Fatal unresponsive villous atrophy of the jejunum, connective tissue disease and diabetes in a girl with intestinal epithelial cell antibody.
The patient presented with a diabetes at the age of 3 years. At the age of 5 years she got persistent diarrhoea, lost weight and showed symptoms or arthritis and pericarditis. She was found to have total villous atrophy of the jejunum, which did not respond to dietary treatment, total parental nutrition, prednisone and cyclophosphamide medication. She had high titres of antinuclear antibodies and elevated serum IgG, but antibodies to DNA and to ribonuclearprotein were negative. A low titre of antibodies to human intestinal epithelial cells was found. The patient died of overwhelming fungal sepsis. We propose that the intestinal damage is part of the autoimmune disease. Careful study of jejunal biopsy specimens is helpful in distinguishing this type of patient from patients with coeliac disease.
D000906:Antibodies / Q000032:analysis*; D001284:Atrophy; D001327:Autoimmune Diseases; D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000150:complications* / Q000276:immunology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000276:immunology; D004848:Epithelium / Q000276:immunology; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology*; D007583:Jejunum / Q000276:immunology / Q000473:pathology*
D000906:Antibodies