Case Report: Which Vasculitis Is It?

Conclusion

Behçet’s disease is a heterogeneous vasculitis with a highly variable clinical presentation. Involvement of the large vessels is relatively rare, but may be the presenting symptom, at times preceding the onset of classic symptoms by years. In these instances, such as our own, diagnosis may be further delayed by treatment for other causes of large vessel vasculitis. Thus, it is important to consider Behçet’s disease in patients with a large-vessel vasculitis who develop new and unusual symptoms that are unexplained by their current diagnosis. 

Mary Buckley, MD, is a fellow in pediatric rheumatology at Duke University, Durham, N.C.

Jeffrey Dvergsten, MD, is an assistant professor of pediatrics, Division of Pediatric Rheumatology, Department of Pediatrics, Duke University, Durham, N.C.

References

1. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol. 2013 Feb;9(2):79–89.
2. Zouboulis CC, Keitel W. A historical review of early descriptions of Adamantiades-Behçet’s disease. J Invest Dermatol. 2002 Jul;119(1):201–205.
3. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med. 1999 Oct 21;341(17):1284–1291.
4. Melikoglu M, Kural-Seyahi E, Tascilar K, Yazici H. The unique features of vasculitis in Behçet’s syndrome. Clin Rev Allergy Immunol. 2008 Oct;35(1–2):40–46.
5. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet’s disease: An update. Curr Opin Rheumatol. 2011 Jan;23(1):24–31.
6. Fei Y, Li X, Lin S, et al. Major vascular involvement in Behçet’s disease: A retrospective study of 796 patients. Clin Rheumatol. 2013 Jun;32(6):845–852.
7. Sarica-Kucukoglu R, Akdag-Kose A, Kayabal IM, et al. Vascular involvement in Behçet’s disease: A retrospective analysis of 2319 cases. Int J Dermatol. 2006 Aug;45(8):919–921.
8. Gürler A, Boyvat A, Türsen U. Clinical manifestations of Behçet’s disease: An analysis of 2147 patients. Yonsei Med J. 1997 Dec;38(6):423–427.
9. Düzgün N, Ateş A, Aydintug OT, et al. Characteristics of vascular involvement in Behçet’s disease. Scand J Rheumatol. 2006 Jan–Feb;35(1):65–68.
10. Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: A 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003 Jan;82(1):60–76.
11. Ozguler Y, Hatemi G. Management of Behçet’s syndrome. Curr Opin Rheumatol. 2016 Jan;28(1):45–50.
12. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008 Dec;67(12):1656–1662.
13. Merashli M, Eid RE, Uthman I. A review of current management of vasculo-Behcet’s. Curr Opin Rheumatol. 2018 Jan;30(1):50–56.
14. Liu Q, Ye W, Liu C, et al. Outcomes of vascular intervention and use of perioperative medications for nonpulmonary aneurysms in Behçet disease. Surgery. 2016 May;159(5):1422–1429.
15. Hatemi G, Seyahi E, Fresko I, et al. One year in review 2019: Behçet’s syndrome. Clin Exp Rheumatol. 2019 Nov–Dec;37 Suppl 121(6):3–17.
16. Desbois AC, Biard L, Addimanda O, et al. Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behçet’s disease: A multicenter observational study of 18 patients. Clin Immunol. 2018 Dec;197:54–59.
17. Emmi G, Vitale A, Silvestri E, et al. Adalimumab-based treatment versus disease-modifying antirheumatic drugs for venous thrombosis in Behçet’s syndrome: A retrospective study of seventy patients with vascular involvement. Arthritis Rheumatol. 2018 Sep;70(9):1500–1507.
18. 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 Nov 1;57(11):2057–2059.