抗 PM-Scl 抗体在系统性硬化症患者中的应用。
Anti-PM-Scl antibody in patients with systemic sclerosis.
机构信息
Drexel University College of Medicine, PA, USA.
出版信息
Clin Exp Rheumatol. 2012 Mar-Apr;30(2 Suppl 71):S12-6. Epub 2012 May 29.
OBJECTIVES
To compare systemic sclerosis (SSc) patients with and without anti-PM-Scl antibody.
METHODS
We reviewed the medical records of 76 anti-PM-Scl antibody positive SSc patients and 2349 anti-PMScl negative SSc patients first evaluated during 1980-2004. Patients were included if they had a clinical diagnosis of SSc either alone or in overlap with another connective tissue disease. Anti-PM-Scl antibody was screened for by indirect immunofluorescence and tested by Ouchterlony double immunodiffusion.
RESULTS
Anti-PM-Scl antibody positive patients had a significantly higher frequency of a positive ANA with nucleolar staining (87% vs. 32%, p<0.0001) and were younger at both symptom onset (p=0.004) and first physician diagnosis of SSc (p<0.001). They were classified more often as having overlap with another connective tissue disease, particularly polymyositis-dermatomyositis, and more frequently had limited cutaneous involvement (72% vs. 52%, p=0.001). Maximal skin thickening was less in anti-PM-Scl antibody patients (mean modified Rodnan total skin score 6.0±6.3 vs. 15.9±14.2, p<0.001). Anti-PM-Scl antibody positive patients less frequently had peripheral vascular (91% vs. 98%, p=0.0002) and gastrointestinal (52% vs. 79%, p=0.0001) disease. Lung involvement overall had a similar distribution between both groups. However, radiographic evidence of pulmonary fibrosis was more frequent in anti-PM-Scl antibody positive patients (50% vs. 37%, p=0.05) and pulmonary arterial hypertension was less often detected (5% vs. 15%, p<0.04). Skeletal muscle involvement (51% vs. 14%, p<0.0001) and subcutaneous calcinosis (p<0.003) were both significantly more often observed in anti-PM-Scl antibody positive patients. Joint, heart, and kidney involvement were similar in both groups. Overall survival was significantly better for anti-PM-Scl antibody positive patients (10 year cumulative survival rate 91% vs. 65%, p=0.0002). After adjustment for age, sex and limited vs. diffuse cutaneous involvement, patients with anti-PM-Scl antibody were significantly less likely to die (HR=0.32, 95% CI, [0.14, 0.72] p=0.006).
CONCLUSIONS
SSc patients with anti-PM-Scl antibody are younger and significantly more often have limited cutaneous involvement, skeletal muscle disease, pulmonary fibrosis and calcinosis compared to anti-PM-Scl antibody negative SSc patients. Ten-year cumulative survival is significantly better in anti-PM-Scl antibody positive SSc patients.
目的
比较抗 PM-Scl 抗体阳性和阴性的系统性硬化症（SSc）患者。
方法
我们回顾了 1980 年至 2004 年间首次评估的 76 例抗 PM-Scl 抗体阳性 SSc 患者和 2349 例抗 PM-Scl 阴性 SSc 患者的病历。如果患者具有 SSc 的临床诊断，无论是单独存在还是与另一种结缔组织疾病重叠，均将其纳入研究。抗 PM-Scl 抗体通过间接免疫荧光法进行筛查，并通过 Ouchterlony 双免疫扩散法进行检测。
结果
抗 PM-Scl 抗体阳性患者的抗核抗体阳性率（核仁染色）显著更高（87% vs. 32%，p<0.0001），且在症状发作（p=0.004）和首次被诊断为 SSc 时（p<0.001）年龄更小。他们更常被归类为患有另一种结缔组织疾病的重叠症，尤其是多发性肌炎/皮肌炎，且更常出现局限性皮肤受累（72% vs. 52%，p=0.001）。抗 PM-Scl 抗体阳性患者的最大皮肤增厚程度较轻（平均改良 Rodnan 总皮肤评分 6.0±6.3 vs. 15.9±14.2，p<0.001）。抗 PM-Scl 抗体阳性患者外周血管（91% vs. 98%，p=0.0002）和胃肠道（52% vs. 79%，p=0.0001）疾病的发生率较低。两组患者的肺部受累总体分布相似。但是，抗 PM-Scl 抗体阳性患者的肺部纤维化影像学证据更常见（50% vs. 37%，p=0.05），且肺动脉高压检出率较低（5% vs. 15%，p<0.04）。抗 PM-Scl 抗体阳性患者的骨骼肌受累（51% vs. 14%，p<0.0001）和皮下钙化（p<0.003）均显著更为常见。关节、心脏和肾脏受累在两组患者中相似。抗 PM-Scl 抗体阳性患者的总体生存率显著更好（10 年累积生存率 91% vs. 65%，p=0.0002）。在校正年龄、性别和局限性与弥漫性皮肤受累后，抗 PM-Scl 抗体阳性患者的死亡风险显著降低（HR=0.32，95%CI，[0.14，0.72]，p=0.006）。
结论
与抗 PM-Scl 抗体阴性的 SSc 患者相比，抗 PM-Scl 抗体阳性的 SSc 患者更年轻，且显著更常出现局限性皮肤受累、骨骼肌疾病、肺纤维化和钙化。抗 PM-Scl 抗体阳性的 SSc 患者的 10 年累积生存率显著更高。
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