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Objectives Behçet's disease (BD) is a unique systemic vasculitis mainly involving veins, in contrast to other vasculitides. Prior studies have shown that pulmonary arteries (PAs) have a similar structure to systemic veins. In this study we aimed to assess PA wall thickness by transthoracic echocardiography (TTE) in BD patients compared with healthy controls (HCs) and patients with non-inflammatory pulmonary embolism (NIPE). Methods Patients with BD (n  = 77) and NIPE (n  = 33) and HCs (n  = 57) were studied. PA wall thickness was measured from the mid-portion of the main PA with TTE by two cardiologists blinded to cases. Results PA wall thickness was significantly lower in HCs [3.6 mm (s. d. 0.3)] compared with NIPE [4.4 mm (s. d. 0.5)] and BD [4.4 mm (s. d. 0.6)] (P  < 0.001 for both). PA wall thickness was similar between BD and NIPE (P  = 0.6). Among patients with BD, PA wall thickness was significantly higher in patients with major organ involvement compared with mucocutaneous limited disease [4.7 mm (s. d. 0.4) vs 3.7 (0.4), P  < 0.001], HCs and NIPE (P  < 0.001 and P  = 0.006, respectively). PA wall thickness was comparable between patients with vascular and non-vascular major organ involvement [4.6 mm (s. d. 0.5) vs 4.7 (0.3), P  = 0.3]. Conclusion We observed that PA wall thickness was significantly higher in BD with major organ involvement compared with patients with only mucocutaneous limited disease, HCs and NIPE. These results suggest that increased PA wall thickness may be a sign of severe disease with major organ involvement in BD. [ABSTRACT FROM AUTHOR]

Amaç: Behçet hastalığı (BH), diğer vaskülitlerin aksine, temelde venöz yapıların tutulumuyla giden bir sistemik vaskülittir. Önceki çalışmalar pulmoner arterlerin (PA) ince duvarlı, düşük dirençli ve artmış kompliansa sahip yapılar oluşu sebebiyle, sistemik venlere benzer özellikte olduğunu göstermiştir. Yakın zamanda grubumuz tarafından, Doppler ultrasonografi ile Behçet hastalarının alt ekstremite venlerinde artmış ven duvar kalınlığı gösterilmişti. Bu çalışmada, Behçet hastalarında transtorasik ekokardiyografi (TTE) ile ölçülen PA duvar kalınlığının, sağlıklı kontroller (SK) ve nonenflamatuvar pulmoner embolili (NIPE) hastalarla karşılaştırılması amaçlanmıştır. Yöntem: Bu çalışmaya, 77 Behçet, 33 NIPE hastası ve 57 SK dahil edildi. PA duvar kalınlığı deneyimli bir kardiyolog tarafından TTE ile, ana PA'nın orta kısmından (pulmoner kapağın yaklaşık 1-2 cm distalinden) ölçüldü (Şekil 1). Bulgular: PA duvar kalınlığı SK'de, NIPE ve Behçet hastalarına kıyasla anlamlı olarak daha düşüktü (her ikisi analiz için p<0,001). PA duvar kalınlığı, majör organ tutulumu olan Behçet hastalarında, SK ve NIPE'ye göre anlamlı olarak daha yüksek bulundu (sırasıyla p<0,001 ve p=0,027). PA duvar kalınlığı, vasküler, oküler ve nörolojik tutulumu olan hastalarda, sadece mukokutanöz tutulumu olan Behçet hastalarına göre daha yüksekti (tüm analizler için p<001) (Tablo 1). Tartışma: Bu çalışmada, majör organ tutulumu olan Behçet hastalarında PA duvar kalınlığının majör organ tutulumu tipinden bağımsız olarak, sadece mukokutanöz tutulumu olan Behçet hastalarına göre anlamlı olarak daha yüksek olduğu bulunmuştur. BH'de PA tutulumu, hastalığın seyrinde, yaygın vasküler enflamasyonun venöz damarlarla sınırlı olmadığını düşündürmektedir. Sadece majör organ tutulumu olan hastalarda saptanan artmış PA duvar kalınlığı, daha ciddi bir hastalığın belirtisi ve hastalık takibi sırasında majör organ tutulumunun habercisi olabilir. [ABSTRACT FROM AUTHOR]

Objectives: Behçet's disease (BD) is a unique systemic vasculitis mainly involving veins, in contrast to other vasculitides. Prior studies have shown that pulmonary arteries (PAs) have a similar structure to systemic veins. In this study we aimed to assess PA wall thickness by transthoracic echocardiography (TTE) in BD patients compared with healthy controls (HCs) and patients with non-inflammatory pulmonary embolism (NIPE)., Methods: Patients with BD (n = 77) and NIPE (n = 33) and HCs (n = 57) were studied. PA wall thickness was measured from the mid-portion of the main PA with TTE by two cardiologists blinded to cases., Results: PA wall thickness was significantly lower in HCs [3.6 mm (s.d. 0.3)] compared with NIPE [4.4 mm (s.d. 0.5)] and BD [4.4 mm (s.d. 0.6)] (P < 0.001 for both). PA wall thickness was similar between BD and NIPE (P = 0.6). Among patients with BD, PA wall thickness was significantly higher in patients with major organ involvement compared with mucocutaneous limited disease [4.7 mm (s.d. 0.4) vs 3.7 (0.4), P < 0.001], HCs and NIPE (P < 0.001 and P = 0.006, respectively). PA wall thickness was comparable between patients with vascular and non-vascular major organ involvement [4.6 mm (s.d. 0.5) vs 4.7 (0.3), P = 0.3]., Conclusion: We observed that PA wall thickness was significantly higher in BD with major organ involvement compared with patients with only mucocutaneous limited disease, HCs and NIPE. These results suggest that increased PA wall thickness may be a sign of severe disease with major organ involvement in BD., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)