Celiac disease

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Long standing abdominal pain, on and off diarrhea, unexplained anemia, weight loss of 6 kg in last 6 months and bilateral lower limb edema for last 2 weeks. Past history of multiple miscarriages.

Patient Data

Age: 30 years
Gender: Female
ct

CT abdomen with intravenous contrast (plain water was used as neutral oral contrast agent).

Prominent jejunal loops with loss of mucosal folds and normal caliber ileal loops with increased number of mucosal folds (jejunoileal fold pattern reversal).

Multiple small sub centimeter mesenteric lymph nodes.

Normal size liver with generalized decreased attenuation (fatty liver).

Mild edematous changes in subcutaneous soft tissues of lower abdomen.

These radiological features (jejunoileal fold pattern reversal and fatty liver) are suggestive of malabsorption, likely due to celiac disease. Upper gastrointestinal endoscopy with small bowel biopsy is advised for histopathological confirmation.

Case Discussion

Prior to CT scan, patient underwent different laboratory investigations among which positive laboratory findings were low serum albumin level, low serum iron and low total iron binding capacity. After CT scan, anti-tissue transglutaminase IgA (tTG-IgA) level was done which was 215.30 Units (> 30 units is moderate to strong positive). 

Patient also underwent upper gastrointestinal endoscopy which showed featureless scalloped mucosal folds of second part of duodenum, normal looking first part of duodenum and multiple shallow and deep chronic ulcers in gastric antrum. Multiple gastric and duodenal biopsies were taken and duodenal biopsy histopathology revealed total villous atrophy, cryptic hyperplasia, goblet cell depletion, increased intraepithelial lymphocytes (more than 30 lymphocytes by 100 enterocytes); based on these features,a diagnosis of celiac disease (atrophy of grade B2/3) was given. Gastric biopsies showed moderate chronic gastritis without atrophy.

Small bowel dilatation, hallmark of untreated celiac disease, is seen in 70-95% patients. It is best seen in mid & distal jejunum and degree of dilatation is related to severity of disease. Decreased number of folds in proximal jejunum (1-3 folds per inch) and increased number of folds in distal ileum (>5 folds per inch) known as jejunoileal fold pattern reversal is the most specific for the diagnosis of uncomplicated celiac disease.

Gold standard of diagnosis is duodenal biopsy whereas serology tests e.g. anti-tissue transglutaminase antibody (anti-tTG IgA) and anti-gliadin antibodies (AGA) can be used for screening and monitoring compliance. Anti-tTG IgA has higher sensitivity & specificity to AGA.

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