Intestinal Lymphangiectasia Secondary to an Inflammatory Process: Case Report and Review of Literature

A 38 year-old male presented with a one-year history of generalized edema and diarrhea. Laboratory tests revealed severe hypoproteinemia, lymphocytopenia, no proteinuria or steatorrhea, and a normal intestinal D-xylose absorption test. Intestinal lymphangiectasia with protein-losing enteropathy was documented by a high alpha-1-antitrypsin clearance rate in stool and an intestinal histopathlogic study. However, there were differences from primary lymphangiectasia, such as later age of onset, high erythrocyte sedimentation rate, leukocytosis, and decreased C3 level. Intestinal lymphangiectasia secondary to an inflammatory process was considered likely in this patient. The response to low dose steroid was unsatisfactory.