Royal Free and University College Medical School, Centre for Rheumatology, London NW3 2PF, UK.
J Rheumatol. 2011 Nov;38(11):2406-9. doi: 10.3899/jrheum.101248. Epub 2011 Aug 15.
To determine the prevalence of systemic sclerosis (SSc) overlap syndrome and autoantibody profile in a large single-center cohort.
SSc diagnoses, subsets, and autoantibody profiles were obtained from clinical records of patients attending the Centre for Rheumatology, Royal Free Hospital, between September 1999 and February 2007.
In total, 332 (20%) of 1700 patients with SSc had overlap syndrome. This comprised myositis (42.8%), rheumatoid arthritis (RA; 32%), Sjögren's syndrome (SS; 16.8%), and systemic lupus erythematosus (SLE; 8.4%). Antinuclear antibody was positive in 96.6% of patients. Anticentromere antibody (ACA) was exclusively present in limited cutaneous SSc (lcSSc) overlap cases (22%), and more common in SSc/SS overlap (44.7%), whereas no difference was found in the prevalence of Scl-70 autoantibody between lcSSc and diffuse cutaneous SSc overlap groups. U1RNP was more frequent in SSc/SLE (44%), while Ro antibody was more likely to be found in SSc/SS (29.8%). ACA was absent and anti-Scl-70 was infrequent in SSc/myositis; polymyositis-scleroderma antibody was more frequent in this group (33.1%). About 50% of patients had raised rheumatoid factor (RF), with no difference between overlap groups irrespective of RF titer. In contrast, anticyclic citrullinated peptide antibody was more frequent in patients with RA features.
About one-fifth of SSc cases had overlap features. There were distinct serological features that may predict specific clinical presentation and disease course.
在一个大型单中心队列中确定系统性硬化症（SSc）重叠综合征的患病率和自身抗体谱。
从 1999 年 9 月至 2007 年 2 月在皇家自由医院风湿病中心就诊的患者的临床记录中获得 SSc 诊断、亚型和自身抗体谱。
在总共 1700 例 SSc 患者中，有 332 例（20%）存在重叠综合征。这包括肌炎（42.8%）、类风湿关节炎（RA；32%）、干燥综合征（SS；16.8%）和系统性红斑狼疮（SLE；8.4%）。抗核抗体在 96.6%的患者中呈阳性。抗着丝点抗体（ACA）仅存在于局限性皮肤 SSc（lcSSc）重叠病例（22%）中，在 SSc/SS 重叠中更为常见（44.7%），而在 lcSSc 和弥漫性皮肤 SSc 重叠组之间，Scl-70 自身抗体的患病率没有差异。U1RNP 在 SSc/SLE 中更为常见（44%），而 Ro 抗体更可能在 SSc/SS 中发现（29.8%）。ACA 缺失，抗 Scl-70 罕见于 SSc/肌炎；肌炎-硬皮病抗体在该组中更为常见（33.1%）。约 50%的患者存在类风湿因子（RF）升高，重叠组之间无论 RF 滴度如何均无差异。相比之下，RA 特征患者的抗环瓜氨酸肽抗体更为常见。
约五分之一的 SSc 病例存在重叠特征。存在独特的血清学特征，这些特征可能预测特定的临床表现和疾病过程。
