Skip to main content
Gut logoLink to Gut
. 1994 Sep;35(9):1244–1249. doi: 10.1136/gut.35.9.1244

Studies on the enteropathy associated with primary hypogammaglobulinaemia.

K Teahon 1, A D Webster 1, A B Price 1, J Weston 1, I Bjarnason 1
PMCID: PMC1375701  PMID: 7959231

Abstract

Twelve patients with primary hypogammaglobulinaemia with diarrhoea not associated with known microbial pathogens were investigated. Histological evidence of inflammation was common in the stomach and jejunum. Moreover, eight of 10 patients undergoing colonoscopy had low grade 'microscopic colitis' with raised intraepithelial lymphocytes and an intact crypt architecture. Five of 12 patients had small intestinal inflammation on 111indium leucocyte scintigrams and all had increased faecal excretion (normal < 1%) of 111indium (over four days), which varied in intensity from mild (faecal excretion of 111indium = 1-3%) to that comparable with moderately active (7-14.5%) Crohn's disease. Three patients had small intestinal strictures superficially resembling Crohn's disease. Histologically, however, these lacked characteristic diagnostic features of Crohn's disease in two and the third patient had non-steroidal anti-inflammatory drug induced diaphragm like strictures. Six of seven who were most severely symptomatic were successfully treated with an elemental diet with rapid improvement of symptoms. The faecal excretion of 111indium was repeated in five and all improved but histologically the colitis remained unchanged. These studies show that some patients with primary hypogammaglobulinaemia have intestinal inflammation unlike that found in classic inflammatory bowel disease. Elemental diet is a useful temporary measure in the treatment of these patients.

