BEHÇET HASTALIĞI'NDA PULMONER ARTER ANEVRİZMASI VE İNTRAKARDİAK LEZYONUN BİRLİKTELİĞİ: 2 OLGU SUNUMU

Yıl 2005, Cilt: 19 Sayı: 2, 57 - 63, 01.10.2005

Hamide Aydoğdu Emel Özden

Öz

Behçet Hastalığı tekrarlayan ağız içi ve genital ülserler ve göz tutulumu ile karekterize tüm sistemleri tutabilen bir hastalıktır. Genellikle hastalığın eksaserbasyonlarında akciğerler tutulabilir ve pulmoner arteriyel tıkanmalara bağlı ölümcül olabilir. Pulmoner arter anevrizması (PAA) ve intrakardiak trombüsün (İKT) birlikte görüldüğü 2 Behçet olgusu sunuyoruz. 28 ve 32 yaşlarında 2 erkek olgu öksürük, hemoptizi, nefes darlığı ve halsizlik yakınması ile başvurdular. Her iki olguda da iki taraflı inferior pulmoner arterlerde anevrizmatik dilatasyon ve emboli ile birlikte sağ ventrik ülde trombüs vardı. Olgunun birisinde sağ ventriküldeki trombüs cerrahi olarak çıkarılmıştı. Her iki olguya da kortikosteroid ve siklofosfamid den oluşan immunosupresif tedavi verildi. Trombüsün cerrahi olarak çıkarıldığı olgu immunsupresif tedavinin dokuzuncu ayında abondan hemoptizi ile kaybedildi. Diğer olgunun tedavisi tamamlandı ve hala yaşıyor ve durumu iyi. Her iki olguda da immunosupresif tedavi ile anevrizmalarda belirgin düzelme saptanmasına rağmen olgunun birisinin ölmesi, Behçet Hastalığı'na bağlı vaskülitlerde tedaviye rağmen mortalitenin yüksek olduğunu gösterir.

Anahtar Kelimeler

Behçet hastalığı, pulmoner arter anevrizması, intrakardiak trombüs

INTRACARDIAC THROMBUS AND CO-EXISTING PULMONARY ARTERY ANEURYSM IN BEHÇET'S DISEASE: TWO CASE REPORTS Enver YALNIZ Berna KÖMÜRCÜOĞLU Mine GAYAF

Öz

Behçet's di sease is a multisystematic disease; characterized with recurrent oral and genital ulcers and eye involvement which may involve all systems. The lung may be involved, usually during disease exacerbations, and may lead to fatal pulmonary arterial occlusion. Here; we reported two patients diagnosed as Behçet's disease with intracardiac thrombus and co-existing pulmonary artery aneurysm. 28 and 32 years old two male patients presented with cough, hemoptysis, dyspnea and weakness applied to hospital. In both patients there were pulmonary artery aneurysm and embolism with intracardiac thrombus. The patient who underwent surgery because of intracardiac thrombus, died in the 9th month of immunosuppressive therapy as a result of massive haemoptysis. The other patient completed immunosuppressive therapy, is stil alive and doing well. In spite of improvement with medical therapy in aneurysms in both of these patients, the death of one patient, shows us that mortality is high in vasculitis of Behçet's disease despite therapy.

Anahtar Kelimeler

Behçet's disease, pulmonary artery aneurysm, intracardiac thrombus

Kaynakça

• 1. International study group for Behçet’s disease criteria for diagnosis of Behçet’s disease. Lancet 1990; 335:1078-80.
• 2. O’Duffy JD. Behçet’s Syndrome. New Engl J Med 1990; 322(5): 326-7.
• 3. Raz I, Okan E, Chajek-Shaul T. Pulmonary manifestations in Behçet’s syndrome. Chest 1989; 95(3): 585-9.
• 4. Erkan F, Gül A, Tarak E. Pulmonary manifestations of Behçet’s Disease. Thorax 2001; 56: 572-8
• 5. Uçan ES, K›ter G., Abado¤lu Ö, et al. Thoracic Manifestations of Behçet’s Disease: Reports of the Turkish Authors. Turkish Respiratory Journal 2001; 2(2):39-44
• 6. ‹nanç M. Behçet hastal›¤›nda venlerin tutulumu. Aktüel T›p Dergisi 1997; 2: 117-88.
• 7. Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms in Behçet’s syndrome: A report of 24 cases. Br J Rheumatol 1994; 33: 48-51.
• 8. Gönlügür U, Atalay H., Kaptano¤lu M, et al. Intracardiac thrombus and co-existing pulmonary artery aneurysm in Behçet’s disease: Two case reports. Turkish Respiratory Journal 2003; 4(3): 153-5.
• 9. Mo¤ulkoç N, Burgess MI, Bishop PW. Intracardiac trombus in Behçet’s disease. A systematic review. Chest 2000; 118: 479-87.
• 10. Baykan M, Erdal C, Baykan EC, et al. Behçet’s disease with a large intracardiac thrombus: A case report. Heart 2001; 85: 7.
• 11. Vaya A, Forner MJ, Estelles A, et al. Intracardiac thrombosis in case of Behçet’s disease associated with the prothrombin 202106-A mutation. Haematologica 2000; 85: 425-8.
• 12. Wechsler B, Le Thi Huong D, De Gennes C, et al. Manifestations arterielles de la maladie de Behçet. Douze observations. Rev Med Interne 1989; 10: 303-11.
• 13. Candan I, Erol C, Samel A, Akal›n H. Behçet’s disease: cardiac and pulmonary involvement. Eur Heart J 1986; 7: 999-1002.
• 14. Kalyoncu AF, Ard›ç S, Artvinli M, et al. Pulmonary aspects of Behçet’s Disease clinical studies on thirty cases. J Health Sci 1990; 2: 51-8.
• 15. Ac›can T, Kaya A, Gürkan ÖU, et al. Pulmonary involvement in Behçet’s Disease. Türkiye T›p Dergisi 1998; 5(6): 360-4.
• 16. Tunac› A, Berkmen YM, Gökmen E. Thoracic involvement in Behçet’s Disease: Pathologic, clinical and imaging features. AJR 1995; 164: 51-6.
• 17. Tunac› M, Özkorkmaz B, Tunac› A et al. CT findings of pulmonary artery aneurysms during treatment for Behçet’s Disease. AJR 1999 ; 172: 729-33.
• 18. Augasten A, Apter S, Theodor R. Right ventricular thrombus and pulmonary arteritis in Behçet’s disease. Isr J Med Sci 1987; 23: 900-1.
• 19. Koç Y, Güllü I, Görgün A, et al. Vascular involvement in Behçet’s disease. J Rheumatol 1992; 19: 402-10.
• 20. Huong DL, Dolmazon C, De Zuttere D et al. Complete recovery of right intraventricular thrombus and pulmonary arteritis in Behçet’s disease. Br J Rheumatol 1997; 36: 130-2.
• 21. Böke E, Sar›gül A, U¤urlu B, et al. Concomitant carotid and popliteal artery aneurysms in a patient with Behçet’s syndrome- A case report. Vas Surg 1992; 26: 685-7.
• 22. Mendes LA, Magraw LL, Aldea GS, Davidoff R. Right ventricular thrombus: an unusual manifestation of Behçet’s disease. J Am Soc Echocardiogr 1994; 7: 438-40.