Talarico Rosaria, Cantarini Luca, d'Ascanio Anna, Figus Michele, Favati Benedetta, Baldini Chiara, Tani Chiara, Neri R, Bombardieri Stefano, Mosca Marta
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Policlinico "Le Scotte", University of Siena, Siena, Italy.
Clin Rheumatol. 2016 Jan;35(1):247-50. doi: 10.1007/s10067-015-2906-1. Epub 2015 Mar 8.
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.
该研究的主要目的是评估白塞病（BS）患者队列随访期间新发主要受累情况的发生率；次要目的是分析出现新发主要受累情况的患者的流行病学特征和长期预后。在我们的120例BS患者队列中，我们评估了疾病早期无主要器官受累的所有受试者；具体而言，在疾病发作时，队列中的52%表现为常见的黏膜皮肤受累。主要结局指标如下：哈泰米等人（《北美风湿病学临床》39(2):245 - 61, 2013）随访期间新发主要受累情况的发生率以及哈泰米等人（《临床与实验风湿病学》32(4增刊84):S112 - 22, 2014）随访期间免疫抑制药物的使用情况。我们将随访期间新发主要受累情况定义为自诊断起至少3年潜伏期后出现严重眼部、血管或中枢神经系统受累。在62例疾病起病较轻的患者中，我们观察到自诊断起至少3年后，21例BS患者（34%）仍出现了严重病变。具体而言，3例患者出现眼部受累，9例患者出现神经受累，9例患者出现血管受累。比较两组的主要流行病学和临床发现，我们观察到出现新发主要受累情况的患者男性更常见且更年轻；此外，这些患者中有95%的特征是疾病起病年轻（p < 0.001）。在BS发病的最初几年无主要器官并发症不一定是预后良好的标志。总体而言，BS病程中出现新发主要受累情况表明在疾病过程中强烈建议进行严格控制。
