Aktuelle Rheumatologie 2021; 46(05): 485-488
DOI: 10.1055/a-1400-2586
Case Report

Behçet’s Syndrome Complicated with Pulmonary Artery Thrombosis: Response to Tocilizumab Treatment

Morbus Behçet kompliziert durch eine Pulmonalarterienthrombose: Ansprechen auf die Behandlung Tocilizumab
Didem Sahin Eroglu
1   Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey
,
Anil Colaklar
2   Department of Radiology, Ankara University Faculty of Medicine, Ankara, Turkey
,
Alparslan Ceran
3   Department of Internal Medicine, Ankara University Faculty of Medicine, Ankara, Turkey
,
Serdar Sezer
1   Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey
,
1   Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey
,
Gulay Kinikli
1   Department of Internal Medicine, Division of Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey
› Author Affiliations

Zusammenfassung

Die Beteiligung der Lungenarterie ist eine seltene, aber tödliche Komplikation des Morbus Behçets (MB). Hier berichten wir über einen männlichen MB-Patienten mit zwei vaskulären Komplikationen, nämlich dem Budd-Chiari-Syndrom und der Pulmonalarterienthrombose. Der Patient litt unter einer rezidivierenden Lungenarterienthrombose, die gegen mehrere immunsuppressive Medikamente, einschließlich hochdosierter Glukokortikoide und dem Anti-TNF-Mittel Infliximab, refraktär war. Infolge mehrerer Rezidive wurde er effektiv mit Tocilizumab behandelt, wodurch auch eine vollständige Rekanalisation der thrombosierten Arterien erreicht wurde.

Abstract

Pulmonary artery involvement is a rare but deadly complication of Behçet’s syndrome (BS). This article presents a male patient with BS complicated with Budd-Chiari syndrome and pulmonary artery thrombosis. The patient had recurrent pulmonary artery thrombosis resistant to several immunosuppressive drugs including high-dose glucocorticoids and the anti-TNF agent infliximab. Following multiple relapses, he was effectively treated with tocilizumab, which also achieved complete recanalization of the thrombosed arteries.



Publication History

Article published online:
27 April 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Yazici H, Seyahi E, Hatemi G. et al. Behçet syndrome: a contemporary view. Nat Rev Rheumatol 2018; 14: 119
  • 2 Tascilar K, Melikoglu M, Ugurlu S. et al. Vascular involvement in Behçet's syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford) 2014; 53: 2018-2022
  • 3 Seyahi E, Yazici H. Behçet’s syndrome: pulmonary vascular disease. Curr Opin Rheumatol 2015; 27: 18-23
  • 4 Seyahi E, Melikoglu M, Akman C. et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore) 2012; 91: 35-48
  • 5 Hatemi G, Christensen R, Bang D. et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis 2018; 77: 808-818
  • 6 The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 2014; 28: 338-347
  • 7 Düzgün N, Ateş A, Aydintuğ OT. et al. Characteristics of vascular involvement in Behçet’s disease. Scand J Rheumatol 2006; 35: 65-68
  • 8 Zhang X, Dai H, Ma Z. et al. Pulmonary involvement in patients with Behçet’s disease: report of 15 cases. Clin Respir J 2015; 9: 414-422
  • 9 Ozguler Y, Dincses E, Bakan S. et al. SAT0525 An update on pulmonary artery involvement in behcet’s syndrome: more pulmonary artery thrombotic disease and a better outcome. Annals of the Rheumatic Diseases 2018; 77: 1118
  • 10 Saadoun D, Wechsler B, Desseaux K. et al. Mortality in Behçet’s disease. Arthritis Rheum 2010; 62: 2806-2812
  • 11 Hamuryudan V, Seyahi E, Ugurlu S. et al. Pulmonary artery involvement in Behçet’s syndrome: Effects of anti-Tnf treatment. Semin Arthritis Rheum 2015; 45: 369-373
  • 12 Gholijani N, Ataollahi MR, Samiei A. et al. An elevated pro-inflammatory cytokines profile in Behcet’s disease: A multiplex analysis. Immunol Lett 2017; 186: 46-51
  • 13 Akman-Demir G, Tüzün E, Içöz S. et al. Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine 2008; 44: 373-376
  • 14 Bonacini M, Soriano A, Cimino L. et al. Cytokine Profiling in Aqueous Humor Samples From Patients With Non-Infectious Uveitis Associated With Systemic Inflammatory Diseases. Front Immunol 2020; 11: 358
  • 15 Ding Y, Li C, Liu J. et al. Tocilizumab in the treatment of severe and/or refractory vasculo-Behçet’s disease: a single-centre experience in China. Rheumatology (Oxford) 2018; 57: 2057-2059
  • 16 Akiyama M, Kaneko Y, Takeuchi T. Effectiveness of tocilizumab in Behcet’s disease: A systematic literature review. Semin Arthritis Rheum 2020; 50: 797-804
  • 17 Ozguler Y, Hatemi G, Cetinkaya F. et al. Clinical course of acute deep vein thrombosis of the legs in Behçet’s syndrome. Rheumatology (Oxford) 2020; 59: 799-806