The invisible part of “ICEBERG”:Extraintestinal findings and celiac diseas

Year 2022, Volume: 13 Issue: 1, 64 - 70, 26.03.2022

Serdar Karakaya , İbrahim Karadağ , Aslıhan Mete , Oktay Bulur , Metin Uzman , Esin Beyan

https://doi.org/10.18663/tjcl.1039075

Abstract

Aim: Clinical manifestations of celiac disease (CD) can be quite different and varying. The number of CDs presenting with atypical symptoms is increasing day by day. Hence, we aimed to determine how many patients with CD were atypical celiac through examining their admission complaints, clinical characteristics, laboratory results, endoscopic findings, and pathological findings.
Material and Methods: Eighty-nine patients with CD aged over 18 years who were followed-up in Ankara Kecioren Training and Research Hospital Internal Medicine Clinic between 2007 and 2014 were included in the study. The files of the patients were reviewed retrospectively. Sex, age, hospital admission complaints, clinical characteristics, laboratory results, endoscopy findings, and pathology results of the patients were recorded.
Results: The median age of the patients in the study was 36, and 68.5% of them were female. The most common symptoms of the patients were fatigue (49.4%), anorexia (33.7%), and dyspepsia (22.5%), while diarrhea (19.1%), which is among the classical symptoms, was less common. 7.8% of the patients had no complaints at the time of admission, whereas 47.1% of them had only extraintestinal symptoms. At admission, 47% of the patients had anemia, 73.4% had iron deficiency, and 55.4% of them had vitamin B12 deficiency. When hemoglobin, ferritin, and vitamin B12 levels were compared before and after a gluten-free diet, a significant increase was determined. Transaminase elevation was detected in 25.3% of our patients at the time of diagnosis. When pre-treatment and post-treatment values were compared, a significant decrease was determined in those with high transaminase levels. In the pre-treatment serological test results of the patients, Ig A EMA positivity was the most common finding with a rate of 90.2%, followed by IgG EMA with 88.9%. In terms of pre-treatment endoscopy findings, 50.2% of our patients had normal appearance, 25.4% had a mosaic pattern, 12.6% had effacement of the duodenal folds, 12.6% had a nodular appearance, and 9.5% had a scalloping of the duodenal folds. When the pathology results of our patients were examined, 83.4% of them were at the advanced stage and with Marsh Type 3.
Conclusion: CD may present with atypical complaints such as extraintestinal findings, as in more than half of the patients in this study. Celiac disease should be taken into consideration, particularly in conditions such as iron deficiency, osteoporosis, and transaminase elevations, which are prevalent in the community. The prevalence of CD in our country is estimated to be around 1-2%; however, it is considered that there are more asymptomatic patients with atypical celiac disease. When diagnosing CD, the most crucial step is to consider the potential presence of CD during pre-diagnoses. Thus, CD should be included in the differential diagnosis, specifically in patients presenting with atypical symptoms.

