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      Extraintestinal Manifestations of Celiac Disease: Early Detection for Better Long-Term Outcomes

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          Abstract

          Population-based screening studies have shown celiac disease to be one of the most common chronic gastrointestinal diseases. Nevertheless, because of the diverse clinical presentation, the great majority of patients remain unrecognized. Particularly difficult to identify are the multifaceted extraintestinal symptoms that may appear at variable ages. Although the pathogenesis and long-term outcome of these manifestations are still poorly established, there is some evidence that unrecognized celiac disease predisposes to severe complications if not diagnosed and prevented with an early-initiated gluten-free diet. Therefore, it is of utmost importance that physicians of different disciplines learn to recognize celiac disease in individuals with non-gastrointestinal symptoms. In the future, more studies are needed to clarify the factors affecting development and prognosis of the extraintestinal manifestations.

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          Most cited references87

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          Global Prevalence of Celiac Disease: Systematic Review and Meta-analysis

          Celiac disease is a major public health problem worldwide. Although initially it was reported from countries with predominant Caucasian populations, it now has been reported from other parts of the world. The exact global prevalence of celiac disease is not known. We conducted a systematic review and meta-analysis to estimate the global prevalence of celiac disease.
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            Characteristics of adult celiac disease in the USA: results of a national survey.

            The clinical spectrum of adults with celiac disease in the United States, where the disease is considered rare, is not known. We sought this information by distributing a survey. A questionnaire was distributed by way of a celiac newsletter, directly to celiac support groups, and through the Internet. Respondents (1,612) were from all United States except one. Seventy-five percent (1,138) were biopsy proven. Women predominated (2.9:1). The majority of respondents were diagnosed in their fourth to sixth decades. Symptoms were present a mean of 11 yr before diagnosis. Diarrhea was present in 85%. Diagnosis was considered prompt by only 52% and 31% had consulted two or more gastroenterologists. Improved quality of life after diagnosis was reported by 77%. Those diagnosed at age > or = 60 yr also reported improved quality of life. Five respondents had small intestinal malignancies (carcinoma 2, lymphoma 3) accounting for a relative risk of 300 (60-876) for the development of lymphoma and 67 (7-240) for adenocarcinoma. Patients with celiac disease in the United States have a long duration of symptoms and consider their diagnosis delayed. Improved quality of life after diagnosis is common. An increased risk of developing small intestine malignancies is present.
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              Duration of exposure to gluten and risk for autoimmune disorders in patients with celiac disease. SIGEP Study Group for Autoimmune Disorders in Celiac Disease.

              The relationship between celiac disease and many autoimmune disorders has been explained by the sharing of a common genetic factor. In a multicenter national study, we examined the relationship between the prevalence of autoimmune disorders in celiac disease and the duration of exposure to gluten. Over a 6-month period, 909 patients with celiac disease (group A; mean age, 16.1 +/- 3.8 years; grouped according to age at diagnosis into three subgroups [group A1, 10 years]), 1268 healthy controls (group B; mean age, 20.8 +/- 4.5 years), and 163 patients with Crohn's disease (group C; mean age, 28.8 +/- 10 years) were evaluated for the presence of autoimmune disorders. Prevalence of autoimmune disorders in group A was significantly higher than in group B (14% vs. 2.8%; P < 0.000001) but not higher than in group C (12.9%). Prevalence of autoimmune disorders in celiac disease increased with increasing age at diagnosis: 5.1% in group A1, 17% in group A2, and 23.6% in group A3 (P = 0.000001). In group A3, the prevalence of autoimmune disorders was significantly higher than in group C. In a logistic regression model, age at diagnosis was the only significant predictor variable of the odds of developing an autoimmune disorder (r = 0.3; P < 0.000001). Our data show for the first time that the prevalence of autoimmune disorders in celiac disease is related to the duration of exposure to gluten.
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                Author and article information

                Journal
                Nutrients
                Nutrients
                nutrients
                Nutrients
                MDPI
                2072-6643
                03 August 2018
                August 2018
                : 10
                : 8
                : 1015
                Affiliations
                [1 ]Celiac Disease Research Center, Faculty of Medicine and Life Sciences, University of Tampere, 33014 Tampere, Finland; laurikka.pilvi.l@ 123456student.uta.fi
                [2 ]Department of Internal Medicine, Hospital District of South Ostrobothnia, 60200 Seinäjoki, Finland
                [3 ]Tampere Center for Child Health Research, Tampere University Hospital and University of Tampere, 33014 Tampere, Finland; nurminen.samuli.j@ 123456student.uta.fi (S.N.); laura.kivela@ 123456fimnet.fi (L.K.)
                Author notes
                [* ]Correspondence: kalle.kurppa@ 123456uta.fi ; Tel.: +358-50-318-6316
                Article
                nutrients-10-01015
                10.3390/nu10081015
                6115849
                30081502
                379c04c3-e730-47cc-96cd-02d15251a0e4
                © 2018 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

                History
                : 01 July 2018
                : 31 July 2018
                Categories
                Review

                Nutrition & Dietetics
                celiac disease,extraintestinal,recognition,diagnosis,clinical presentation,gluten-free diet,prognosis

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