Serpa Pinto Luísa, Xavier Pires Sara, Silva Berta, Farinha Fátima, Vasconcelos Carlos, Araújo Correia João
Internal Medicine Department, Centro Hospitalar e Universitário do Porto (CHUP), Porto, Portugal.
Internal Medicine Department, Centro Hospitalar e Universitário do Porto (CHUP), Porto, Portugal.
Reumatol Clin (Engl Ed). 2022 Aug-Sep;18(7):410-415. doi: 10.1016/j.reumae.2021.03.011.
Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described.
To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort.
A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points.
We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p<0.001), specifically erythema nodosum (OR=8.381, p<0.001), and pseudofolliculitis (OR=2.910, p<0.001). In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR=1.961, p=0.047), erythema nodosum (Adjusted OR=8.561, p<0.001) and pseudofolliculitis (Adjusted OR=2.372, p=0.007).
Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician.
白塞病（BD）是一种病因不明的系统性血管炎。该疾病的范围从皮肤黏膜表现到其他具有相关发病率的器官疾病。疾病严重程度与男性、发病年龄较早以及结节性红斑的存在之间的关联已被描述。
评估单中心队列中与严重疾病表现相关的临床因素。
一项纵向、前瞻性、单中心队列研究，对1981年至2020年间在专门门诊就诊的患者进行随访。严重白塞病定义为克劳斯总临床严重程度评分>4分。
我们纳入了243例患者，其中31%为男性，平均随访时间为14.6年。关于器官表现，所有患者均有黏膜表现（N = 243，100%），133例（55%）有皮肤表现，104例（43%）有关节表现，71例（29%）有眼部表现，48例（20%）有血管表现，47例（19%）有神经表现，22例（9%）有胃肠道表现，1例（0.4%）有心脏受累。156例（64%）患者被分类为患有严重白塞病。严重白塞病在男性中更常见（OR = 2.004，p = 0.024），随年龄增加（每年OR = 1.021，p = 0.037），在有皮肤表现时（OR = 4.711，p < 0.001），特别是结节性红斑（OR = 8.381，p < 0.001）和假性毛囊炎（OR = 2.910，p < 0.001）。在多变量模型中，与严重白塞病独立相关的变量是男性（调整后OR = 1.961，p = 0.047）、结节性红斑（调整后OR = 8.561，p < 0.001）和假性毛囊炎（调整后OR = 2.372，p = 0.007）。
男性、结节性红斑和假性毛囊炎与严重白塞病形式独立相关，因此应作为临床医生的警示信号。