Full text

PDF
1247

Images in this article

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abramowsky C. R., Sorensen R. U. Regional enteritis-like enteropathy in a patient with agammaglobulinemia: histologic and immunocytologic studies. Hum Pathol. 1988 Apr;19(4):483–486. doi: 10.1016/s0046-8177(88)80503-3. [DOI] [PubMed] [Google Scholar]
  2. Ament M. E. Immunodeficiency syndromes and the gut. Scand J Gastroenterol Suppl. 1985;114:127–135. doi: 10.3109/00365528509093773. [DOI] [PubMed] [Google Scholar]
  3. Ammann A. J., Hong R. Selective IgA deficiency: presentation of 30 cases and a review of the literature. Medicine (Baltimore) 1971 May;50(3):223–236. [PubMed] [Google Scholar]
  4. Asquith P., Thompson R. A., Cooke W. T. Serum-immunoglobulins in adult coeliac disease. Lancet. 1969 Jul 19;2(7612):129–131. doi: 10.1016/s0140-6736(69)92440-4. [DOI] [PubMed] [Google Scholar]
  5. Binder H. J., Reynolds R. D. Control of diarrhea in secondary hypogammaglobulinemia by fresh plasma infusions. N Engl J Med. 1967 Oct 12;277(15):802–803. doi: 10.1056/NEJM196710122771507. [DOI] [PubMed] [Google Scholar]
  6. Bjarnason I., Hayllar J., Smethurst P., Price A., Gumpel M. J. Metronidazole reduces intestinal inflammation and blood loss in non-steroidal anti-inflammatory drug induced enteropathy. Gut. 1992 Sep;33(9):1204–1208. doi: 10.1136/gut.33.9.1204. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Bjarnason I., Zanelli G., Smith T., Prouse P., Williams P., Smethurst P., Delacey G., Gumpel M. J., Levi A. J. Nonsteroidal antiinflammatory drug-induced intestinal inflammation in humans. Gastroenterology. 1987 Sep;93(3):480–489. doi: 10.1016/0016-5085(87)90909-7. [DOI] [PubMed] [Google Scholar]
  8. Bookman M. A., Bull D. M. Characteristics of isolated intestinal mucosal lymphoid cells in inflammatory bowel disease. Gastroenterology. 1979 Sep;77(3):503–510. [PubMed] [Google Scholar]
  9. COHEN N., PALEY D., JANOWITZ H. D. Acquired hypogammaglobulinemia and sprue: report of a case and review of the literature. J Mt Sinai Hosp N Y. 1961 Sep-Oct;28:421–427. [PubMed] [Google Scholar]
  10. COLLINS J. R., ELLIS D. S. AGAMMAGLOBULINEMIA, MALABSORPTION AND RHEUMATOID-LIKE ARTHRITIS. Am J Med. 1965 Sep;39:476–482. doi: 10.1016/0002-9343(65)90214-7. [DOI] [PubMed] [Google Scholar]
  11. Dawson J., Bryant M. G., Bloom S. R., Peters T. J. Jejunal mucosal enzyme activities, regulatory peptides and organelle pathology of the enteropathy of common variable immunodeficiency. Gut. 1986 Mar;27(3):273–277. doi: 10.1136/gut.27.3.273. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Dawson J., Hodgson H. J., Pepys M. B., Peters T. J., Chadwick V. S. Immunodeficiency, malabsorption and secretory diarrhea. A new syndrome. Am J Med. 1979 Sep;67(3):540–546. doi: 10.1016/0002-9343(79)90808-8. [DOI] [PubMed] [Google Scholar]
  13. Diaz-Buxo J. A., Hermans P. E., Huizenga K. A. Gastrointestinal dysfunction in immunoglobulin deficiency. Effect of corticosteroids and tetracycline. JAMA. 1975 Sep 15;233(11):1189–1191. [PubMed] [Google Scholar]
  14. Eggert R. C., Wilson I. D., Good R. A. Agammaglobulinemia and regional enteritis. Ann Intern Med. 1969 Sep;71(3):581–585. doi: 10.7326/0003-4819-71-3-581. [DOI] [PubMed] [Google Scholar]
  15. Fillit H., Bernstein L., Davidson M., Brandt L., Bezahler G., Cohen M. Primary acquired hypogammaglobulinemia and regional enteritis. Arch Intern Med. 1977 Sep;137(9):1252–1254. [PubMed] [Google Scholar]
  16. Giardiello F. M., Lazenby A. J., Bayless T. M., Levine E. J., Bias W. B., Ladenson P. W., Hutcheon D. F., Derevjanik N. L., Yardley J. H. Lymphocytic (microscopic) colitis. Clinicopathologic study of 18 patients and comparison to collagenous colitis. Dig Dis Sci. 1989 Nov;34(11):1730–1738. doi: 10.1007/BF01540051. [DOI] [PubMed] [Google Scholar]
  17. HUIZENGA K. A., WOLLAEGER E. E., GREEN P. A., McKENZIE B. F. Serum globulin deficiencies in non-tropical sprue, with report of two cases of acquired agammaglobulinemia. Am J Med. 1961 Oct;31:572–580. doi: 10.1016/0002-9343(61)90141-3. [DOI] [PubMed] [Google Scholar]
  18. Hermans P. E., Diaz-Buxo J. A., Stobo J. D. Idiopathic late-onset immunoglobulin deficiency. Clinical observations in 50 patients. Am J Med. 1976 Aug;61(2):221–237. doi: 10.1016/0002-9343(76)90173-x. [DOI] [PubMed] [Google Scholar]
  19. Hermans P. E., Huizenga K. A. Association of gastric carcinoma with idiopathic late-onset immunoglobulin deficiency. Ann Intern Med. 1972 Apr;76(4):605–609. doi: 10.7326/0003-4819-76-4-605. [DOI] [PubMed] [Google Scholar]
