Immunoproliferative small intestinal disease (
IPSID) is prevalent in the Mediterranean region and in many Third World countries but is rare in Southeast Asia. Between 1980-1990, 4 cases of
IPSID were admitted to Ramathibodi Hospital, Bangkok. Three were males and the mean age was 32 +/- 20.2 years. All patients presented with chronic
diarrhea of 7 months to 6 years duration, and
weight loss of 15 to 31 kg. All were malnourished, three cachectic, and one patient showed growth retardation. Intestinal parasites were found in all cases: two had multiple
infections and three had uncommon protozoal
infections (coccidium, cryptosporidium).
Barium radiographs revealed intestinal mucosal fold thickening with malabsorption pattern in all cases. Alpha chain
IgA was detected in one patient. The remainder underwent exploratory
laparotomy and the histological finding was of plasma lymphocytic infiltration of the small intestinal mucosa. All patients responded to oral
tetracycline with complete remission occurring in one case. During the follow-up period, 3 cases had progressive retractable clinical courses but all died 2 to 5 years after the diagnosis. The causes of death in these patients were secondary
bacterial infection (1 case), intestinal
tuberculosis (1 case),
fungal infection (1 case) and
immunoblastic sarcoma in another case. The results of this study confirm the occurrence of
IPSID in Thailand.
IPSID responds to oral
antibiotic therapy and complete remission may be achieved during the early reversible benign phase, thus an awareness of its occurrence is of clinical importance.