Zusammenfassung
Die neuen kombinierten serologischen Testverfahren mit hoher diagnostischer Signifikanz lassen als Screeningverfahren das Ausmaß bisher unerkannter Zöliakiefälle erahnen. In Deutschland ist von einer Prävalenz von 1:500 auszugehen. Die klinische Symptomatik der Zöliakie ist in Abhängigkeit von Alter, Krankheitsdauer, Ausdehnung der Zottenveränderungen und der extraintestinalen Manifestationen sehr variabel. Die klassische Form der Zöliakie mit chronischen Durchfällen, vorgewölbtem Abdomen, Appetitlosigkeit und Gedeihstörung (Malabsorption)—im Erwachsenenalter als Sprue bekannt—stellt nur die Spitze des "Eisbergs" dar. Daneben gibt es eine noch viel größere Zahl atypischer, oligosymptomatischer Fälle mit extraintestinalen Manifestationen (z. B. an der Haut, den Gelenken, dem Skelett und den Zähnen) und mit neurologischen Symptomen oder Infertilität und Aborten. Assoziationen zu Autoimmunerkrankungen (Typ-1-Diabetes, Thyreoiditis) und Chromosomenanomalien (Down- und Turner-Syndrom) sind bekannt. In der Differenzialdiagnose unklarer Krankheitsbilder muss die Zöliakie aufgrund der hohen Prävalenz viel häufiger in Betracht gezogen werden.
Abstract
Newly developed serological assays with high diagnostic significance as screening tools have increased the recognition of hitherto undetected cases of celiac disease. The prevalence in Germany is 1:500. The clinical manifestations vary markedly with the age of the patient, the duration and extent of disease, and the presence of extraintestinal pathology. The classic gastrointestinal form with chronic diarrhea, abdominal distention, anorexia, and failure to thrive (malabsorption) represents only the tip of the "celiac iceberg". This classic clinical picture also exists in the gastrointestinal form of adult sprue. However, a much higher number of atypical and oligosymptomatic cases with extraintestinal symptoms of the skin, joints, bones, and teeth and with neurological manifestations, infertility, or recurrent abortions has been described. There are associations with insulin-dependent diabetes mellitus type 1, autoimmune thyroiditis, and chromosomal anomalies such as Turner and Down syndrome. Because of its high prevalence, the "chameleon" celiac disease has to be considered much more frequently in the differential diagnosis of unsolved clinical cases.
Literatur
Auricchio S, Greco L, Troncone R (1988) Gluten-sensitive enteropathy in childhood. Pediatr Clin North Am 35: 157–187
Bardella MTh, Vecchi M, Conte D, Del Ninno E, Fraquelli M, Pacchetti S, Minola E, Landoni M, Cesana BM, De Frachnis R (1999) Chronic unexplained hypertransaminasemia may be caused by occult celiac disease. Hepatology 29: 654–657
Bürgin-Wolff A, Gaze H, Hadziselimovic F, Huber H, Lentze MJ, Nussle D, Reymondberthet C (1991) Antigliadin and antiendomysium antibody determination for coeliac disease. Arch Dis Child 66: 941–947
Clemente MG, Musu MP, Frau F, Lucia C, De Virgilis S (2002) Antitissue transglutaminase antibodies outside celiac disease. J Pediatr Gastroenterol Nutr 34: 31–34
Dieterich W, Ehnis T, Bauer M, Donner P, Volta U, Riecken EO, Schuppan D (1997) Identification of tissue transglutaminase as the autoantigen of celiac disease. Nat Med 3: 797–801
Fabiani E, Taccari LM, Rätsch I-M, Di Giuseppe S, Coppa GV, Catassi C (2000) Compliance with gluten-free diet in adolescents with screening-detected celiac disease: a 5-year follow-up study. J Pediatr 136: 841–843
Fasano A, Catassi C (2001) Current approaches to diagnosis and treatment of celiac disease: an evolving spectrum. Gastroenterology 120: 636–651
Hill I, Fasano A, Schwartz R, Counts D, Glock M, Horvath K (2000) The prevalence of celiac disease in at-risk groups of children in the United States. J Pediatr 136: 86–90
Holmes GKT, Prior P, Lane MR, Pope RN, Allan RN (1989) Malignancy in coeliac disease—effect of a gluten-free diet. Gut 30: 333–338
Ivarsson A, Persson LA, Nyström L, Ascher H, Cavell B, Danielsson L, Dannaeus A, Lindberg T, Lindquist B, Stenhammar L, Hernell O (2000) Epidemic of coeliac disease in Swedish children. Acta Paediatr 89: 165–171
Keller K-M (1996) Antigliadin-, Antiretikulin- und Antiendomysium-Antikörper in der Diagnostik der Zöliakie. In: Kist M, Caspary WF, Lentze MJ (Hrsg) Ökosystem Darm VII. Springer, Berlin Heidelberg New York, pp 243–260
Mäki M, Collin P (1997) Coeliac disease. Lancet 349: 1755–1759
Marsh NN (1992) Gluten, major histocompatibility complex, and the small intestine: a molecular and immunobiologic approach to the spectrum of gluten sensitivity ("celiac sprue"). Gastroenterology 102: 330–354
Martinelli P, Troncone R, Paparo F, Torre P, Trapanese E, Fasano C, Lamberti A, Budillon G, Nardone G, Greco L (2000) Coeliac disease and unfavourable outcome of pregnancy. Gut 46: 332–335
Naveh Y, Rosenthal E, Ben-Arieh Y, Etzioni A (1999) Celiac disease-associated alopecia in childhood. J Pediatr 134: 362–364
Troncone R, Ferguson A (1991) Anti-gliadin antibodies. Review. J Pediatr Gastroenterol Nutr 12: 150–158
Ventura A, Magazzù G, Greco L, for the SIGEP Study Group for Autoimmune Disorders in Celiac Disease (1999) Duration of exposure to gluten and risk for autoimmune disorders in patients with celiac disease. Gastroenterology 117: 297–303
Ventura A, Neri E, Ughi C, Leopaldi A, Città A, Not T (2000) Gluten-dependent diabetes-related and thyroid-related autoantibodies in patients with celiac disease. J Pediatr 137: 263–265
Walker-Smith JA, Guandalini S, Schmitz J, Shmerling DH, Visakorpi JK (1990) Revised diagnostic criteria for coeliac disease. Report of a working group of the European Society for Paediatric Gastroenterology and Nutrition. Arch Dis Child 65: 908–911
Walker-Smith JA (1997) Celiac disease. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watkins JB (eds) Pediatric gastrointestinal disease. Mosby-Year Book, St Louis, pp 840–861
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Keller, KM. Klinische Symptomatik: "Zöliakie, ein Eisberg". Monatsschr Kinderheilkd 151, 706–714 (2003). https://doi.org/10.1007/s00112-003-0751-9
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00112-003-0751-9