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Small Bowel Amyloidosis

  • Small Intestine (J Sellin, Section Editor)
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Abstract

Amyloidosis often involves the gastrointestinal tract. The small intestine is the most commonly involved gastrointestinal site. Gastrointestinal manifestations of amyloidosis involvement of the small intestine include diarrhea, gastrointestinal bleeding, and obstruction. High index of suspicion leading to early diagnosis is important in tailoring appropriate therapeutic management of these patients.

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References

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  1. Fonnescu C, Giovinale M, Verrecchia E, et al. Gastrointestinal amyloidosis: a case of chronic diarrhea. Eur Rev Med Pharmacol Sci. 2009;13 suppl 1:45–50.

    Google Scholar 

  2. Falk RH, Comenzo RL, Skinner M. The systemic amloidosis. N Engl J Med. 1997;337(13):898–909.

    Article  PubMed  CAS  Google Scholar 

  3. Kyle RA, Linos A, Beard CM, et al. Incidence and natural history of primary systemic amyloidosis in Olmstead County, Minnesota, 1950 through 1989. Blood. 1992;79:1817–22.

    PubMed  CAS  Google Scholar 

  4. Gilat T, Spiro H, et al. Amyloidosis and the gut. Am J Dig Dis. 1968;13:619–33.

    Article  PubMed  CAS  Google Scholar 

  5. •• Sattianayagam PT, Gillmore JD, Pinney JH, et al. Inflammatory bowel disease and systemic AA amyloidosis. Dig Dis Sci. 2013;58(6):1689–97. Largest series of patients with AA amyloidosis and IBD to date. Provides natural history of disease and outcome of a subset of patients who underwent renal transplantation.

    Article  PubMed  CAS  Google Scholar 

  6. Greenstein AJ, Sachar DB, Panday AK, et al. Amyloidosis and inflammatory bowel disease. A 50-year experience with 25 patients. Medicine (Baltimore). 1992;71:261–70.

    CAS  Google Scholar 

  7. • Guardiola-Arévalo A, Alcántara-Torres M, Valle-Muñoz J, Lorente-Poyatos RH, Romero-Gutiérrez M, Rodríguez-Merlo R, et al. Amyloidosis and Crohn’s disease. Rev Esp Enferm Dig. 2011;103(5):268–74. This article is a case series of 4 patients with Crohn’s disease and secondary amyloidosis, with discussion on the presentation and management of these patients.

    Article  PubMed  Google Scholar 

  8. Bhat A, Selmi C, Naguwa SM, et al. Current concepts on the immunopathology of amyloidosis. Clin Rev Allergy Immunol. 2010;38:97–106.

    Article  PubMed  Google Scholar 

  9. Otsubo S, Kimata N, Okutsu I, et al. Characteristics of dialysis- related amyloidosis in patients on haemodialysis therapy for more than 30 years. Nephrol Dial Transplant. 2009;24(5):1593–8.

    Article  PubMed  Google Scholar 

  10. Yoshimatsu S et al. Endoscopic and pathological manifestation of the gastrointestinal tract in familial amyloidotic polyneuropathy type 1 (Met30). J Intern Med. 1998;243:65–72.

    Article  PubMed  CAS  Google Scholar 

  11. Röcken C, Saeger W, Linke RP. Gastrointestinal amyloid deposits in old age. Report on 110 consecutive auopsical patients and 98 retrospective bioptic specimes. Pathol Res Pract. 1994;190:641–9.

    Article  PubMed  Google Scholar 

  12. Matsuni H, Hoshii Ysetoguchi M, et al. Vascular amyloid of unknown origin and senile transthyretin amyoid in the lung and gastrointestinal tract or old age: histological and immunohistochemial studies. Pathol Int. 2001;51:326–32.

    Article  Google Scholar 

  13. •• Cowan AJ, Skinner M, Seldin DC, et al. Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience. 2013;98(1):141–6. Retrospective review of 76 patients with amyloid involvement of gastrointestinal tract.

  14. Petterson T, Wegelius O. Biopsy diagnosis of amyloidosis in rheumatoid arthritis: malabsorption caused by intestinal amyloid deposits. Gastroenterology. 1972;62:22–7.

    Google Scholar 

  15. Jarnum S. Gastrointestinal haemorrhage and protein losing primary amyloidosis. Gut. 1965;6:14–8.

    Article  PubMed  CAS  Google Scholar 

  16. Koppelman RN, Stollman NH, Baigorri F. Acute small bowel pseudo-obstruction due to AL amyloidosis: a case report and literature review. Am J Gastroenterol. 2000;95(1):294–6.

