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Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

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Modern Rheumatology

Abstract

An association between thrombotic thrombocytopenic purpura (TTP) and Sjögren’s syndrome (SS) is rare. This is the first report of two patients with TTP who had inhibitory autoantibodies to ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) complicating primary SS. A rapid diagnosis of TTP, which is a potentially lethal condition, made it possible to treat the two cases successfully. Only eight similar cases with TTP complicating SS have been reported in the literature. The possible presentation of primary SS without classic sicca symptoms, but with haematological abnormalities including TTP, should be recognised. Furthermore, it is important to measure ADAMTS13 activity and anti-ADAMTS13 antibodies, because TTP with SS seems to be a concurrent overlapping autoimmune disorder. We suggest that plasma exchanges in combination with corticosteroids should be administered as early as possible, since they appeared to be effective in treating TTP with SS, including in our cases.

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References

  1. Remuzzi G. HUS and TTP. Variable expression of a single entity. Kidney Int. 1987;32:292–308.

    Article  PubMed  CAS  Google Scholar 

  2. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001;98:1662.

    Article  PubMed  CAS  Google Scholar 

  3. Levy GG, Motto DG, Ginsburg D. ADAMTS13 turns 3. Blood. 2005;106:11.

    Google Scholar 

  4. Remuzzi G, Ruggenenti P. The hemolytic uremic syndrome. Kidney Int. 1995;48:2.

    Article  PubMed  CAS  Google Scholar 

  5. Chow TW, Turner NA, Chintagumpala M, et al. Increased von Willebrand factor binding to platelets in single episode and recurrent types of thrombotic thrombocytopenic purpura. Am J Hematol. 1998;57:293.

    Article  PubMed  CAS  Google Scholar 

  6. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585.

    Article  PubMed  CAS  Google Scholar 

  7. Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413:488.

    Article  PubMed  CAS  Google Scholar 

  8. Licht C, Stapenhorst L, Simon T, et al. Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS). Kidney Int. 2004;66:955.

    Article  PubMed  CAS  Google Scholar 

  9. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood. 2004;103:4043.

    Article  PubMed  CAS  Google Scholar 

  10. Rieger M, Mannucci PM. Kremer Hovinga JA, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood. 2005;106:1262.

    Article  PubMed  CAS  Google Scholar 

  11. Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren’s syndrome: new clinical and therapeutic concepts. Ann Rheum Dis. 2005;64:347.

    Article  PubMed  CAS  Google Scholar 

  12. Theander E, Jacobsson LT. Relationship of Sjögren’s syndrome to other connective tissue and autoimmune disorders. Rheum Dis Clin North Am. 2008;34:935.

    Article  PubMed  Google Scholar 

  13. Thompson CE, Damon LE, Ries CA, Linker CA. Thrombotic microangiopathies in the 1980s: clinical features, response to treatment, and the impact of the human immunodeficiency virus epidemic. Blood. 1992;80:1890–5.

    PubMed  CAS  Google Scholar 

  14. Kasper CK, Pool JG. Letter: measurement of mild factor VIII inhibitors in Bethesda units. Thromb Diath Haemorrh. 1975;34:875–6.

    PubMed  CAS  Google Scholar 

  15. Kato S, Matsumoto M, Matsuyama T, Isonishi A, Hiura H, Fujimura Y. Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity. Transfusion. 2006;46:1444–52.

    Article  PubMed  CAS  Google Scholar 

  16. Pierangeli SS, Harris EN. Clinical laboratory testing for the antiphospholipid syndrome. Clin Chim Acta. 2005;357:17–33.

    Article  PubMed  CAS  Google Scholar 

  17. Parker Levine S. Thrombotic thrombocytopenic purpura and other forms of nonimmunologic platelet destruction. In: Wintrobe’s clinical hematology, 10th ed. Baltimore: Williams & Wilkins; 1999. p. 1612–22.

  18. Caramaschi P, Riccetti MM, Pasini AF, et al. Systemic lupus erythematosus and thrombotic thrombocytopenia purpura. Report of three cases and review of the literature. Lupus. 1998;7:37–41.

    Article  PubMed  CAS  Google Scholar 

  19. Brunner HI, Freedman M, Silverman ED. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenia purpura in childhood. Arthritis Rheum. 1999;42:2346–55.

    Article  PubMed  CAS  Google Scholar 

  20. Towheed TE, Anastassiades TP, Ford SE, Ford PM, Lee P. Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis. J Rheumatol. 1999;26:1613–6.

