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Successful treatment of protein-losing enteropathy due to AA amyloidosis with octreotide in a patient with rheumatoid arthritis

  • Case Report
  • Published:
Modern Rheumatology

Abstract

Protein-losing enteropathy (PLE) is a rare syndrome of gastrointestinal protein loss that may complicate a variety of diseases. This excessive protein loss across the gut epithelium can be explained by several mechanisms, such as augmentation of the intestinal mucosal capillary permeability, mucosal disruption, intestinal or mesenteric vasculitis, and lymphangiectasia. However, these pathophysiologic alterations of the gut are closely linked to the underlying cause, and primary treatment for PLE should be directed at the underlying condition. Here, we report a female patient with rheumatoid arthritis who developed severe PLE due to AA amyloidosis and was successfully treated with octreotide. She had been suffered from rheumatoid arthritis for 18 years, and her arthritic symptoms at the time of presentation were not definite but manifested as severe diarrhea and general edema with hypoalbuminemia. PLE due to gastrointestinal amyloidosis was confirmed by increased fecal α1-antitrypsin clearance and a colonoscopic biopsy that was positive for amyloid deposits. The diarrhea dissipated with conventional treatment, but the general edema resolved only after introducing a long-acting somatostatin analog (octreotide), along with a gradual recovery of the serum albumin level. This case teaches us that in the case of PLE due to AA amyloidosis that is refractory to conventional treatment, the administration of octreotide should be considered.

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References

  1. Umar SB, DiBaise JK. Protein-losing enteropathy: case illustrations and clinical review. Am J Gastroenterol. 2010;105:43–9 (quiz 50).

    Google Scholar 

  2. Fushimi T, Takahashi Y, Kashima Y, et al. Severe protein losing enteropathy with intractable diarrhea due to systemic AA amyloidosis, successfully treated with corticosteroid and octreotide. Amyloid. 2005;12:48–53.

    Article  PubMed  Google Scholar 

  3. Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med. 2003;349:583–96.

    Article  PubMed  CAS  Google Scholar 

  4. Khan MF, Falk RH. Amyloidosis. Postgrad Med J. 2001;77:686–93.

    Article  PubMed  CAS  Google Scholar 

  5. Nakamura T, Amyloid A. Amyloidosis secondary to rheumatoid arthritis: pathophysiology and treatments. Clin Exp Rheumatol. 2011;29:850–7.

    PubMed  Google Scholar 

  6. Ishii W, Kishida D, Suzuki A, et al. A case with rheumatoid arthritis and systemic reactive AA amyloidosis showing rapid regression of amyloid deposition on gastroduodenal mucosa after a combined therapy of corticosteroid and etanercept. Rheumatol Int. 2011;31:247–50.

    Article  PubMed  Google Scholar 

  7. Narvaez J, Hernandez MV, Ruiz JM, Vaquero CG, Juanola X, Nollaa JM. Rituximab therapy for AA-amyloidosis secondary to rheumatoid arthritis. Joint Bone Spine. 2011;78:101–3.

    Article  PubMed  CAS  Google Scholar 

  8. Lee HL, Han DS, Kim JB, Jeon YC, Sohn JH, Hahm JS. Successful treatment of protein-losing enteropathy induced by intestinal lymphangiectasia in a liver cirrhosis patient with octreotide: a case report. J Korean Med Sci. 2004;19:466–9.

    Article  PubMed  Google Scholar 

  9. Yam LT, Oropilla SB. Octreotide for diarrhea in amyloidosis. Ann Intern Med. 1991;115:577.

    PubMed  CAS  Google Scholar 

  10. Jeong YS, Jun JB, Kim TH, et al. Successful treatment of protein-losing enteropathy due to AA amyloidosis with somatostatin analogue and high dose steroid in ankylosing spondylitis. Clin Exp Rheumatol. 2000;18:619–21.

    PubMed  CAS  Google Scholar 

  11. Cunnane G. Amyloid precursors and amyloidosis in inflammatory arthritis. Curr Opin Rheumatol. 2001;13:67–73.

    Article  PubMed  CAS  Google Scholar 

  12. Lachmann HJ, Goodman HJ, Gilbertson JA, et al. Natural history and outcome in systemic AA amyloidosis. N Engl J Med. 2007;356:2361–71.

    Article  PubMed  CAS  Google Scholar 

  13. Gillmore JD, Lovat LB, Persey MR, Pepys MB, Hawkins PN. 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 

  14. Gomez-Casanovas E, Sanmarti R, Sole M, Canete JD, Munoz-Gomez J. The clinical significance of amyloid fat deposits in rheumatoid arthritis: a systematic long-term followup study using abdominal fat aspiration. Arthritis Rheum. 2001;44:66–72.

