Font Josep, Cervera Ricard, Ramos-Casals Manuel, García-Carrasco Mario, Sents Juan, Herrero Carme, del Olmo Jose-Antonio, Darnell Alexandre, Ingelmo Miguel
Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, School of Medicine, University of Barcelona, Spain.
Semin Arthritis Rheum. 2004 Feb;33(4):217-30. doi: 10.1053/s0049-0172(03)00133-1.
To analyze the prevalence and characteristics of the main clinical, hematologic, and immunologic manifestations of systemic lupus erythematosus (SLE) in a cohort of 600 consecutive patients from a single center, and to determine the specific characteristics of organ involvement in a homogeneous SLE population.
Patients were consecutively seen in our department either as inpatients or outpatients between 1980 and 2001. All had documented medical histories and underwent a medical interview as well as a routine general physical examination. Clinical and serologic characteristics of all patients were consecutively collected in a protocol form.
The final cohort (survival cohort) consisted of 533 (89%) women and 67 (11%) men (female to male ratio, 8:1), with an average of 29 new patients per year. Mean age at onset of symptoms attributable to the disease was 31 years (range, 5 to 84 years) and mean age at the time of diagnosis of SLE was 33 years (range, 6 to 85 years). The most frequent SLE involvement was articular involvement, found in 498 patients (83%), followed by hematologic involvement in 451 patients (75%), specific SLE cutaneous involvement in 354 patients (59%), constitutional features in 252 patients (42%), and nephropathy in 203 patients (34%). Patients enrolled in the protocol before 1991 had a higher frequency of central nervous system (CNS) involvement (27% vs 10%, P <.001), thrombotic events (17% vs 9%, P =.003), and abnormal hematologic parameters (85% vs 66%, P <.01), but a lower frequency of articular involvement (79% vs 86%, P =.038) than those enrolled after 1991. The following were observed associations: specific SLE cutaneous involvement was associated with anti-Sm antibodies; renal involvement with hemolytic anemia and anti-double-stranded DNA antibodies; CNS involvement with thrombocytopenia and immunoglobulin G-anticardiolipin; thrombotic events with low total hemolytic complement, immunoglobulin G-anticardiolipin, and lupus anticoagulant; and myositis with anemia and anti-ribonucleoprotein antibodies.
This large study, performed in a single center, has shown cluster associations between certain clinical, hematologic, and immunologic features of SLE, reflecting specific patterns of disease expression. The accurate evaluation of clinical features and laboratory markers at disease diagnosis and during evolution may improve the clinical treatment of SLE patients.
分析来自单一中心的600例连续系统性红斑狼疮（SLE）患者队列中主要临床、血液学和免疫学表现的患病率及特征，并确定同质SLE人群中器官受累的具体特征。
1980年至2001年期间，患者作为住院患者或门诊患者在我们科室连续就诊。所有患者均有病历记录，并接受了医学访谈以及常规全身检查。所有患者的临床和血清学特征均以方案形式连续收集。
最终队列（生存队列）包括533名（89%）女性和67名（11%）男性（女性与男性比例为8:1），平均每年有29名新患者。疾病相关症状出现时的平均年龄为31岁（范围为5至84岁），SLE诊断时的平均年龄为33岁（范围为6至85岁）。最常见的SLE受累是关节受累，见于498例患者（83%），其次是血液学受累451例患者（75%）、特异性SLE皮肤受累354例患者（59%）、全身症状252例患者（42%）和肾病203例患者（34%）。1991年前纳入方案的患者中枢神经系统（CNS）受累频率较高（27%对10%，P<.001）、血栓形成事件发生率较高（17%对9%，P=.003）以及血液学参数异常发生率较高（85%对66%，P<.01），但关节受累频率低于1991年后纳入的患者（79%对86%，P=.038）。观察到以下关联：特异性SLE皮肤受累与抗Sm抗体相关；肾脏受累与溶血性贫血和抗双链DNA抗体相关；CNS受累与血小板减少和免疫球蛋白G-抗心磷脂相关；血栓形成事件与总溶血补体降低、免疫球蛋白G-抗心磷脂和狼疮抗凝物相关；以及肌炎与贫血和抗核糖核蛋白抗体相关。
这项在单一中心进行的大型研究显示了SLE某些临床、血液学和免疫学特征之间的聚类关联，反映了疾病表达的特定模式。在疾病诊断和病程中对临床特征和实验室指标进行准确评估可能会改善SLE患者的临床治疗。
