No abstract

Purpose-Autoimmune enteropathy is a rare cause of intractable diarrhea associated with circulating gut autoantibodies and a predisposition to autoimmunity. It is rarely observed in adults with only eleven cases reported to date. Results-The study population was 87% Caucasian, 47% females, with median age of 55 years (interquartile range: 42 to 67 years). All patients had diarrhea, weight loss and malnutrition. Celiac disease was excluded by lack of response to gluten free diet or absence of the celiac disease susceptibility HLA genotypes. Fourteen patients were tested for gut epithelial cell antibodies and 93% were positive for anti-enterocyte and/or anti-goblet cell antibodies. Predisposition to autoimmune diseases was noted in 80%, as indicated by a variety of circulating autoantibodies. Small intestinal histopathologic findings included subtotal villous atrophy and lymphoplasmacytic infiltration in the lamina propria with relatively few surface intraepithelial lymphocytes. T-cell receptor gene rearrangement studies were negative in all cases. Immunosuppressive therapy was required in 93% cases. Clinical improvement was noted in 60% after 1-8 weeks of steroid therapy. Methods-FifteenConclusion-Autoimmune enteropathy is a heterogeneous disease and should be considered in the differential diagnosis of malabsorption and small bowel villous atrophy. The presence of gut epithelial cell antibodies can help confirm the diagnosis. No single agent is unequivocally effective in inducing remission, and immunosuppressive therapy is required in most cases.

Fresh peripheral blood mononuclear cells (PBM) isolated from healthy human donors and activated with concanavalin A (Con A) induced suppression of the proliferation response to Con A in an allogeneic mixed leukocyte culture reaction (MLC). When PBM were incubated in media alone for 24 hr prior to Con A activation of suppressor cells there was a significant decrease in the suppressive response. PBM incubated in the presence of thymosin for 24 hr prior to Con A activation of the suppressor cells promoted a further augmentation of mitogen-induced proliferation. When PBM were incubated with thymosin in the absence of Con A activation of suppressor cells, enhancement of proliferation was observed in the MLC. These experiments suggest that thymosintreated PBM from healthy donors promote a helper rather than suppressive effect on mitogen-induced proliferation of allogeneic responder lymphocytes.