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THU0329 Budd-chiari syndrome in behÇet's disease: a retrospective multicenter study
  1. L Akyol1,
  2. B Toz2,
  3. Ö Bayındır3,
  4. O Zengin4,
  5. D Üsküdar Cansu5,
  6. M Yiğit6,
  7. G Çetin Yıldırım7,
  8. A Omma8,
  9. A Erden9,
  10. O Küçükşahin10,
  11. MS Altuner1,
  12. BS Çorba11,
  13. AU Ünal12,
  14. H Küçük13,
  15. A Küçük14,
  16. A Balkarlı15,
  17. E Gönüllü16,
  18. AN Tufan17,
  19. S Üreyen Bakırcı18,
  20. S Öner Yılmaz19,
  21. S Kobak20,
  22. A Yazıcı21,
  23. M Özgen1,
  24. A Şahin22,
  25. SS Koca23,
  26. B Erer2,
  27. A Gül2,
  28. K Aksu3,
  29. G Keser3,
  30. AM Onat4,
  31. B Kısacık4,
  32. T Kaşifoğlu5,
  33. A Çefle21,
  34. U Kalyoncu9,
  35. M Sayarlıoğlu1
  1. 1Internal Medicine, Div. of Rheumatology, Ondokuz Mayıs University School of Medicine, Samsun
  2. 2Internal Medicine, Div. of Rheumatology, Istanbil University School of Medicine, Istanbil
  3. 3Internal Medicine, Div. of Rheumatology, Ege University School of Medicine, Izmir
  4. 4Internal Medicine, Div. of Rheumatology, Gaziantep University School of Medicine, Gaziantep
  5. 5Internal Medicine, Div. of Rheumatology, Osmangazi University School of Medicine, Eskişehir
  6. 6Internal Medicine, Div. of Rheumatology, Pamukkale University School of Medicine, Denizli
  7. 7Internal Medicine, Div. of Rheumatology, Sütçü İmam University School of Medicine, Kahramanmaraş
  8. 8Internal Medicine, Div. of Rheumatology, Numune Hospital
  9. 9Internal Medicine, Div. of Rheumatology, Hacettepe University School of Medicine
  10. 10Internal Medicine, Div. of Rheumatology, Yıldırım Beyazıt University School of Medicine, Ankara
  11. 11Statistics, Ondokuz Mayıs University School of Medicine, Samsun
  12. 12Internal Medicine, Div. of Rheumatology, Marmara University School of Medicine, Istanbil
  13. 13Internal Medicine, Div. of Rheumatology, Gazi University School of Medicine, Ankara
  14. 14Internal Medicine, Div. of Rheumatology, Necmettin Erbakan University School of Medicine, Konya
  15. 15Internal Medicine, Div. of Rheumatology, Antalya Training and research hospital, Antalya
  16. 16Internal Medicine, Div. of Rheumatology, State hospital, Eskişehir
  17. 17Internal Medicine, Div. of Rheumatology, Uludağ University School of Medicine, Bursa
  18. 18Internal Medicine, Div. of Rheumatology, Training and Research hospital, Sakarya
  19. 19Internal Medicine, Div. of Rheumatology, Bakırköy Dr. Sadi Konuk Training and Research Hospital
  20. 20Internal Medicine, Div. of Rheumatology, Liv Hospital, Istanbil
  21. 21Internal Medicine, Div. of Rheumatology, Kocaeli University School of Medicine, Kocaeli
  22. 22Internal Medicine, Div. of Rheumatology, Cumhuriyet University School of Medicine, Sivas
  23. 23Internal Medicine, Div. of Rheumatology, Fırat University School of Medicine, Elazığ, Turkey

Abstract

Background The aim of this study was to determine the demographic, clinical, laboratory and management characteristics along with the clinical course of Budd-Chiari syndrome (BCS) associated with Behçet's disease (BD).

Methods Sixty patients with BD with BCS (40 male, 20 female) were identified in 23 rheumatology centers (Group I). A total of 169 consecutive patients (100 male, 69 female) with BD who did not have clinically apparent BCS during the follow-up were evaluated as the control group (Group II).

Results Comparison of the demographic and clinical findings between the Group I and the Group II were as follows: The mean age of disease onset was 23.1 +/- 6.7 years vs. 26.8±0.6 years (p=0.013), mean age at diagnosis was 27.2±0.9 vs. 30.4±0.6 years (p=0.008), arthritis was 10% vs. 28.4% (p=0.002), papulopustular skin lesion was 48.3% vs 69.2% (p=0.003), central nervous system (CNS) involvement 10% vs. 3% (p=0.03), cardiac involvement was 16.7% vs. 2.4% (p<0.001), superficial thrombophlebitis was 23.3% vs. 4.7% (p<0.001), and deep vein thrombosis was 58.3% vs. 15.4% (p<0.01). On diagnosis 50% of BD patients with BCS were classified as Child-Pugh A. Inferior vena cava obstruction was observed in 38.3% and portal vein thrombosis was seen in 3.3% of the patients with BCS. Mortality in BCS patients with BD was 18.3%. BCS related treatment after diagnosis in patients with BD were as follows: 71.7% of patients were treated with monthly cyclophosphamide intravenous pulses, 53.3% received intravenous pulse corticosteroids, 55.9% used azathioprine, 54.2% had warfarine treatment, and 50.8% were treated with low molecular weight heparin.

Conclusions This study shows a higher frequency of cardiac and CNS involvement, superficial thrombophlebitis, papulopustular skin lesion, deep vein thrombosis in BD patients with BCS. Arthritis was observed less common in BD patients with BCS. The mean age onset was lower in patients with BCS. Medical treatment with immunosuppressive agents and anticoagulation appears to be the treatment of choice in BD patients with BCS. The majority of the patients with BCS were Child–Pugh class A on diagnosis. The inferior vena cava is frequently involved and, often associated with deep vein thrombosis and cardiac involvement.

Disclosure of Interest None declared

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