Ramos-Casals Manuel, Anaya Juan-Manuel, García-Carrasco Mario, Rosas José, Bové Albert, Claver Gisela, Diaz Luis-Aurelio, Herrero Carmen, Font Josep
From the Departments of Autoimmune Diseases (MR-C, MG-C, AB, GC, JF), Clinical Institute of Infections and Immunology, and Dermatology (CH), Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), School of Medicine, University of Barcelona, Barcelona, Spain; Rheumatology Unit (JR), Hospital de la Vila-Joiosa, Vila-Joiosa, Alacant, Spain; and Department of Rheumatology (J-MA, L-AD), Unidad de Biología Celular e Inmunogenética, Corporación para Investigaciones Biomédicas, and Clínica Universitaria Bolivariana, Medellín, Colombia.
Medicine (Baltimore). 2004 Mar;83(2):96-106. doi: 10.1097/01.md.0000119465.24818.98.
To analyze the different clinical and histologic types of cutaneous vasculitis in patients with primary Sjögren syndrome (SS), we investigated the clinical and immunologic characteristics of 558 consecutive patients with primary SS from our units and selected those with clinical evidence of cutaneous lesions, excluding drug reactions and xeroderma. All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. A total of 89 (16%) patients presented with cutaneous involvement (88 female patients and 1 male; mean age, 51.8 yr). The main cutaneous involvement was cutaneous vasculitis, present in 52 (58%) patients. There were 51 (98%) female patients and 1 (2%) male, with a mean age at diagnosis of cutaneous vasculitis of 51 years (range, 20-80 yr). Fourteen presented with cryoglobulinemic vasculitis, 11 with urticarial vasculitis, and the remaining 26, with cutaneous purpura not associated with cryoglobulins. A skin biopsy specimen was obtained in 38 patients (73%). Involvement of small-sized vessels was observed in 36 (95%) patients (leukocytoclastic vasculitis), while the remaining 2 (5%) presented with medium-sized vessel vasculitis (necrotizing vasculitis). Patients with cutaneous vasculitis had a higher prevalence of articular involvement (50% vs 29%, p = 0.044), peripheral neuropathy (31% vs 4%, p < 0.001), Raynaud phenomenon (40% vs 15%, p = 0.008), renal involvement (10% vs 0%, p = 0.028), antinuclear antibodies (88% vs 60%, p = 0.002), rheumatoid factor (78% vs 48%, p = 0.004), anti-Ro/SS-A antibodies (70% vs 43%, p = 0.011), and hospitalization (25% vs 4%, p = 0.005) compared with SS patients without vasculitis. Six (12%) patients died, all of whom had multisystemic cryoglobulinemia.In conclusion, cutaneous involvement was detected in 16% of patients with primary SS, with cutaneous vasculitis being the most frequent process. The main characteristics of SS-associated cutaneous vasculitis were the overwhelming predominance of small versus medium vessel vasculitis and leukocytoclastic versus mononuclear vasculitis, with a higher prevalence of extraglandular and immunologic SS features. Small vessel vasculitis manifested as palpable purpura, urticarial lesions, or erythematosus maculopapules, with systemic involvement in 44% of patients in association with cryoglobulins in 30%. Life-threatening vasculitis was closely related to cryoglobulinemia.
为分析原发性干燥综合征（SS）患者皮肤血管炎的不同临床和组织学类型，我们调查了来自我们科室的558例连续性原发性SS患者的临床和免疫学特征，并选择有皮肤病变临床证据的患者，排除药物反应和干皮病。所有患者均符合欧洲共同体研究组1993年提出的SS诊断标准中的4项或更多项。共有89例（16%）患者出现皮肤受累（88例女性患者和1例男性；平均年龄51.8岁）。主要的皮肤受累表现为皮肤血管炎，52例（58%）患者出现该症状。其中有51例（98%）女性患者和1例（2%）男性，皮肤血管炎诊断时的平均年龄为51岁（范围20 - 80岁）。14例表现为冷球蛋白血症性血管炎，11例为荨麻疹性血管炎，其余26例为不伴有冷球蛋白的皮肤紫癜。38例患者（73%）进行了皮肤活检。36例（95%）患者观察到小血管受累（白细胞破碎性血管炎），其余2例（5%）表现为中血管血管炎（坏死性血管炎）。与无血管炎的SS患者相比，皮肤血管炎患者关节受累（50%对29%，p = 0.044）、周围神经病变（31%对4%，p < 0.001）、雷诺现象（40%对15%，p = 0.008）、肾脏受累（10%对0%，p = 0.028）、抗核抗体（88%对60%，p = 0.002）、类风湿因子（78%对48%，p = 0.004）、抗Ro/SS - A抗体（70%对43%，p = 0.011）及住院率（25%对4%，p = 0.005）的患病率更高。6例（12%）患者死亡，所有这些患者均有多系统冷球蛋白血症。
总之，16%的原发性SS患者出现皮肤受累，其中皮肤血管炎是最常见的情况。与SS相关的皮肤血管炎的主要特征是小血管炎与中血管炎、白细胞破碎性血管炎与单核细胞性血管炎的压倒性优势，以及腺体外和免疫学SS特征的更高患病率。小血管炎表现为可触及的紫癜、荨麻疹样损害或红斑性斑丘疹，44%的患者有全身受累，30%的患者与冷球蛋白有关。危及生命的血管炎与冷球蛋白血症密切相关。