    Article  PubMed  CAS  Google Scholar 

  17. Tada S, Iida M, Yao T, et al. Intestinal pseudoobstruction in patients with amyloidosis: clinipathologic differences between chemical types of amyloid protein. Gut. 1993;34:1412–7.

    Article  PubMed  CAS  Google Scholar 

  18. Kim SH, Kang EJ, Park JW, et al. Gastrointestinal amyloidosis presenting with multiple episodes of gastrointestinal bleeding. Cardiovasc Intervent Radiol. 2009;32:577–580b.

    Article  PubMed  Google Scholar 

  19. Akbarian M, Fenton J. Perforation of small bowel in amyloidosis. Arch Intern Med. 1962;109:595–600.

    Article  Google Scholar 

  20. Griffel B, Man B, Kraus L. Selective massive amyloidosis of small intestine. Arch Surg. 1975;110(2):215–7.

    Article  PubMed  CAS  Google Scholar 

  21. Sattianayagam PT, Hawkins PN, Gillmore JD. Systemic amyloidosis and the gastrointestinal tract. Nat Rev Gastroenterol Hepatol. 2009;6:608–17.

    Article  PubMed  CAS  Google Scholar 

  22. Sellin JH, Hart R. Glucose malabsorption associated with rapid transit. Am J Gastroenterol. 1992;87(5):584–9.

    PubMed  CAS  Google Scholar 

  23. Guirl MJ, Högenauer C, Santa Ana CA, et al. Rapid intestinal transit as a primary cause of severe chronic diarrhea in patients with amyloidosis. Am J Gastroenterol. 2003;98(10):2219–25.

    Article  PubMed  Google Scholar 

  24. Steen L, Ek B. Familial amyloidosis with polyneuropathy: a long term follow up of 21 patients with special reference to gastrointestinal symptoms. Acta Med Scand. 1983;214(5):387–97.

    Article  PubMed  CAS  Google Scholar 

  25. Gertz MA, Lacy MQ, Dispenzieri A. Amyloidosis. Hematol Oncol Clin North Am. 1999;13:1211–33.

    Article  PubMed  CAS  Google Scholar 

  26. Maeshima E, Yamada Y, Yukawa S. Massive gastrointestinal hemorrhage in a case of amyloidosis secondary to rheumatoid arthritis. Scand J Rheumatol. 1999;28:262–4.

    Article  PubMed  CAS  Google Scholar 

  27. Kaiserling E, Krober S. Massive intestinal hemorrhageassociated with intestinal amyloidosis. An investigation of underlying pathologic processes. Gen Diagn Pathol. 1995;141:147–54.

    PubMed  CAS  Google Scholar 

  28. Högenauer C, Hammer HF. Maldigestion and malabsorption. In: Feldman M, Friedman LS, Brandt LJ, editors. Gastrointestinal and liver disease. 9th ed. Philadelphia: WB Saunder; 2010. p. 1756.

    Google Scholar 

  29. Libbey CA, Skinner M, Cohen AS. Use of abominal fat tissue aspirate in the diagnosis of systemic amyloidosis. Arch Intern Med. 1983;143:1549–52.

    Article  PubMed  CAS  Google Scholar 

  30. Duston MA, Skinner M, Shiranama T, Cohen AS. Diagnosis of amyloidosis by andominal fat aspiration: analysis of four years’ experience. Am J Med. 1987;82:412–4.

    Article  PubMed  CAS  Google Scholar 

  31. Tada S, Iida M, Iwashita A, et al. Endoscopic and biopsy findings of the upper digestive tract in patients with amyloidosis. Gastrointest Endosc. 1990;36(1):10–4.

    Article  PubMed  CAS  Google Scholar 

  32. Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol. 2008;103:776–87.

    Article  PubMed  Google Scholar 

  33. • Yilmaz M, Unsal A, Sokmen M, et al. Duodenal biopsy for diagnosis of renal involvement in amyloidosis. Clin Nephrol. 2012;77(2):114–8. This article highlights that duodenal biopsy is sensitive in detecting amyloidosis in patients with chronic kidney disease.

    Article  PubMed  Google Scholar 

  34. Tada S, Iida M, Yao T, et al. Endoscopic features in amyloidosis of the small intestine: clinical and morphologic differences between chemical types of amyloid protein. Gastrointest Endosc. 1994;40(1):45–50.

