Abstract：
OBJECTIVE: To clarify the impact of anti-U1RNP antibodies on the clinical features and prognosis of patients with SSc.
METHODS: We conducted a monocentric case-control, retrospective, longitudinal study. For each patient with SSc and anti-U1RNP antibodies (SSc-RNP+), one patient with mixed connective tissue disease (MCTD) and 2 SSc patients without anti-U1RNP antibodies (SSc-RNP-) were matched for age, sex, and date of inclusion.
RESULTS: Sixty-four SSc-RNP+ patients were compared to 128 SSc-RNP- and 64 MCTD patients. Compared to SSc-RNP-, SSc-RNP+ patients were more often of Afro-Caribbean origin (31.3% vs. 11%, p < 0.01), and more often had an overlap syndrome than SSc-RNP- patients (53.1 % vs. 22.7%, p < 0.0001), overlapping with Sjögren\'s syndrome (n = 23, 35.9%) and/or systemic lupus erythematosus (n = 19, 29.7%). SSc-RNP+ patients were distinctly different from MCTD patients but less often had joint involvement (p < 0.01). SSc-RNP+ patients more frequently developed interstitial lung disease (ILD) (73.4% vs. 55.5% vs. 31.3%, p < 0.05), pulmonary fibrosis (PF) (60.9% vs. 37.5% vs. 10.9%, p < 0.0001), SSc associated myopathy (29.7% vs. 6.3% vs. 7.8%, p < 0.0001), and kidney involvement (10.9% vs. 2.3% vs. 1.6%, p < 0.05). Over a 200-month follow-up period, SSc-RNP+ patients had worse overall survival (p < 0.05), worse survival without PF occurrence (p < 0.01), ILD or PF progression (p < 0.01 and p < 0.0001).
CONCLUSIONS: In SSc patients, anti-U1RNP antibodies are associated with a higher incidence of overlap syndrome, a distinct clinical phenotype, and poorer survival compared to SSc-RNP- and MCTD patients. Our study suggests that SSc-RNP+ patients should be separated from MCTD patients and may constitute an enriched population for progressive lung disease.
METHODS: We conducted a monocentric case-control, retrospective, longitudinal study. For each patient with SSc and anti-U1RNP antibodies (SSc-RNP+), one patient with mixed connective tissue disease (MCTD) and 2 SSc patients without anti-U1RNP antibodies (SSc-RNP-) were matched for age, sex, and date of inclusion.
RESULTS: Sixty-four SSc-RNP+ patients were compared to 128 SSc-RNP- and 64 MCTD patients. Compared to SSc-RNP-, SSc-RNP+ patients were more often of Afro-Caribbean origin (31.3% vs. 11%, p < 0.01), and more often had an overlap syndrome than SSc-RNP- patients (53.1 % vs. 22.7%, p < 0.0001), overlapping with Sjögren\'s syndrome (n = 23, 35.9%) and/or systemic lupus erythematosus (n = 19, 29.7%). SSc-RNP+ patients were distinctly different from MCTD patients but less often had joint involvement (p < 0.01). SSc-RNP+ patients more frequently developed interstitial lung disease (ILD) (73.4% vs. 55.5% vs. 31.3%, p < 0.05), pulmonary fibrosis (PF) (60.9% vs. 37.5% vs. 10.9%, p < 0.0001), SSc associated myopathy (29.7% vs. 6.3% vs. 7.8%, p < 0.0001), and kidney involvement (10.9% vs. 2.3% vs. 1.6%, p < 0.05). Over a 200-month follow-up period, SSc-RNP+ patients had worse overall survival (p < 0.05), worse survival without PF occurrence (p < 0.01), ILD or PF progression (p < 0.01 and p < 0.0001).
CONCLUSIONS: In SSc patients, anti-U1RNP antibodies are associated with a higher incidence of overlap syndrome, a distinct clinical phenotype, and poorer survival compared to SSc-RNP- and MCTD patients. Our study suggests that SSc-RNP+ patients should be separated from MCTD patients and may constitute an enriched population for progressive lung disease.
摘要：
目的：阐明抗U1RNP抗体对SSc患者临床特征及预后的影响。
方法：我们进行了单中心病例对照，回顾性,纵向研究。对于每位具有SSc和抗U1RNP抗体（SSc-RNP）的患者，1例混合性结缔组织病（MCTD）患者和2例无抗U1RNP抗体（SSc-RNP-）的SSc患者的年龄相匹配，性别,和列入日期。
结果：将64例SSc-RNP+患者与128例SSc-RNP-和64例MCTD患者进行了比较。与SSc-RNP-相比，SSc-RNP+患者更常见于非洲裔加勒比裔(31.3%vs.11%,p<0.01),与SSc-RNP-患者相比，重叠综合征更常见(53.1%vs.22.7%,p<0.0001),与干燥综合征（n=23，35.9%）和/或系统性红斑狼疮（n=19，29.7%）重叠。SSc-RNP+患者与MCTD患者明显不同，但关节受累较少（p<0.01）。SSc-RNP+患者更常发生间质性肺病（ILD）（73.4%vs.55.5%与31.3%,p<0.05)，肺纤维化（PF）（60.9%vs.37.5%与10.9%,p<0.0001),SSc相关肌病（29.7%vs.6.3%vs.7.8%,p<0.0001),和肾脏受累(10.9%vs.2.3%vs.1.6%,p<0.05)。经过200个月的随访，SSc-RNP+患者总生存期较差（p<0.05），无PF发生的生存率较差（p<0.01），ILD或PF进展(p<0.01和p<0.0001)。
结论：在SSc患者中，抗U1RNP抗体与较高的重叠综合征发生率相关，一个独特的临床表型，与SSc-RNP和MCTD患者相比，生存率较差。我们的研究表明，应将SSc-RNP患者与MCTD患者分开，并可能构成进行性肺病的丰富人群。
方法：我们进行了单中心病例对照，回顾性,纵向研究。对于每位具有SSc和抗U1RNP抗体（SSc-RNP）的患者，1例混合性结缔组织病（MCTD）患者和2例无抗U1RNP抗体（SSc-RNP-）的SSc患者的年龄相匹配，性别,和列入日期。
结果：将64例SSc-RNP+患者与128例SSc-RNP-和64例MCTD患者进行了比较。与SSc-RNP-相比，SSc-RNP+患者更常见于非洲裔加勒比裔(31.3%vs.11%,p<0.01),与SSc-RNP-患者相比，重叠综合征更常见(53.1%vs.22.7%,p<0.0001),与干燥综合征（n=23，35.9%）和/或系统性红斑狼疮（n=19，29.7%）重叠。SSc-RNP+患者与MCTD患者明显不同，但关节受累较少（p<0.01）。SSc-RNP+患者更常发生间质性肺病（ILD）（73.4%vs.55.5%与31.3%,p<0.05)，肺纤维化（PF）（60.9%vs.37.5%与10.9%,p<0.0001),SSc相关肌病（29.7%vs.6.3%vs.7.8%,p<0.0001),和肾脏受累(10.9%vs.2.3%vs.1.6%,p<0.05)。经过200个月的随访，SSc-RNP+患者总生存期较差（p<0.05），无PF发生的生存率较差（p<0.01），ILD或PF进展(p<0.01和p<0.0001)。
结论：在SSc患者中，抗U1RNP抗体与较高的重叠综合征发生率相关，一个独特的临床表型，与SSc-RNP和MCTD患者相比，生存率较差。我们的研究表明，应将SSc-RNP患者与MCTD患者分开，并可能构成进行性肺病的丰富人群。