Keywords

Atypical Celiac, Celiac Disease, extraintestinal findings

Buzdağının görünmeyen yüzü: Ekstraintestinal Bulgular ve Çölyak Hastalığı

Abstract

Amaç: Çölyak hastalığı (ÇH) kliniği oldukça farklı ve değişken olabilir. Atipik semptomlarla başvuran ÇH sayısı gün geçtikçe artmaktadır. Bu nedenle ÇH tanısı alan bireylerin hastaneye başvuru şikayetleri, klinik özellikleri, laboratuvar bulguları, endoskopik bulguları, patolojik bulguları inceleyerek hastaların ne kadarının atipik çölyak olduğunu saptamayı amaçladık.
Gereç ve Yöntemler: Çalışmaya Ankara Keçiören Eğitim ve Araştırma Hastanesi İç Hastalıları kliniğine 2007-2014 tarihleri arasında takip edilen 18 yaş üstü tüm ÇH tanısı alan 89 hasta dahil edildi. Hastaların dosyaları retrospektif tarandı. Hastaların cinsiyetleri, yaşları, hastaneye başvuru şikayetleri, klinik özellikleri, laboratuvar tetkikleri, endoskopi bulguları, patoloji sonuçları kaydedildi.
Bulgular: Çalışmadaki hastaların median yaşı 36 ve % 68.5’i kadındı. Hastalarda en sık halsizlik (%49.4), iştahsızlık (%33.7) ve dispepsi (%22.5) görülürken klasik semptomlardan diare (%19.1) daha az sıklıkla görüldü. Hastaların %7.8’inde başvuru anında herhangi bir şikayeti yokken %47.1’inde yalnızca ekstrainestinal semptomlar mevcuttu. Başvuruda hastaların %47’sinde anemi, %73.4’ünde demir eksikliği, %55.4’ünde vitamin B12 eksikliği vardı. Glutensiz diyet öncesi ve sonrası hemoglobin, ferritin, vitamin B12 değerleri karşılaştırıldığında istatiksel anlamlı artış saptandı. Hastalarımızın tanı anında %25.3’ünde transaminaz yüksekliği saptandı. Tedavi öncesi ve sonrası karşılaştırıldığında transaminaz seviyesi yüksek olanlarda istatiksel anlamlı düşüş gözlendi. Hastaların tedavi öncesi serolojik testlerinde en sık Ig A EMA %90.2, Ig G EMA %88.9 pozitifliği vardı. Hastalarımızın tedavi öncesi endoskopi bulguları %50.2 normal görünüm, %25.4’ünde mozaik patern, %12.6’sında foldlarda silinme, %12.6’sında nodüler görünüm ve %9.5’inde tarak sırtı görünümü mevcuttu. Hastalarımızın patoloji raporları incelendiğinde %83.4’ü ileri evre olup Marsh Tip 3’tü.
Sonuç: Sonuç olarak ÇH bu çalışmadaki hastaların yarısından fazlasında olduğu gibi extraintestinal bulgular gibi atipik şikayetlerle başvurabilir. Özellikle toplumda sık görülen demir eksikliği, osteoporoz, transaminaz yükseklikleri gibi durumlarda çölyak hastalığı göz önünde bulundurulmalıdır. Ülkemizde ÇH prevelansının %1-2 civarında olduğu tahmin edilmekte ancak bundan daha fazla atipik çölyak hastalığı asemptomatik hastaların olduğu düşünülmektedir. ÇH tanı koyarken en önemli aşama ön tanılarda akılda bulundurmaktır. Bu yüzden özellikle atipik semptomlarla başvuran hastalarda ÇH ayırıcı tanılar arasında bulundurulmalıdır.