    Article  PubMed  CAS  Google Scholar 

  35. Hayman SR, Lacy MQ, Kyle RA. Primary systemic amyloidosis: a cause of malabsorption syndrome. Am J Med. 2001;111:535–40.

    Article  PubMed  CAS  Google Scholar 

  36. Pettersson T, Konttinen YT. Amyloidosis- recent developments. Semin Arthritis Rheum. 2010;39(5):356–68.

    Article  PubMed  CAS  Google Scholar 

  37. Comenzo RL, Vosburgh E, Simms RW, et al. Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: one-year follow-up in five patients. Blood. 1996;88:2801–6.

    PubMed  CAS  Google Scholar 

  38. Dember LM, Sanchorawala V, Seldin DC, et al. Effect of dose-intensive intravenous melphalan and autologous blood stem-cell transplantation on AL amyloidosis-associated renal disease. Ann Intern Med. 2001;134:746–53.

    Article  PubMed  CAS  Google Scholar 

  39. Palladini G, Anesi E, Perfetti V, et al. A modified high dose dexamethasone regmin for primary sestemic (AL) amyloidosis. Br J Haematol. 2001;113:1044–6.

    Article  PubMed  CAS  Google Scholar 

  40. • Fossmark R, Skarsvåg E, Aarset H, Hjorth-Hansen H, Waldum HL. Symptomatic primary (AL) amyloidosis of the stomach and duodenum. Case Rep Gastrointest Med. 2013;2013:525439. A case report of AL amyloidosis involving the stomach and duodenum. Presentation, including endoscopy findings, work-up, management, and disease course are covered.

    PubMed  Google Scholar 

  41. •• Katoh N, Matsuda M, Tsuchiya-Suzuki A, Ikeda S. Regression of gastroduodenal amyloid deposition in systemica AL amyloidosis after intensive chemotherapies. Br J Haematol. 2011;153(4):535–8. A retrospective study evaluating the histopathologic deposition of amyloid in the stomach and duodenum. Amyloid deposits on biopsy decreased in patients who achieved complete hematologic remission.

    Article  PubMed  Google Scholar 

  42. •• Denis MA, Cosyns J-P, Persu A. Control of AA amyloidosis complication Crohn’s disease: a clinico-pathological study. Eur J Clin Invest. 2013;43(3):292–301. A retrospective evaluation of 16 patients with Crohn’s disease and AA amyloidosis with specific focus on immunosuppressive medication and kidney transplantation in managing the disease.

    Article  PubMed  CAS  Google Scholar 

  43. Iizuka M, Konno S, Horie Y. Infliximab as a treatment for systemic amyloidosis associated with Crohn’s disease. Gut. 2006;55:744–74.

    Article  PubMed  CAS  Google Scholar 

  44. Gillmore JD, Lovat LB, Persey MR, et al. Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein. Lancet. 2001;358:24–9.

    Article  PubMed  CAS  Google Scholar 

  45. • Wang Y, Lee H, Shen B. Systemic secondary amyloidosis in a 70- year old patient with Crohn’s disease: treatment with oral budesonide. Inflamm Bowel Dis. 2013;19(5):E74–5. This case report offers another treatment regimen to be considered in patients with IBD and amyloidosis.

    Article  PubMed  Google Scholar 

  46. • Shin J-K, Jung Y-H, Bae M-N. Successful treatment of protein-losing enteropathy due to AA amyloidosis with octreotide in a patient with rheumatoid arthritis. Mod Rheumatol. 2013;23:406–11. A case report of octreotide improving protein-losing enteropathy, after traditional treatment was unsuccessful.

    Article  PubMed  Google Scholar 

  47. •• Coelho T, Adams D, Silva A, et al. Safety and Efficacy of RNAi Therapy for Transthyretic Amyloidosis. N Engl J Med 2013;369:819–29. RNAi therapy for the treatment of transthyretic amyloidosis.

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Milena Gould, Neda Zarrin-Khameh, and Joseph Sellin declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Correspondence to Milena Gould.

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This article is part of the Topical Collection on Small Intestine

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Gould, M., Zarrin-Khameh, N. & Sellin, J. Small Bowel Amyloidosis. Curr Gastroenterol Rep 15, 350 (2013). https://doi.org/10.1007/s11894-013-0350-4

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  • DOI: https://doi.org/10.1007/s11894-013-0350-4

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