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Renal amyloidosis in Behçet’s disease: clinicopathologic features of 8 cases

International Urology and Nephrology volume 45, pages 785–794 (2013)Cite this article

Abstract

Purpose

Behçet’s disease (BD) is a rare inflammatory disorder with variable articular and systemic manifestations. Amyloidosis is an uncommon complication of BD. The aim of this study is to present clinicopathological characteristics and outcome of 8 patients with renal amyloidosis secondary to BD.

Methods

A total of 220 cases of secondary-type renal amyloidosis diagnosed over 26-year period (1981–2007) in a single institution via needle biopsy were reviewed retrospectively, and accompanying BD was found in ten of them. Clinical and follow-up information of the patients was gathered from hospital records and computer-based data system. Two cases were excluded from the study due to another concomitant preamyloidotic condition. Biopsy slides of remaining 8 cases were culled from the archives and re-examined. Extent and pattern of renal amyloid deposition were explored. Amyloid typing was accomplished via immunohistochemistry. Factors acting on outcome and renal survival were explored.

Results

Patients were all male (age range 27–56). Duration of BD before diagnosis of amyloidosis varied from 24 to 192 months, and mean follow-up period was 89 months. Nephrotic syndrome was the most common presentation. Immunohistochemical typing revealed AA-amyloid in all cases. Two cases showed glomerular-dominant, one case vascular-dominant amyloid deposition, and rest were codominant glomerular and vascular, except one that was indeterminate. Patients were treated with steroids and/or different immunosuppressives, three progressed to end-stage kidney failure.

Conclusion

Behçet’s disease is one of the rare diseases that lead to AA amyloidosis. Morphology of renal amyloidosis due to BD has diversities in terms of differential amyloid deposition in renal compartments. Patients follow variable clinical courses accordingly.

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References

  1. Alpsoy E, Donmez L, Onder M, Gunasti S, Usta A, Karincaoglu Y, Kandi B, Buyukkara S, Keseroglu O, Uzun S, Tursen U, Seyhan M, Akman A (2007) Clinical features and natural course of Behcet’s disease in 661 cases: a multicentre study. Br J Dermatol 157:901–906

    PubMed  Article  CAS  Google Scholar 

  2. Tursen U, Gurler A, Boyvat A (2003) Evaluation of clinical findings according to sex in 2313 Turkish patients with Behcet’s disease. Int J Dermatol 42:346–351

    PubMed  Article  Google Scholar 

  3. Pipitone N, Boiardi L, Olivieri I, Cantini F, Salvi F, Malatesta R, La Corte R, Triolo G, Ferrante A, Filippini D, Paolazzi G, Sarzi-Puttini P, Restuccia G, Salvarani C (2004) Clinical manifestations of Behcet’s disease in 137 Italian patients: results of a multicenter study. Clin Exp Rheumatol 22:S46–S51

    PubMed  CAS  Google Scholar 

  4. Criteria for diagnosis of Behcet’s disease (1990) International study group for Behcet’s disease. Lancet 335:1078–1080

    Google Scholar 

  5. Shiiki H, Shimokama T, Yoshikawa Y, Toyoshima H, Kitamoto T, Watanabe T (1988) Renal amyloidosis. Correlations between morphology, chemical types of amyloid protein and clinical features. Virchows Arch A Pathol Anat Histopathol 412:197–204

    PubMed  Article  CAS  Google Scholar 

  6. Verine J, Mourad N, Desseaux K, Vanhille P, Noel LH, Beaufils H, Grateau G, Janin A, Droz D (2007) Clinical and histological characteristics of renal AA amyloidosis: a retrospective study of 68 cases with a special interest to amyloid-associated inflammatory response. Hum Pathol 38:1798–1809

    PubMed  Article  Google Scholar 

  7. Akpolat T, Akkoyunlu M, Akpolat I, Dilek M, Odabas AR, Ozen S (2002) Renal Behcet’s disease: a cumulative analysis. Semin Arthritis Rheum 31:317–337

