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Eosinophilic gastrointestinal disorder in cardiac transplant recipient with Behҫet's disease: A case report


Eosinophilic gastrointestinal disorders (EGID) comprise a group of chronic, inflammatory diseases of the gastrointestinal (GI) tract, that are characterized, clinically, by symptoms of GI tract dysfunction, and histologically, by excess mucosal eosinophils causing histological changes, in the absence of an identifiable secondary cause. Compared to eosinophilic esophagitis (EoE), non-EoE EGIDs such as eosinophilic gastritis (EG) are quite rare. The exact pathophysiology is not clear. Maladaptive T-helper cell type 2 immunity has been implicated. We report a rare case of EoE and EG in a cardiac transplant recipient with Behҫet's disease.

We present a case of a 2 year old female who received a heart transplant in 2019 for dilated cardiomyopathy. A year after her transplant, she developed oral and perianal ulcers. Her immunosuppression at that time included tacrolimus and mycophenolate mofetil. She was admitted due to symptomatic COVID-19 infection and was treated with therapeutic dosing of acyclovir for presumed disseminated herpes simplex virus despite negative blood and titers. Her mycophenolate mofetil was later switched to sirolimus because of neutropenia. She did not respond to acyclovir. Four months after her initial presentation, she was readmitted with fever, poor appetite, diarrhea, vomiting, dehydration, and oral and perianal ulcers. Infectious evaluation was negative. Upper and lower endoscopy performed showed normal appearing mucosa throughout. Biopsies noted esophagitis with eosinophilia (mild to moderate spongiosis, papillary elongation, and basal layer hyperplasia with up to 30 eosinophils per HPF) and eosinophilic gastritis. Duodenal mucosa had borderline/very mild increase eosinophils in lamina propria, and colonic mucosa had very mild eosinophilia in lamina propria. The histology findings were consistent with either a drug reaction or EGID. Given these findings, tacrolimus was discontinued and switched to Cyclosporin. High-dose omeprazole 2 mg/kg/day was started with plan to rescope in 2-3 months. She continued to have recurrent fevers and mouth sores, so she was diagnosed with Behҫet's disease by rheumatology. She was given a course of steroids and started on colchicine. Ulcers resolved with the steroid course and this was discontinued one month prior to her second endoscopy.

Repeated endoscopy noted visually edema of distal esophagus, erythematous and nodular rectum with tiny ulcers, and patchy nodular and erythematous colon. Biopsies showed mild active duodenitis without eosinophilia, normal esophageal mucosa with up to 6 eosinophils per HPF, mild chronic gastritis with mild increased eosinophils and focal to mild active colitis without eosinophilia. There were no histological features of intestinal Behҫet's.  

She is currently diagnosed with EoE responsive to proton pump inhibitors, EG and Behҫet's disease without intestinal involvement. Her case provides several diagnostic difficulties with many confounding factors. To diagnose EGID, other causes of intestinal eosinophilia should be excluded. A drug reaction has been ruled out, but it may be too early to rule out intestinal Behҫet's disease. Though there were no histological features of intestinal Behҫet's, findings may progress with time or represent atypical histological findings of intestinal Behҫet's. Tacrolimus is thought to skew response from Th1 to Th2 and Th2 is implicated in eosinophilic disorders. For now, she is diagnosed with EGID but that may change with time. She will be followed closely with repeat endoscopies.

Wednesday, December 15, 2021