Development of de novo major involvement during follow-up in Behçet’s syndrome
- 206 Downloads
The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet’s syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52 % of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al. (Rheum Dis Clin North Am 39(2):245–61, 2013) the incidence of de novo major involvement during the follow-up and Hatemi et al. (Clin Exp Rheumatol 32(4 Suppl 84):S112–22, 2014) the use of immunosuppressive drugs during the follow-up. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular, vascular or CNS involvement after a latency period from the diagnosis of at least 3 years. Among 62 patients characterized by a mild onset of disease, we observed that after at least 3 years from the diagnosis, 21 BS patients (34 %) still developed serious morbidities. Specifically, three patients developed ocular involvement, nine patients developed neurological involvement and nine patients presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that patients who developed de novo major involvement were more frequently males and younger; furthermore, 95 % of these patients were characterized by a young onset of disease (p < 0.001). Being free of major organ complication in the first years of BS is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of BS suggests that a tight control is strongly recommended during the course of the disease.
KeywordsBehçet’s syndrome Major organ involvement Prognosis
Conflict of interest
The authors declare they have no conflicts of interest.
- 3.Koné-Paut I, Darce-Bello M, Shahram F, Gattorno M, Cimaz R, Ozen S, Cantarini L, Tugal-Tutktun I, Assaad-Khalil S, Hofer M, Kuemmerle-Deschner J, Benamour S, Al Mayouf S, Pajot C, Anton J, Faye A, Bono W, Nielsen S, Letierce A, Tran TA, PED-BD International Expert Committee (2011) Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet’s disease: an international cohort study of 110 patients. One-year follow-up data. Rheumatology (Oxford) 50:184–188. doi: 10.1093/rheumatology/keq324 CrossRefGoogle Scholar
- 7.Cantarini L, Vitale A, Lucherini OM, De Clemente C, Caso F, Costa L, Emmi G, Silvestri E, Magnotti F, Maggio MC, Prinzi E, Lopalco G, Frediani B, Cimaz R, Galeazzi M, Rigante D (2014) The labyrinth of autoinflammatory disorders: a snapshot on the activity of a third-level center in Italy. Clin RheumatolGoogle Scholar
- 9.Cantarini L, Vitale A, Scalini P, Dinarello CA, Rigante D, Franceschini R, Simonini G, Borsari G, Caso F, Lucherini OM, Frediani B, Bertoldi I, Punzi L, Galeazzi M, Cimaz R (2013) Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. doi: 10.1007/s10067-013-2443-8
- 11.International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–80Google Scholar
- 16.Hamuryudan V, Hatemi G, Tascilar K, Sut N, Ozyazgan Y, Seyahi E, Mat C, Yurdakul S, Yazici H (2010) Prognosis of Behcet’s syndrome among men with mucocutaneous involvement at disease onset: long-term outcome of patients enrolled in a controlled trial. Rheumatology (Oxford) 4(1):173–177CrossRefGoogle Scholar
- 21.Caso F, Costa L, Rigante D, Lucherini OM, Caso P, Bascherini V, Frediani B, Cimaz R, Marrani E, Nieves-Martín L, Atteno M, Raffaele CG, Tarantino G, Galeazzi M, Punzi L, Cantarini L (2014) Biological treatments in Behçet’s disease: beyond anti-TNF therapy. Mediat Inflamm. doi: 10.1155/2014/107421