    PubMed  CAS  Google Scholar 

  21. Hovinga JA, Vesely SK, Terrell DR, et al. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115:1500.

    Article  Google Scholar 

  22. Koga T, Yamasaki S, Nakamura H, Kawakami A, Furusu A, Taguchi T, Eguchi K. Renal thrombotic microangiopathies/thrombotic thrombocytopenic purpura in a patient with primary Sjögren’s syndrome complicated with IgM monoclonal gammopathy of undetermined significance. Rheumatol Int. 2010. (Epub ahead of print).

  23. Schattner A, Friedman J, Klepfish A. Thrombotic thrombocytopenic purpura as an initial presentation of primary Sjögren’s syndrome. Clin Rheumatol. 2002;21:57–9.

    Article  PubMed  CAS  Google Scholar 

  24. Settefrati N, Bonomini M, Lodi M, D’Andrea T, Angelone A, Amoroso L, Palmieri PF, Albertazzi A. Sjögren’s syndrome associated with haemolytic-uraemic syndrome as the presenting clinical manifestation. Nephrol Dial Transpl. 1996;11:2316–9.

    Article  CAS  Google Scholar 

  25. Abe H, Tsuboi N, Yukawa S, Tsuji S, Hayashi H, Yukawa N, Takanashi H, Tahara K, Tonozuka N, Hayashi T. Thrombotic thrombocytopenic purpura complicating Sjögren’s syndrome with crescentic glomerulonephritis and membranous nephritis. Mod Rheumatol. 2004;14:174–8.

    Article  PubMed  Google Scholar 

  26. Campbell GN, Gallo JH. Relapsing thrombotic thrombocytopenic purpura (TTP) in Sjögren’s syndrome. Aust N Z J Med. 1998;28:214.

    Article  PubMed  CAS  Google Scholar 

  27. Noda M, Kitagawa M, Tomoda F, Iida H. Thrombotic thrombocytopenic purpura as a complicating factor in a case of polymyositis and Sjögren’s syndrome. Am J Clin Pathol. 1990;94:217–21.

    PubMed  CAS  Google Scholar 

  28. Steinberg AD, Green WT Jr, Talal N. Thrombotic thrombocytopenic purpura complicating Sjögren’s syndrome. JAMA. 1971;215:757–61.

    Article  PubMed  CAS  Google Scholar 

  29. Agapitos E, Pavlopoulos PM, Vaiopoulos G, Davaris P. Primary Sjögren’s syndrome complicated by hemolytic uremic syndrome. Scand J Rheumatol. 1996;25:263–5.

    Article  PubMed  CAS  Google Scholar 

  30. George JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med. 2006;354:1927.

    Article  PubMed  CAS  Google Scholar 

  31. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood. 2004;103:4043.

    Article  PubMed  CAS  Google Scholar 

  32. Eknoyan G, Riggs SA. Renal involvement in patients with thrombotic thrombocytopenic purpura. Am J Nephrol. 1986;6:117.

    Article  PubMed  CAS  Google Scholar 

  33. Hebert LA, Cosio FG, Neff JC. Diagnostic significance of hypocomplementemia. Kidney Int. 1991;39:811.

    Article  PubMed  CAS  Google Scholar 

  34. Burns ER, Zucker-Franklin D. Pathologic effects of plasma from patients with thrombotic thrombocytopenic purpura on platelets and cultured vascular endothelial cells. Blood. 1982;60:1030–7.

    PubMed  CAS  Google Scholar 

  35. Feldman JD, Mardiney MR, Unanue ER, Cutting H. The vascular pathology of thrombotic thrombocytopenic purpura: an immunohistochemical and ultrastructural study. Lab Invest. 1966;15:927–46.

    PubMed  CAS  Google Scholar 

  36. Remuzzi G, Ruggenenti P, Bertani T. Thrombotic microangiopathy. In: Tisher CC, Brenner BM, editors. Renal pathology: with clinical and functional correlations. 2nd ed. Philadelphia: Lippincott; 1994. p. 1162–4.

    Google Scholar 

  37. Kaufman I, Schwartz D, Caspi D, Paran D. Sjögren’s syndrome—not just Sicca: renal involvement in Sjögren’s syndrome. Scand J Rheumatol. 2008;37:213–8.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Rheumatic Diseases, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku, Tokyo, 162-8655, Japan

    Hiroyuki Yamashita, Yuko Takahashi, Hiroshi Kaneko, Toshikazu Kano & Akio Mimori

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  1. Hiroyuki Yamashita
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  2. Yuko Takahashi
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  3. Hiroshi Kaneko
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  4. Toshikazu Kano
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  5. Akio Mimori
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Correspondence to Hiroyuki Yamashita.

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Yamashita, H., Takahashi, Y., Kaneko, H. et al. Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review. Mod Rheumatol 23, 365–373 (2013). https://doi.org/10.1007/s10165-012-0644-7

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  • DOI: https://doi.org/10.1007/s10165-012-0644-7

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