    PubMed  Article  Google Scholar 

  8. Amr SS, Hamzeh YS (1985) Renal amyloidosis in Jordan. Trop Geogr Med 37:167–170

    PubMed  CAS  Google Scholar 

  9. Demircin G, Delibas A, Bek K, Erdogan O, Bulbul M, Baysun S, Oksal A, Memis L, Oner A (2009) A one-center experience with pediatric percutaneous renal biopsy and histopathology in Ankara, Turkey. Int Urol Nephrol 41:933–939

    PubMed  Article  Google Scholar 

  10. Bergesio F, Ciciani AM, Santostefano M, Brugnano R, Manganaro M, Palladini G, Di Palma AM, Gallo M, Tosi PL, Salvadori M (2007) Renal involvement in systemic amyloidosis–an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant 22:1608–1618

    PubMed  Article  Google Scholar 

  11. Chevrel G, Jenvrin C, McGregor B, Miossec P (2001) Renal type AA amyloidosis associated with rheumatoid arthritis: a cohort study showing improved survival on treatment with pulse cyclophosphamide. Rheumatology (Oxford) 40:821–825

    Article  CAS  Google Scholar 

  12. Chugh KS, Datta BN, Singhal PC, Jain SK, Sakhuja V, Dash SC (1981) Pattern of renal amyloidosis in Indian patients. Postgrad Med J 57:31–35

    PubMed  Article  CAS  Google Scholar 

  13. Ensari C, Ensari A, Tumer N, Ertug E (2005) Clinicopathological and epidemiological analysis of amyloidosis in Turkish patients. Nephrol Dial Transplant 20:1721–1725

    PubMed  Article  Google Scholar 

  14. Paydas S (1999) Report on 59 patients with renal amyloidosis. Int Urol Nephrol 31:619–631

    PubMed  Article  CAS  Google Scholar 

  15. Akpolat T, Dilek M, Aksu K, Keser G, Toprak O, Cirit M, Oguz Y, Taskapan H, Adibelli Z, Akar H, Tokgoz B, Arici M, Celiker H, Diri B, Akpolat I (2008) Renal Behcet’s disease: an update. Semin Arthritis Rheum 38:241–248

    PubMed  Article  Google Scholar 

  16. Akpolat T, Diri B, Oguz Y, Yilmaz E, Yavuz M, Dilek M (2003) Behcet’s disease and renal failure. Nephrol Dial Transplant 18:888–891

    PubMed  Article  CAS  Google Scholar 

  17. Dilsen N, Konice M, Aral O, Erbengi T, Uysal V, Kocak N, Ozdogan E (1988) Behcet’s disease associated with amyloidosis in Turkey and in the world. Ann Rheum Dis 47:157–163

    PubMed  Article  CAS  Google Scholar 

  18. Skhiri H, Mahjoub S, Harzallah O, Achour A, Bouraoui S, Frih A, Dhia NB, El May M (2004) Secondary amyloidosis, a fatal complication of Behcet’s disease: three case reports. Saudi J Kidney Dis Transpl 15:57–60

    PubMed  Google Scholar 

  19. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 84:1–11

    Article  Google Scholar 

  20. Imirzalioglu N, Dursun A, Tastan B, Soysal Y, Yakicier MC (2005) MEFV gene is a probable susceptibility gene for Behcet’s disease. Scand J Rheumatol 34:56–58

    PubMed  Article  CAS  Google Scholar 

  21. Schwartz T, Langevitz P, Zemer D, Gazit E, Pras M, Livneh A (2000) Behcet’s disease in familial Mediterranean fever: characterization of the association between the two diseases. Semin Arthritis Rheum 29:286–295

    PubMed  Article  CAS  Google Scholar 

  22. Touitou I, Magne X, Molinari N, Navarro A, Quellec AL, Picco P, Seri M, Ozen S, Bakkaloglu A, Karaduman A, Garnier JM, Demaille J, Kone-Paut I (2000) MEFV mutations in Behcet’s disease. Hum Mutat 16:271–272

