-defined celiac disease. In this study, there were no gastric, pancreatic or hepato-biliary tract cancers seen. Genetic and environmental dietary factors may play a role, however, the rarity of colorectal cancer in other celiac-associated colonic diseases (eg., collagenous or lymphocytic colitis) suggests that an immune-mediated protective effect may occur.

Immune Thrombocytopenia in Adult Celiac Disease

Celiac disease has been associated with a number of immune-mediated hematologic disorders, including idiopathic or immune thrombocytopenic purpura (ITP). Review of the literature suggests that children are particularly affected, often with overt skin findings, but in this report, a 39-yr old male with biopsy-defined celiac disease was detected with a persistent immune-mediated reduction in his platelet count despite treatment and follow-up for more than a decade. Although the celiac disease resolved with a strict gluten-free diet, there was no effect on the ITP. Experience suggests that ITP may be rarely appreciated in adults (compared to children) because it may be limited in severity and occurs without clinically obvious purpura or evidence of bleeding, even with diagnostic or therapeutic biopsy procedures. A subsequent retrospective review of over 300 celiac disease patient records from a single teaching hospital clinical practice experience spanning almost 40 years confirmed no additional cases of thrombocytopenia. ITP appears to be rarely associated with celiac disease in adults and, in spite of resolution of the enteropathy, did not appear to respond to a gluten-free diet, even after more than a decade of dietary treatment.

Lymphoma-like presentation of hepatocellular carcinoma

REFERENCES 1. Kaukinen K, Halme L, Collin P, et al. Celiac disease in patients with severe liver disease: gluten-free diet may reverse hepatic failure. Gastroenterology 2002; 122: 881-8. 2. Demir H, Yuce A, Caglar M, et al. Cirrhosis in children with celiac disease. J Clin Gastroenterol 2005; 39: 630-3. 3. Freeman HJ. Lymphoproliferative and intestinal malignancies in 214 patients with biopsy-defined celiac disease. J Clin Gastroenterol 2004; 38: 429-34. 4. Oruc N, Ozutemiz O, Tekin F, et al. Celiac disease associated with B-cell lymphoma. Turk J Gastroenterol 2010; 21: 168-71. 5. Fattovich G, Stroffolini T, Zagni I, et al. Hepatocellular carcinoma in cirrhosis: incidence and risk factors. Gastroenterology 2004; 127(Suppl): S35-50. 6. Sorensen HT, Friis S, Olsen JH, et al. Risk of liver and other types of cancer in patients with cirrhosis: a nationwide cohort study in Denmark. Hepatology 1998; 28: 921-5.

Detection of adult celiac disease with duodenal screening biopsies over a 30-year period.

Serological studies suggest that celiac disease may be present in approximately 0.5% to 1% of the North American population. Screening data based on small intestinal biopsy performed during routine endoscopic evaluations are not available. Patients referred between January 1982 and December 2011 for evaluation of gastrointestinal symptoms and requiring elective investigative upper endoscopic evaluation underwent duodenal biopsies to determine whether changes of adult celiac disease were present. A total of 9665 patients, including 4008 (41.5%) males and 5657 (68.5%) females, underwent elective endoscopies and duodenal biopsies. Of these, 234 (2.4%) exhibited changes of celiac disease including 73 males (1.8%) and 161 females (2.8%). During the first 20 years, the number of biopsy-positive patients in five-year intervals progressively decreased and, subsequently, during the next 10 years, the number progressively increased. Celiac disease is far more common in specialist practice than has been suggested in the evaluation of healthy populations using serological screening studies. Endoscopic duodenal biopsy is an important method of identifying underlying celiac disease and should be routinely considered in all patients undergoing an elective endoscopic evaluation. Noninherited factors, possibly environmental, may play a role in the appearance of biopsy-defined celiac disease and alter detection over time.

Collagenous colitis as the presenting feature of biopsy-defined celiac disease.

