A typical adult celiac disease: Report of cases and review of the literature

Mohamed Ismail Yasawy; Abdulaziz Abdulatif Al-Quorain; Dalal Mohammed Tamimi

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CASE REPORT

Year : 2004 | Volume : 10 | Issue : 2 | Page : 99-102

A typical adult celiac disease: Report of cases and review of the literature

Mohamed Ismail Yasawy¹, Abdulaziz Abdulatif Al-Quorain¹, Dalal Mohammed Tamimi²
¹ Department of Medicine, King Fahad Hospital of the University, AI-Khobar, Saudi Arabia
² Department of Pathology, King Fahad Hospital of the University, AI-Khobar, Saudi Arabia

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Date of Submission	23-Dec-2002
Date of Acceptance	17-Nov-2003

How to cite this article:
Yasawy MI, Al-Quorain AA, Tamimi DM. A typical adult celiac disease: Report of cases and review of the literature. Saudi J Gastroenterol 2004;10:99-102

How to cite this URL:
Yasawy MI, Al-Quorain AA, Tamimi DM. A typical adult celiac disease: Report of cases and review of the literature. Saudi J Gastroenterol [serial online] 2004 [cited 2021 Jun 19];10:99-102. Available from: https://www.saudijgastro.com/text.asp?2004/10/2/99/33344

Celiac Disease (CD) is a disease in which the mucosal lining of the small intestine is damaged in response to ingestion of gluten and similar proteins, which are found in wheat, oats, rye, barley and other grains. Adult CD is well known, but most reports have come from Western countries. It is uncommon among Arabs, with few or scantly reports (Rawashdeh and Manoha), while it is a major cause of malabsorption syndrome in some countries such as Ireland with an incidence in adults of 1% and a female to male ratio of 2:1. The adult peak of onset is during the 4^th and 5^th decades in women and 5^th and 6^th decades in mend^{[1],[2],[3],[4],[5]} . Adult CD has a wide variety of presentations and symptoms. We report three cases where the main presenting complaints were either nongastrointestinal (amenorrhea, hair fall and anorexia) or a typical gastrointestinal (GI) symptoms suggestive of irritable bowel syndrome (IBS).

Case 1

A 42-year-old Saudi male was presented to us with two years history of excessive hair fall and flatulence with recent worsening of his symptoms for further investigations and management. Additional history revealed weight loss of 10kg in the last two years and occasional episodes of diarrhea without blood or mucous. He was diagnosed as having IBS and managed accordingly. The clinical examination revealed thin, pale and ill-looking patient with sunken eyes, scanty hair, angular stomatitis and an unevenly spaced beard. On laboratory investigation, he was found to be anaemic (Hb lOg/dl MCV 65), serum iron, TIBC and folate levels were all low.

Upper GI endoscopy showed loss of kerckring folds in the descending duodenum and the histopathology revealed complete villous atrophy. The serology markers were positive for antigliadin, antireticuline and anti-endomysial antibodies. Based on the above findings, the diagnosis of celiac disease was strongly suspected and the patient was managed with gluten- free diet, vitamin and minerals. He responded nicely and all his symptoms and signs improved and he had 35kg gain. Repeated duodenal biopsy after one year of the treatment showed normal villous pattern.

Case 2

A 25-year-old Saudi female was complaining of longstanding anorexia and flatulence. She was seen indifferent health centers and diagnosed as having IBS and managed accordingly with negative response. She described regular deterioration of her symptoms. On physical examination she was pale and depressed, spleen was just palpable and lower limbs edema were noticed.

The laboratory investigations revealed anemic patient (Hb 9.5 mg/dl and MCV 65). Her serum albumin was on the lower side. Adult celiac disease was entertained in the differential diagnosis. Upper GI endoscopy was performed, duodenal biopsy obtained and histopathology revealed total villous atrophy. The antireticuline and antiendomysial antibodies were positive, but antigliadin antibody was negative. The patient was managed as celiac disease and she responded perfectly. Repeated endoscopy and biopsy after one year showed normalization of her duodenal villi, anemia and weight (she gained 10 kg).

Case 3

This Patient is a 30-year-old Palestinian female, who presented with palpitation and secondary amenorhea. She was seen and evaluated for possible mitral valve prolapse and hyperthyroidism. On examination, she was pale and ill-looking with few aphtous ulcers in her oral cavity. Gynecological examination revealed no abnormalities. Her investigation showed Hb of 8.8mg/dl with iron and folate deficiency and evidence of osteoporoses. Antigliadin and antiendomysial antibodies were positive. Endoscopic duodenal biopsy revealed total villous atrophy [Figure - 1]. Following the management for adult celiac disease, all her symptoms improved and she regained her periods. Repeated duodenal biopsy and histopathology revealed partial reconstruction of the villous structure [Figure - 2].

Discussion

Since the recognition of celiac disease in the 1950's, it has increasingly been diagnosed in both adults and children^[2] . The incidence varies from (1/100 to 1/1500). It has become apparent that the prevalence of CD is higher than previously thought, because of an increasing awareness of atypical, mildly symptomatic or silent cases^[6] . In fact, all our three cases had a typical presentations.

This study showed that a typical CD is associated with a wide variety of extra intestinal manifestations, highlighting the association between atypical celiac disease and extra gastrointestinal manfestations including miscarriage infertility and hair fall^[7],[8],[12] . Serologic markers (antibody titers to gliadin, reticulin, and endomysial) are known to be raised. in individuals with untreated celiac disease. These titres usually fall on treatment with gluten-free diet. Antiglaidin antibody (AGA) and endomysial tests are the most accurate. A recent study reported the sensitivity of IgG AGAs and IgA AGAs to be 100% and 89% respectively in untreated children, the specificity was 86% for IgG and 95% for IgA. Antiendomysial antibodies (EMA) are connective tissue protein found between myofibrils in the gastro-intestinal tract of primates. The sensitivity and specificity of EMAs have been reported to be 97-100% and 98-99% respectively^{[9],[10],[11]} Anti tissue transglutaminase antibodies (t TG) is another immunological marker, which are highly sensitive, but less specific than EMA.. Further studies are required to establish the role of isolated anti t TG positivity. Small bowel biopsy remains the gold standard for the diagnosis of glutensensitive enteropathy. Since untreated celiac disease can be life threatening, a combination of clinical suspicion, IgAEMA total IgA Antibody level and small intestinal biopsies are required to identify most cases of atypical or sub clinical celiac disease. However, a more precise description of sub clinical/silent celiac disease can only emerge from screening studies on general population. In conclusion this report and review confirms the presence of CD in our society and highlights the extremely polymorphic nature of this disease, which can affect any organ^{[8],[11],[12],[13],[14]} . A High index of suspicion is needed to identify the atypical forms of the disease.

References

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4.	Rawashdeh MO, Khalil B, Raweily E. Celiac disease in Arabs. J Pediatr Gastroenterol Nutr 1996; 23: 415-8.
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