Keywords

Atipik Çölyak, Çölyak Hastalığı, ekstraintestinal bulgular

References

• 1. Rubin CE, Branborg LL, Phelps PC, et al. Apparent identical and specific nature of the duodenal and proximal jejunal lesion in celiac disease and idiopathic sprue. Gastroenterology 1960; 38: 28-49.
• 2. Arranz E, Ferguson A. Intestinal antibody pattern of celiac disease: occurrence in patients with normal jejunal biopsy histology. Gastroenterology. 1993; 104: 1263-72.
• 3. Sulkanen S, Halttunen T, Laurila K, et al. Tissue transglutaminase autoantibody enzyme-linked immunosorbent assay in detecting celiac disease. Gastroenterology 1998; 115: 1322-8.
• 4. Sanders DS, Carter MJ, Hurlstone DP, et al. Association of adult coeliac disease with irritable bowel syndrome: a case-control study in patients fulfilling ROME II criteria referred to secondary care. Lancet. 2001; 358: 1504-8.
• 5. Corazza GR, Di Sario A, Cecchetti L, et al. Bone mass and metabolism in patients with celiac disease. Gastroenterology 1995; 109: 122-8.
• 6. West J, Logan RF, Hill PG, et al. Seroprevalence, correlates and characteristics of undetected coeliac disease in England. Gut 2003; 52: 960–5.
• 7. Fasano A, Berti I, Gerrarduzzi T, et al. Prevalence of celiac disease in at-risk and not-at-risk groups in the United States: a large multicenter study. Arch Intern Med 2003; 163: 286–92.
• 8. Ertekin V, Selimoglu MA, Kardas F, et al. Prevalence of celiac disease in Turkish children. J Clin Gastroenterol 2005; 39: 689-91.
• 9. Reilly NR, Fasano A, Green PH. Presentation of celiac disease. Gastrointest Endosc Clin N Am. 2012; 22: 613-21
• 10. Harmancı Ö. Erişkin yaş grubunda çölyak hastalığının klinik özellikleri (Yan dal uzmanlık tezi). Ankara: Hacettepe Üniversitesi; 2008.
• 11. Merter M. Erişkin çölyak hastalarının klinik ve labaratuar özellikleri ( uzmanlık tezi). Bursa: Uludağ Üniversitesi ; 2013.
• 12. Bardella MT, Fraquelli M, Quatrini M. Prevalence of hypertransaminasaemia in adult coeliac patients and effect of gluten-free diet. Hepatology 1995; 22: 833-6.
• 13. Selimoglu MA, Ertekin V, Altinkaynak S, Hyper-CK-emia in pediatric celiac disease: prevelance and clinical importance. J Clin Gastroenterol. 2007; 41: 667-70
• 14. Harper JW, Holleran SF, Ramakrishnan R, et al. Anemia in celiac disease is multifactorial in etiology. American Journal of Hematology.2007; 82: 996-1000.
• 15. Keskin Ç. Çölyak hastalarında anemi parametrelerinin değerlendirilmesi ve serum hepsidin düzeyleri ile ilişkisi ( uzmanlık tezi). Ankara: Gazi Üniversitesi ; 2013.
• 16. Kemppainen T, Corazza GR, Di Sario A, et al. Osteoporosis in adult patients with celiac disease. Bone 1999; 24: 249-55
• 17. Ross JR, Gibb SP, Hoffman DE, et al. Gluten enteropathy and skeletal disease. JAMA 1966; 196: 270-4
• 18. Sulkanen S, Halttunen T, Laurila K, et al. Tissue transglutaminase autoantibody enzyme-linked immunosorbent assay in detecting celiac disease. Gastroenterology 1998; 115: 1322-8.
• 19. Stern M. Comparative evaluation of serologic tests for celiac disease: A European initiative toward standardization. J Pediatr Gastroenterol Nutr 2000; 31: 513-9.
• 20. Dieterich W, Laag E, Schopper H, et al. Autoantibodies to tissue transglutaminase as predictors of celiac disease. Gastroenterology 1998; 115:1 317-21.
• 21. Savas N, Akbulut S, Saritas U, Koseoglu T, . Correlation of clinical and histopathological with endoscopic findings of celiac disease in the turkish population.2007; 52: 1299-1303
• 22. Bardella MT, Minoli G, Radaelli F, Quatrini M, et al. Reevulation of duodenal endoscopic markers in the diagnosis of celiac disease. Gastrointest Endosc 51: 717-720
• 23. Magazzu G, Bottari M, Tuccari G, et al. Upper gastrointestinal endoscopy can be a reliable tool for celiac sprue in adults. J Clin Gastroenterol. 1994; 19: 255-9.
• 24. Donaldson MR, Book LS, Leiferman KM. Strongly positive tissue transglutaminase antibodies are associated with Marsh 3 histopathology in adult and pediatric celiac disease. J Clin Gastroenterol 2008; 42: 256-60.
• 25. Simondi D, Ribaldone DG, Bonagura GA, et al. Helicobacter pylori in celiac disease and in duodenal intraepithelial lymphocytosis: Active protagonist or innocent bystander? Clin Res Hepatol Gastroenterol. 2015; 2270-7401
• 26. Lasa J, Zubiaurre I, Dima G, et al. Helicobacter pylori prevalence in patients wirh celiac disease: results from a cross sectional study. Arq. Gastroenterol. 2015; 52: 1678-4219