Sharma A, et al. - The researchers designed this retrospective study to examine the demographic, clinical, and histologic features of adults with autoimmune enteropathy (AIE) compared to adults with refractory celiac disease type 1. Moreover, they aimed to report outcomes of treatment with open-label budesonide. Distinct demographic, clinical, and histologic characteristics were noted in AIE compared to refractory celiac disease type 1. Most patients with AIE (85%) had a clinical response to budesonide, all of whom were unsuccessfully treated with conventional therapies.

Methods

• A retrospective case-control was performed of patients with AIE (n=30) seen at the Mayo Clinic (in Rochester, Minnesota) from 2000 through 2015.
• For this study, patients with refractory celiac disease type 1 who were treated with open-label budesonide served as controls (n=42).
• The researchers reviewed biopsy specimens for all patients.
• Demographic, clinical, biochemical and histologic data were collected from patients.
• Moreover, they collected data on responses to open-capsule budesonide from patients with AIE (available from 22 patients) and controls (available from 42 patients).
• The median duration of follow up was 28 months (range, 0-1421 months).

Results

• Compared to patients with refractory celiac disease type 1 (29% men; P=.002 and mean age, 57±16 years; P=.007), patients with AIE included a higher proportion of men (60%) and were younger (mean, 44±18 years).
• A higher proportion of patients with AIE, compared to patients with refractory celiac disease type 1, presented with chronic diarrhea (100%) and weight loss (90%) (71%; P<.001 and 71%; P=.05, respectively).
• No significant difference was noted in degree of villous atrophy in intestinal tissues from patients with AIE vs controls (P=.68), based on histologic analysis.
• However, a greater proportion of patients with refractory celiac disease) had increased intraepithelial lymphocytes (>40 per 100 epithelial cells in 100%) than the patients with AIE (in 50%) (P=.003).
• Before treatment with open-capsule budesonide was initiated, conventional therapy (systemic steroids) had failed in most patients with AIE (a complete clinical response was reported in only 7 patients).
• Compared to 92% of controls (68% complete response, 24% partial response), 85% of patients with AIE (50% complete response, 35% partial response) reported a clinical response to open-capsule budesonide.