Refractory coeliac disease: or is it?

Abstract

A previously healthy 33-year-old man presented with a 3-month history of nausea, anorexia and weight loss. Coeliac disease was diagnosed at another hospital with positive serology and D2 biopsies and he was started on a gluten-free diet. The details of these tests were not available to us. Despite good adherence to a gluten-free diet, he continued to lose weight and became anaemic. A repeat gastroscopy showed D2 ulcers. infection was excluded, coeliac serology remained negative but D2 biopsies showed partial duodenal villous flattening with intraepithelial lymphocytosis. Type 1 refractory coeliac disease was diagnosed. Prednisolone and azathioprine were commenced but the vomiting and weight loss progressed. A subsequent gastroscopy and CT scan revealed a D3 stricture and duodenal dilatation, respectively, in keeping with superior mesenteric artery syndrome. An infracolic duodenojejunostomy was performed and immunosuppression stopped. Subsequently, all his symptoms resolved and he remains well on a gluten-free diet.

Superior mesenteric artery syndrome: Diagnosis and management

“…Various complications of SMA syndrome have been reported (Table 3 )[ 35 , 62 - 86 ]. Notably, unrecognized or severe cases may progress to life-threatening complications, such as hypovolemic shock, aspiration pneumonia, and sudden death, even in young patients.…”

“…Laboratory tests are not diagnostic, but they are necessary to identify the presence of electrolytic complications and pancreato-biliary abnormalities. As for differential diagnosis, almost all disorders mimicking SMA syndrome are summarized in Table 5 [ 14 , 26 , 44 , 55 - 59 , 86 , 101 - 127 ]. Patients with eating disorders, functional dyspepsia, and peptic ulcer disease present non-specific symptoms masquerading as SMA syndrome.…”