    PubMed  Article  CAS  Google Scholar 

  23. Atagunduz P, Ergun T, Direskeneli H (2003) MEFV mutations are increased in Behcet’s disease (BD) and are associated with vascular involvement. Clin Exp Rheumatol 21:S35–S37

    PubMed  CAS  Google Scholar 

  24. Akpolat T, Yilmaz E, Akpolat I, Dilek M, Karagoz F, Balci B, Ozen S (2002) Amyloidosis in Behcet’s disease and familial Mediterranean fever. Rheumatology (Oxford) 41:592–593

    Article  CAS  Google Scholar 

  25. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, Yurdakul S, Yazici H (2003) The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 82:60–76

    Article  Google Scholar 

  26. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, Faezi T, Ghodsi Z, Faridar A, Ashofteh F, Sadeghi Abdollahi B (2010) Behcet’s disease: from East to West. Clin Rheumatol 29:823–833

    PubMed  Article  Google Scholar 

  27. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, Faezi T, Sadeghi Abdollahi B (2010) How to deal with Behcet’s disease in daily practice. Int J Rheum Dis 13:105–116

    PubMed  Article  Google Scholar 

  28. Shahram F, Nadji A, Jamshidi A, Chams H, Chams C, Shafaie N, Akbarian M, Gharibdoost F, Davatchi F (2004) Behcet’s disease in Iran, analysis of 5,059 cases. Arch Iranian Med 7:9–14

    Google Scholar 

  29. Strege RJ, Saeger W, Linke RP (1998) Diagnosis and immunohistochemical classification of systemic amyloidoses. Report of 43 cases in an unselected autopsy series. Virchows Arch 433:19–27

    PubMed  Article  CAS  Google Scholar 

  30. Satoskar AA, Burdge K, Cowden DJ, Nadasdy GM, Hebert LA, Nadasdy T (2007) Typing of amyloidosis in renal biopsies: diagnostic pitfalls. Arch Pathol Lab Med 131:917–922

    PubMed  Google Scholar 

  31. Kebbel A, Rocken C (2006) Immunohistochemical classification of amyloid in surgical pathology revisited. Am J Surg Pathol 30:673–683

    PubMed  Article  Google Scholar 

  32. Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, Hawkins PN (2007) Natural history and outcome in systemic AA amyloidosis. N Engl J Med 356:2361–2371

    PubMed  Article  CAS  Google Scholar 

  33. Akse-Onal V, Sag E, Ozen S, Bakkaloglu A, Cakar N, Besbas N, Gucer S (2010) Decrease in the rate of secondary amyloidosis in Turkish children with FMF: are we doing better? Eur J Pediatr 169:971–974

    PubMed  Article  Google Scholar 

  34. von Gise H, Helmchen U, Mikeler E, Bruning L, Walther C, Christ H, Mackensen S, Bohle A (1978) Correlations between the morphological and clinical findings in a patient recovering from secondary generalised amyloidosis with renal involvement. Light- and electron microscopic investigations on serial biopsies. Virchows Arch A Pathol Anat Histopathol 379:119–129

    Article  Google Scholar 

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Acknowledgments

This study is a part of a larger study granted by Hacettepe University Scientific Research Unit, Grant number: 08D01101003.

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Authors and Affiliations

  1. Department of Pathology, Hacettepe University Hospital, Sihhiye, Ankara, 06100, Turkey

    Kemal Kosemehmetoglu & Dilek Ertoy Baydar

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  1. Kemal Kosemehmetoglu
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  2. Dilek Ertoy Baydar
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Correspondence to Dilek Ertoy Baydar.

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Kosemehmetoglu, K., Baydar, D.E. Renal amyloidosis in Behçet’s disease: clinicopathologic features of 8 cases. Int Urol Nephrol 45, 785–794 (2013). https://doi.org/10.1007/s11255-012-0268-z

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  • DOI: https://doi.org/10.1007/s11255-012-0268-z

Keywords

  • Behçet’s disease
  • Amyloidosis
  • Kidney
  • Biopsy
  • Pathology