  20. Hermans P. E., Huizenga K. A., Hoffman H. N., Brown A. L., Jr, Markowitz H. Dysgammaglobulinemia associated with nodular lymphoid hyperplasia of the small intestine. Am J Med. 1966 Jan;40(1):78–89. doi: 10.1016/0002-9343(66)90189-6. [DOI] [PubMed] [Google Scholar]
  21. Johnson R. L., VanArsdel P. P., Jr, Tobe A. D., Ching Y. Adult hypogammaglobulinemia with malabsorption and iron deficiency anemia. Am J Med. 1967 Dec;43(6):935–943. doi: 10.1016/0002-9343(67)90252-5. [DOI] [PubMed] [Google Scholar]
  22. Kingham J. G., Levison D. A., Ball J. A., Dawson A. M. Microscopic colitis-a cause of chronic watery diarrhoea. Br Med J (Clin Res Ed) 1982 Dec 4;285(6355):1601–1604. doi: 10.1136/bmj.285.6355.1601. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Kordossis T., Joseph A. E., Gane J. N., Bridges C. E., Griffin G. E. Fecal leukocytosis, indium-111-labelled autologous polymorphonuclear leukocyte abdominal scanning, and quantitative fecal indium-111 excretion in acute gastroenteritis and enteropathogen carriage. Dig Dis Sci. 1988 Nov;33(11):1383–1390. doi: 10.1007/BF01536992. [DOI] [PubMed] [Google Scholar]
  24. Lang J., Price A. B., Levi A. J., Burke M., Gumpel J. M., Bjarnason I. Diaphragm disease: pathology of disease of the small intestine induced by non-steroidal anti-inflammatory drugs. J Clin Pathol. 1988 May;41(5):516–526. doi: 10.1136/jcp.41.5.516. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Lazenby A. J., Yardley J. H., Giardiello F. M., Jessurun J., Bayless T. M. Lymphocytic ("microscopic") colitis: a comparative histopathologic study with particular reference to collagenous colitis. Hum Pathol. 1989 Jan;20(1):18–28. doi: 10.1016/0046-8177(89)90198-6. [DOI] [PubMed] [Google Scholar]
  26. MacDermott R. P., Nash G. S., Bertovich M. J., Mohrman R. F., Kodner I. J., Delacroix D. L., Vaerman J. P. Altered patterns of secretion of monomeric IgA and IgA subclass 1 by intestinal mononuclear cells in inflammatory bowel disease. Gastroenterology. 1986 Aug;91(2):379–385. doi: 10.1016/0016-5085(86)90572-x. [DOI] [PubMed] [Google Scholar]
  27. MacDermott R. P., Stenson W. F. Alterations of the immune system in ulcerative colitis and Crohn's disease. Adv Immunol. 1988;42:285–328. doi: 10.1016/s0065-2776(08)60848-2. [DOI] [PubMed] [Google Scholar]
  28. Mir-Madjlessi S. H., Tavassolie H., Kamalian N. Malakoplakia of the colon and recurrent colonic strictures in a patient with primary hypogammaglobulinemia: an association not previously described. Dis Colon Rectum. 1982 Oct;25(7):723–727. doi: 10.1007/BF02629549. [DOI] [PubMed] [Google Scholar]
  29. SWIFT P. N. Hypogammaglobulinaemia, steatorrhoea and megaloblastic anaemia. Response to gluten-free diet and folic acid. Postgrad Med J. 1962 Nov;38:633–639. doi: 10.1136/pgmj.38.445.633. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Strauss R. G., Ghishan F., Mitros F., Ebensberger J. R., Kisker C. T., Tannous R., Younoszai M. K. Rectosigmoidal colitis in common variable immunodeficiency disease. Dig Dis Sci. 1980 Oct;25(10):798–801. doi: 10.1007/BF01345303. [DOI] [PubMed] [Google Scholar]
  31. Söltoft J., Petersen L., Kruse P. Immunoglobulin deficiency and regional enteritis. Scand J Gastroenterol. 1972;7(3):233–236. doi: 10.3109/00365527209181158. [DOI] [PubMed] [Google Scholar]
  32. Teahon K., Bjarnason I., Pearson M., Levi A. J. Ten years' experience with an elemental diet in the management of Crohn's disease. Gut. 1990 Oct;31(10):1133–1137. doi: 10.1136/gut.31.10.1133. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Teahon K., Smethurst P., Pearson M., Levi A. J., Bjarnason I. The effect of elemental diet on intestinal permeability and inflammation in Crohn's disease. Gastroenterology. 1991 Jul;101(1):84–89. doi: 10.1016/0016-5085(91)90463-u. [DOI] [PubMed] [Google Scholar]
  34. Webster A. D., Slavin G., Shiner M., Platts-Mills T. A., Asherson G. L. Coeliac disease with severe hypogammaglobulinaemia. Gut. 1981 Feb;22(2):153–157. doi: 10.1136/gut.22.2.153. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Wolber R., Owen D., Freeman H. Colonic lymphocytosis in patients with celiac sprue. Hum Pathol. 1990 Nov;21(11):1092–1096. doi: 10.1016/0046-8177(90)90144-t. [DOI] [PubMed] [Google Scholar]
  36. Yardley J. H., Lazenby A. J., Giardiello F. M., Bayless T. M. Collagenous, "microscopic," lymphocytic, and other gentler and more subtle forms of colitis. Hum Pathol. 1990 Nov;21(11):1089–1091. doi: 10.1016/0046-8177(90)90143-s. [DOI] [PubMed] [Google Scholar]

Articles from Gut are provided here courtesy of BMJ Publishing Group

RESOURCES