A Case of Sequential Development of Celiac Disease and Ulcerative Colitis

William Dickey

Disclosures

Nat Clin Pract Gastroenterol Hepatol. 2007;4(8):463-467. 

In This Article

The Case

A 65-year-old white female presented to a gastroenterology clinic with a 3-month history of excess flatulence/flatus, and weight loss of 9.5 kg (21 lbs) in the preceding 12 months. Her medical history was otherwise unremarkable; she reported no abdominal pain, alteration in bowel habit or hemochezia. She denied a family history of colon neoplasia, celiac disease, Crohn's disease or ulcerative colitis. Physical examination did not reveal anything of note.

Initial laboratory investigations revealed that the patient had a normal hemoglobin concentration with normal levels of serum ferritin, vitamin B12, folate, C-reactive protein (CRP) and thyroxine and thyroid-stimulating hormone. Her erythrocyte sedimentation rate (ESR) was also normal. An autoimmune profile of the patient, which included tests for antinuclear, antinucleolar, anti-smooth-muscle and antimitochondrial antibodies, was negative. The patient's serum was negative for endomysial antibody (EmA), as measured by indirect immunofluorescence in an assay that used primate esophagus substrate (Biodiagnostics, Upton-upon-Severn, UK). The patient's tissue transglutaminase antibody (TTGA) level, measured by use of a guinea pig antigen-based enzyme-linked immunosorbent assay (ELISA; Immco Diagnostics, Buffalo, NY, USA), was 8 ELISA units (normal level 0-25 ELISA units).

No abnormalities were revealed by esophagogastroduodenoscopy. Assessment of four forceps biopsy samples obtained from the descending duodenum of the patient, in order to look for evidence of celiac disease, revealed normal intraepithelial lymphocyte counts and normal villous height (Figure 1). A colonoscopy was performed and reached the terminal ileum and findings were normal except for sigmoid diverticulosis. CT scans of the chest, abdomen and pelvis were also normal. During the initial follow-up period, the patient's weight stabilized, her symptoms responded to treatment with an antispasmodic (mebeverine, 135 mg three times daily), and she was discharged back to the care of her general practitioner.

Figure 1.

Histologic image of a duodenal biopsy sample from the case patient, obtained soon after the initial presentation. No evidence of celiac disease can be seen--intraepithelial lymphocyte counts are normal and villi are of normal height.

The patient was referred back to the gastroenterology clinic approximately 5 years later, after having presented to her general practitioner with a 6-month history of fatigue; she did not report any other symptoms. A low concentration of hemoglobin in the patient's blood (115 g/l; normal concentration >125 g/l) had prompted her general practitioner to request celiac serology tests. The results of celiac serology revealed that the patient's serum was positive for EmA and contained an abnormal level of TTGA (54 ELISA units). No abnormalities were seen when the patient underwent push enteroscopy to the jejunum. Biopsy samples obtained from the patient's duodenum confirmed the presence of villous atrophy with crypt hyperplasia and increased numbers of intraepithelial lymphocytes (Figure 2). No histologic abnormalities were found in jejeunal biopsy samples. The patient declined repeat colonoscopy. CT scans of her chest, abdomen and pelvis were normal.

Figure 2.

Histologic image of a duodenal biopsy sample from the case patient obtained at the patient's second presentation, almost 5 years after the initial presentation. Villous atrophy, crypt hyperplasia and an increased number of intraepithelial lymphocytes can be seen.

Additional blood work revealed that the patient had a low serum ferritin level (38 pmol/l [17 ng/ml]; normal value >58 pmol/l [>26 ng/ml]) indicative of iron deficiency; however, the ESR and levels of B12, folate and CRP were normal. A dual X-ray absorptiometry scan revealed that the patient had osteoporosis in the lumbar spine and osteopenia in the left femur. It was concluded that her anemia and osteoporosis were the result of celiac disease. The patient began a gluten-free diet under dietetic supervision. She began maintenance treatment with daily calcium and vitamin D supplements and weekly oral alendronic acid (70 mg) for her reduced bone density. Within 6 months she was asymptomatic, her hemoglobin concentration had risen to 134 g/l without the need for iron supplements, and her celiac serology had reverted to normal.

Approximately a year later (6.5 years after her initial presentation) the patient reported diarrhea for the first time. She underwent colonoscopy that revealed granular, erythematous mucosa with multiple ulcers, distributed continuously from the rectum to the splenic flexure (Figure 3). Histologic analysis of biopsy samples revealed a chronic inflammatory infiltrate, with ulceration, cryptitis and crypt abscess formation and no granulomata, consistent with a diagnosis of ulcerative colitis. At this time the patient's hemoglobin concentration had fallen to 97 g/l, her ESR was 90 mm/h, her CRP concentration was 165 mg/l (normal concentration <8 mg/l) and her celiac serology remained negative.

Figure 3.

Image obtained from colonoscopy performed after the patient's third presentation, 6.5 years after her initial presentation. Granular, erythematous mucosa with multiple ulcers can be seen, consistent with active ulcerative colitis.

Duodenal biopsy results showed complete villous recovery with no excess of intraepithelial lymphocytes, and wireless capsule endoscopy revealed no pathology elsewhere in the small bowel. Although the patient's diarrhea settled initially when she was prescribed oral mesalazine (1,000 mg twice daily) as maintenance therapy, she relapsed with diarrhea and hemochezia 3 months later. A course of oral prednisolone (commenced at a dose of 40 mg daily and reduced progressively over an 8 week period) was required to achieve satisfactory remission once again, after which oral mesalazine successfully maintained remission during an additional 12 months of follow-up. The patient remains under review at a specialist celiac clinic.

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