Is immunoproliferative small intestinal disease uncommon in India?

Till date only three series of immunoproliferative small intestinal disease (IPSID) describing 22 patients have been reported from India. Seven patients with IPSID in two tertiary referral centers in India are included in the study. Diagnosis was based on typical clinical features [diarrhoea (7/7), weight loss (7/7), clubbing (6/7), fever (3/7), abdominal pain and lump (3/7)], biochemical evidence of malabsorption and duodenal biopsy findings. All patients were young males (mean age 29.8 +/- 11.8 years, range 17-53). Atypical features included gastric involvement (1/7), colonic involvement (1/7) and appearance of pigmented nails following anti-cancer chemotherapy (1/7) which disappeared six months after omitting doxorubin from chemotherapy regimen. Parasitic infestation was common. Ascaris lumbricoides (1/7), Giardia lamblia and hookworm (1/7), Strongyloides stercoralis and Trichuris trichura (1/7). In the latter patient S. stercoralis became disseminated after anti-malignant chemotherapy. One patient had gastric H. pylori infection. Four of the seven patients who were misdiagnosed as tropical sprue were treated with tetracycline. This raises doubt on efficacy of tetracycline alone in treatment of IPSID. One other patient was misdiagnosed and treated as intestinal tuberculosis. Early diagnosis and administration of chemotherapy may improve survival in this disease.