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Decreased protein S activity is related to the disease activity of Behcet’s disease

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Abstract

We wanted to see whether the active inflammation in Behcet’s disease (BD) can cause a thrombotic disorder by decreasing the protein S (PS) activity, and we evaluated the relationship between the decreased PS activity and the disease activity of BD. We included 122 patients with BD whose PS activity levels were measured. In 51 patients, the PS activity was measured again when there were changes in the number of items of “The Behcet’s Disease Current Activity Form (BDCAF)”. The thrombosis rate was 2.5% (3/122), and the PS activity was low in all three of the patients with thrombosis. The incidence of low PS activity in the total 122 BD patients was 27% (33/122). The incidence of the low PS activity in the active BD patients was 33.7% (31/92), and this was significantly more frequent than in the inactive BD patients, (6.7%, 2/30) (χ2-test, P value = 0.0038). The decrease of PS activity had good correlation with the increase of the number of BDCAF items (r = −0.351, P value = 0.012). The PS activity decrease is related to the BD activity. The low PS activity can be the risk factor for thrombotic disorder and also the activity marker for BD and other inflammatory diseases.

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References

  1. Kaklamani VG, Vaiopoulos G, Kaklamanis PG (1998) Behcet’s disease. Semin Arthritis Rheum 27:197–217

    Article  PubMed  CAS  Google Scholar 

  2. Kluft C, Michiels JJ, Wijngaards G (1980) Factual or artificial inhibition of fibrinolysis and the occurrence of venous thrombosis in 3 cases of Behcet’s disease. Scand J Haematol 25:423–430

    Article  PubMed  CAS  Google Scholar 

  3. Koc Y, Gullu I, Akpek G, Akpolat T, Kiraz S, Batman F et al (1992) Vascular involvement in Behcet’s disease. J Rheumatol 19:402–410

    PubMed  CAS  Google Scholar 

  4. Lie JT (1992) Vascular involvement in Behcet’s disease: arterial and venous and vessels of all sizes. J Rheumatol 19:341–343

    PubMed  CAS  Google Scholar 

  5. Wechsler B, Vidailhet M, Piette JC, Bousser MG, Dell IB, Blentry O et al (1992) Cerebral venous thrombosis in Behcet’s disease: clinical study and long-term follow-up of 25 cases. Neurol 42:614–618

    CAS  Google Scholar 

  6. Hampton KK, Chamberlain MA, Menon DK, Davis JA (1991) Coagulation and fibrinolytic activity in Behcet’s disease. Thromb Haemost 66:292–294

    PubMed  CAS  Google Scholar 

  7. Ozoran K, Duzgun N, Gurler A, Tutkak H, Tokgoz G (1995) Plasma von Willebrand factor, tissue plasminogen activator, plasminogen activator inhibitor, and antithrombin III levels in Behcet’s disease. Scand J Rheumatol 24:376–382

    PubMed  CAS  Google Scholar 

  8. Oner AF, Gurgey A, Gurler A, Mesci L (1998) Factor V Leiden mutation in patients with Behcet’s disease. J Rheumatol 25:496–498

    PubMed  CAS  Google Scholar 

  9. Gul A, Ozbek U, Ozturk C, Inanc M, Konkce M, Ozcelik T (1996) Coagulation factor V mutation increases the risk of venous thrombosis in Behcet’s disease. Br J Rheumatol 35:1178–1180

    Article  PubMed  CAS  Google Scholar 

  10. Mader R, Ziv M, Adawi M, Mader R, Lavi I (1992) Thrombophilic factors and their relation to thromboembolic and other clinical manifestations in Behcet’s disease. J Rheumatol 26:2404–2408

    Google Scholar 

  11. Sengul N, Demirer S, Yerdel MA, Terzioglu G, Akin B, Gurler A et al (2000) Comparison of coagulation parameters for healthy subject and Behcet’s disease patients with and without vascular involvement. World J Surg 24:1584–1588

    Article  PubMed  CAS  Google Scholar 

  12. Demier S, Sengul N, Yerder MA, Tuzuner A, Ulus AT, Gurler A et al (2000) Haemostatis in patients with Behcet’s disease. Eur J Vasc Endovasc Surg 19:570–574

    Article  Google Scholar 

  13. Borgel D, Gandrille S, Aiach M (1997) Protein S deficiency. Thromb Haemost 78:351–356

    PubMed  CAS  Google Scholar 

  14. Lee JH, Kim SW, Kim JS (2000) Sagittal sinus thrombosis associated with transient free protein S deficiency after l-asparaginase treatment: case report and review of the literature. Clin Neurol Neurosurg 102:33–36

    Article  PubMed  CAS  Google Scholar 

  15. D’Angelo A, Valle PD, Crippa L, Pattarini E, Grimaldi L, D’Angelo SV (1993) Autoimmune protein S deficiency in a boy with severe thromboemblimc disease. N Engl J med 328:1753–1757