    Google Scholar 

  15. Obici L, Raimondi S, Lavatelli F, Bellotti V, Merlini G. Susceptibility to AA amyloidosis in rheumatic diseases: a critical overview. Arthritis Rheum. 2009;61:1435–40.

    Article  PubMed  CAS  Google Scholar 

  16. Wakhlu A, Krisnani N, Hissaria P, Aggarwal A, Misra R. Prevalence of secondary amyloidosis in Asian North Indian patients with rheumatoid arthritis. J Rheumatol. 2003;30:948–51.

    PubMed  Google Scholar 

  17. Koivuniemi R, Paimela L, Suomalainen R, Tornroth T, Leirisalo-Repo M. Amyloidosis is frequently undetected in patients with rheumatoid arthritis. Amyloid. 2008;15:262–8.

    Article  PubMed  CAS  Google Scholar 

  18. Okuda Y, Takasugi K, Oyama T, Onuma M, Oyama H. Amyloidosis in rheumatoid arthritis—clinical study of 124 histologically proven cases. Ryumachi. 1994;34:939–46.

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  20. Suzuki C, Higaki S, Nishiaki M, et al. 99mTc-HSA-D scintigraphy in the diagnosis of protein-losing gastroenteropathy due to secondary amyloidosis. J Gastroenterol. 1997;32:78–82.

    Article  PubMed  CAS  Google Scholar 

  21. Okuda Y, Takasugi K, Oyama T, Oyama H, Nanba S, Miyamoto T. Intractable diarrhoea associated with secondary amyloidosis in rheumatoid arthritis. Ann Rheum Dis. 1997;56:535–41.

    Article  PubMed  CAS  Google Scholar 

  22. Kuroda T, Otaki Y, Sato H, et al. A case of AA amyloidosis associated with rheumatoid arthritis effectively treated with infliximab. Rheumatol Int. 2008;28:1155–9.

    Article  PubMed  Google Scholar 

  23. Yazici Y, Erkan D, Levine DM, Parker TS, Lockshin MD. Protein-losing enteropathy in systemic lupus erythematosus: report of a severe, persistent case and review of pathophysiology. Lupus. 2002;11:119–23.

    Article  PubMed  CAS  Google Scholar 

  24. Bhattacharya S, Vijayasekar C, Worlding J, Mathew G. Octreotide in chemotherapy induced diarrhoea in colorectal cancer: a review article. Acta Gastroenterol Belg. 2009;72:289–95.

    PubMed  CAS  Google Scholar 

  25. Szilagyi A, Shrier I. Systematic review: the use of somatostatin or octreotide in refractory diarrhoea. Aliment Pharmacol Ther. 2001;15:1889–97.

    Article  PubMed  CAS  Google Scholar 

  26. Kubba AK, Dallal H, Haydon GH, Hayes PC, Palmer KR. The effect of octreotide on gastroduodenal blood flow measured by laser Doppler flowmetry in rabbits and man. Am J Gastroenterol. 1999;94:1077–82.

    Article  PubMed  CAS  Google Scholar 

  27. Reubi JC, Laissue JA, Waser B, Steffen DL, Hipkin RW, Schonbrunn A. Immunohistochemical detection of somatostatin sst2a receptors in the lymphatic, smooth muscular, and peripheral nervous systems of the human gastrointestinal tract: facts and artifacts. J Clin Endocrinol Metab. 1999;84:2942–50.

    Article  PubMed  CAS  Google Scholar 

  28. Elliott DE, Li J, Blum AM, Metwali A, Patel YC, Weinstock JV. SSTR2A is the dominant somatostatin receptor subtype expressed by inflammatory cells, is widely expressed and directly regulates T cell IFN-gamma release. Eur J Immunol. 1999;29:2454–63.

    Article  PubMed  CAS  Google Scholar 

  29. Karalis K, Mastorakos G, Sano H, Wilder RL, Chrousos GP. Somatostatin may participate in the antiinflammatory actions of glucocorticoids. Endocrinology. 1995;136:4133–8.

    Article  PubMed  CAS  Google Scholar 

  30. Paran D, Elkayam O, Mayo A, et al. A pilot study of a long acting somatostatin analogue for the treatment of refractory rheumatoid arthritis. Ann Rheum Dis. 2001;60:888–91.

    PubMed  CAS  Google Scholar 

  31. Hachulla E, Grateau G. Diagnostic tools for amyloidosis. Joint Bone Spine. 2002;69:538–45.

    Article  PubMed  Google Scholar 

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Correspondence to Ki-Jo Kim.

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Shin, JK., Jung, YH., Bae, MN. et al. Successful treatment of protein-losing enteropathy due to AA amyloidosis with octreotide in a patient with rheumatoid arthritis. Mod Rheumatol 23, 406–411 (2013). https://doi.org/10.1007/s10165-012-0675-0

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  • DOI: https://doi.org/10.1007/s10165-012-0675-0

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