Prior case reports have linked celiac disease with collagenous colitis, but serological and retrospective pathologic survey studies of small intestinal biopsies have produced conflicting results. In this report, consecutive patients with chronic diarrhea and the initial finding of collagenous colitis were evaluated to determine if occult celiac disease was also present. All 36 patients with no exclusions were offered upper endoscopy and biopsies. If the changes of untreated celiac disease were detected in the small intestinal biopsies, then a gluten-free diet was instituted. Diet response was based on resolution of diarrhea and normalization of repeated small intestinal biopsies. Eight patients, or over 20%, had severely abnormal small bowel biopsy changes characteristic of untreated celiac disease. In some, lymphocytic or collagenous gastritis were also detected with or without celiac disease. All 8 patients were treated with a gluten-free diet alone and, in all, diarrhea improved and small intestinal biopsies normalized. In 5, however, repeated colonic biopsies showed persistent collagen deposits. Autoimmune thyroid disease or neoplastic diseases, like lymphoma known to be associated with celiac disease were also noted in this cohort with collagenous colitis, often in the absence of celiac disease. This study indicates that collagenous colitis may be the presenting clinical and pathologic feature of celiac disease. Diagnosis of collagenous colitis should lead the clinician to consider exclusion of underlying occult celiac disease.

Lymphoproliferative and Intestinal Malignancies in 214 Patients With Biopsy-defined Celiac Disease

Prior studies have suggested that the incidence of some neoplastic disorders, particularly malignant lymphoma, is increased in celiac disease. In the present study, lymphoproliferative and intestinal cancers in 214 consecutive biopsy-defined celiac disease patients, including 148 females (69.2%) and 66 males (30.8%), seen by a single clinician over more than 20 years were tabulated. Of the 214 patients, 151 were diagnosed with celiac disease before age 60 and 63 at or after age 60. In total, 18 malignant lymphomas and 3 small intestinal adenocarcinomas were detected. While the overall incidence of malignant lymphoma was 8.4%, similar to other European centers, the incidence in elderly celiacs in this study was 22.2%. Celiac disease was detected before or even after the diagnoses of lymphoma or small intestinal adenocarcinoma were established. In some, epithelial lymphocytosis was evident in gastric, colonic, or biliary ductal epithelium. In addition, other immune-mediated disorders, dermatitis herpetiformis, and autoimmune thyroid disease were common, suggesting a distinct clinical and pathologic phenotype in celiac disease that may predispose to malignant complications. Finally, except for a single hypopharyngeal carcinoma in a celiac disease patient with a malignant lymphoma, other malignant disorders of esophagus, stomach, and colon were not detected.

Biopsy-defined adult celiac disease in Asian-Canadians.

Celiac disease is thought to be a genetically based disorder reported mainly from European countries as well as countries to which Europeans have emigrated, including North America. This report documents a clinical experience of biopsy-defined celiac disease in 14 Asians diagnosed since 1988 in a single Canadian teaching hospital. Eleven were Indo-Canadians, including 10 of Punjabi descent. Other ethnic groups were also represented, including two Japanese and one Chinese patient. Abdominal pain was the most frequent presenting symptom. Anemia, particularly associated with a deficiency of iron was common, along with diarrhea and weight loss. Endoscopic studies documented lymphocytic gastric and colonic mucosal changes in over one-third of the cases while antibodies for tissue transglutaminase were positive in all patients tested. Dermatitis herpetiformis, diabetes mellitus and autoimmune liver disease were also documented. These findings indicate for the first time that adult celiac disease occurs in Asian populations living in North America, particularly in those of Punjabi descent.

Failure of added dietary gluten to induce small intestinal histopathological changes in patients with watery diarrhea and lymphocytic colitis.

Lymphocytic colitis is a form of microscopic colitis usually characterized by watery diarrhea and often associated with biopsy-defined celiac disease. Two patients with lymphocytic colitis and normal small intestinal biopsies who were administered 40 g of added dietary gluten for four consecutive weeks are presented. Small intestinal biopsies from multiple sites in the proximal small bowel were done after three and four weeks to determine whether pathological changes in latent celiac disease could be induced in these patients with a high gluten-containing diet. In addition, colorectal biopsies were done to determine whether the colitis was sensitive to oral gluten. No alterations in the small intestinal biopsies were detected in either patient and no changes occurred in colitis severity. Although microscopic forms of colitis have been linked to celiac disease, this study indicates that lymphocytic colitis is a heterogeneous clinicopathological disorder that, in some patients, is independent of any gluten-induced intestinal pathological changes.