    Article  PubMed  CAS  Google Scholar 

  16. Stahl CP, Wideman CS, Spira TJ, Haff EC, Hixon GJ, Evatt BL (1993) Protein S deficiency in men with long-term human immunodeficiency virus infection. Blood 81:1801–1807

    PubMed  CAS  Google Scholar 

  17. Nguyen P, Reynaud J, Pouzol P, Munzer M, Richard O, Francois P (1994) Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children. Eur J Pediatr 153:646–649

    Article  PubMed  CAS  Google Scholar 

  18. Deitcher SR, Erban JK, Limentani SA (1996) Acquired free protein S deficiency associated with multiple myeloma: a case report. Am J Hematol 51:319–323

    Article  PubMed  CAS  Google Scholar 

  19. Song KS, Park YS, Kim HK (2000) Prevalence of anti-protein S antibodies in patients with systemic lupus erythematosus. Arthritis Rheum 43:557–560

    Article  PubMed  CAS  Google Scholar 

  20. International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. Lancet 335:1078–1080

    Google Scholar 

  21. Lawton G, Bhakta BB, Chamberlain MA, Tennant A (2004) The Behcet’s Disease activity index. Rheumatol 43:73–78

    Article  CAS  Google Scholar 

  22. Lundwall A, Dackowski W, Cohen E, Shaffer M, Mahr A, Dahlback B et al (1986) Isolation and sequence of the cDNA for human protein S, a regulator of blood coagulation. Proc Natl Acad Sci USA 83:6716–6720

    Article  PubMed  CAS  Google Scholar 

  23. Webb JH, Blom AM, Dahlback B (2002) Vitamin K-dependent protein S localizing complement regulator C4b-binding protein to the surface of apoptotic cells. J Immunol 169:2580–2586

    PubMed  CAS  Google Scholar 

  24. Anderson HA, Maylock CA, Williams JA, Paweletz CP, Shu H, Shacter E (2003) Serum-derived protein S binds to phosphatidylserine and stimulates the phagocytosis of apoptotic cells. Nat immunol 4:87–91

    Article  PubMed  CAS  Google Scholar 

  25. Ohkohchi K, Tornuki W, Tagami H (1989) Plasma concentration of complement-modulation proteins (C1 inhibitor, C4 binding protein, factor H and factor I) in inflammatory dermatoses with special reference to psoriasis. Dermatologica 179(Suppl 1):30–34

    Article  PubMed  Google Scholar 

  26. Kim HA, Choi KW, Song YW (1997) Arthropathy in Behcet’s disease. Scand J Rheumatol 26:125–129

    Article  PubMed  CAS  Google Scholar 

  27. Lee YJ, Kang SW, Yang JI, Choi YM, Sheen D, Lee EB et al (2002) Coagulation parameters and plasma total homocysteine levels in Behcet’s disease. Thromb Res 106:19–24

    Article  PubMed  CAS  Google Scholar 

  28. Ozatli D, Sayinalp N, Buyukasik Y, Karakus S, Haznedaroglu IC, Kirazli S et al (2002) Unchanged global fibrinolytic capacity despite increased factor VIIa activity in Behcet’s disease: evidence of a prethrombotic state. Rheumatol Int 21:137–140

    Article  PubMed  CAS  Google Scholar 

  29. Yazici C, Kose K, Calis M, Demlr M, Kirnap M, Ates F (2004) Increased advanced oxidation protein products in Behcet’s disease: a new activity marker? Br J Dermatol 151:105–111

    Article  PubMed  CAS  Google Scholar 

  30. Duzgun N, Ayaslioglu E, Tutkak H (2004) Serum soluble CD30 levels in Behcet’s disease. Clin Exp Rheumatol 22(Suppl 34):S17–20

    PubMed  CAS  Google Scholar 

  31. Ozoran K, Duzgun N, Tutkak H, Gurler A, Tokgoz G (1996) Fibronectin and circulation immune complexes in Behcet’s disease. Rheumatol Int 15:221–224

    Article  PubMed  CAS  Google Scholar 

  32. Chang HK, Cheon KS (2002) The clinical significance of a pathergy reaction in patients with Behcet’s disease. J Korean Med Sci 17:371–374

    PubMed  Google Scholar 

  33. Hamuryndan V, Fresko I, Direskeneli H, Tenant MJ, Yurdakul S, Akoglu T et al (1999) Evaluation of the Turkish translation of a disease activity form for Behcet’s syndrome. Rheumatol 38:374–376

    Google Scholar 

  34. Bhakta BB, Brennan P, James TE, Chamberlain MA, Noble BA, Silman AJ (1999) Behcet’s disease: evaluation of a new instrument to measure clinical activity. Rheumatol 38:728–733

    Article  CAS  Google Scholar 

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Correspondence to Won Park.

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This work was supported by Inha University Research Grant.

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Kwon, S.R., Lim, M.J., Park, S.G. et al. Decreased protein S activity is related to the disease activity of Behcet’s disease. Rheumatol Int 27, 39–43 (2006). https://doi.org/10.1007/s00296-006-0214-4

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  • DOI: https://doi.org/10.1007/s00296-006-0214-4

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