STUDIES ON THE ENTEROPATHY-ASSOCIATED WITH PRIMARY HYPOGAMMAGLOBULINEMIA

Twelve patients with primary hypogammaglobulinaemia with diarrhoea not associated with known microbial pathogens were investigated. Histolog ical evidence of inflammation was common in the stomach and jejunum. M oreover, eight of 10 patients undergoing colonoscopy had low grade 'mi croscopic colitis) with raised intraepithelial lymphocytes and an inta ct crypt architecture. Five of 12 patients had small intestinal inflam mation on (111)indium leucocyte scintigrams and all had increased faec al excretion (normal <1%) of (111)indium (over four days), which varie d in intensity from mild (faecal excretion of (111)indium = 1-3%) to t hat comparable with moderately active (7-14.5%) Crohn's disease. Three patients had small intestinal strictures superficially resembling Cro hn's disease. Histologically, however, these lacked characteristic dia gnostic features of Crohn's disease in two and the third patient had n on-steroidal anti-inflammatory drug induced diaphragm Like strictures. Six of seven who were most severely symptomatic were successfully tre ated with an elemental diet with rapid improvement of symptoms. The fa ecal excretion of (111)indium was repeated in five and all improved bu t histologically the colitis remained unchanged. These studies show th at some patients with primary hypogammaglobulinaemia have intestinal i nflammation unlike that found in classic inflammatory bowel disease. E lemental diet is a useful temporary in the treatment of these measure patients.