Introduction: Autoimmune enteropathy is an X-linked autoimmune disease. A syndrome of intractable diarrhea, varying levels of villous atrophy
of small intestine , presence of circulating autoantibodies to enterocytes. Diagnostic criteria is any 3 of the following features 1) features of
malabsorption 2) Diarrhea > 6 weeks 3) HPE showing villous blunting with crypt and intraepithelial lymphocytosis 4) exclusion of other causes of
villous atrophy like celiac disease and refractory sprue 5) serology positive for antibodies like anti-enterocyte and anti-goblet. Aims & objectives :
The aim of this case report was to report a rare case of autoimmune enteropathy in an Indian female , with an atypical presentation with diagnostic
and treatment challenges. The typical presentation of autoimmune enteropathy i Discussion: s chronic diarrhea with features of malnutrition and
weight loss. But the presentation in this patient was more in favour of presentation of koch's abdomen , mostly of TB peritonitis with ascitic
component. . Adult autoimmune enteropathy being a rare entity is thought late in differential diagnosis. The presence of antibodies , intraop
ndings and response to steroids favour autoimmune enteropathy. Although AIE is a rare entity,a multifactorial and a high degree of suspicion is
necessary for diagnosis. In a country like India, where tuberculosis is so prevalent, Conclusion: other differential diagnosis should also be thought
of before coming to a conclusion.,as other diseases like autoimmunity previously thought to be rare are not so. ATT should only be started after
conrming the diagnosis with histopathology report
