西班牙单中心研究系统性硬皮病患者抗 RNA 聚合酶 III 抗体的临床特征。
Clinical features of systemic sclerosis patients with anti-RNA polymerase III antibody in a single centre in Spain.
机构信息
Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Parc Taulí, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain.
Unit of Systemic Autoimmune Diseases, Institut de Recerca Vall d'Hebron, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain.
出版信息
Clin Exp Rheumatol. 2019 Jul-Aug;37 Suppl 119(4):41-48. Epub 2019 Feb 11.
OBJECTIVES
To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre.
METHODS
We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Epidemiological, clinical, immunological features and survival were analysed.
RESULTS
In patients with anti-RNAP III positivity diffuse cutaneous SSc (dcSSc) subset was the most prevalent (20, 76.9% vs. 35, 17.9%, p < 0.001), with shorter diagnosis delay (4.11 ± 7.34 years vs. 6.77 ± 9.22 years, p = 0.005). Patients with anti-RNAP III antibodies had higher frequency of arterial hypertension (13, 50% vs. 55, 28.2%, p = 0.024), scleroderma renal crisis (SRC) (3, 11.5% vs. 3, 1.5%, p = 0.023), arthritis (9, 34.6% vs. 35, 17.9%, p = 0.046), tendon friction rubs (4, 15.4% vs. 1, 0.5%, p = 0.001) and contractures (5, 19.2% vs. 10, 5.1%, p = 0.02). There were no differences found in the presence of cancer or in global survival. In the multivariate survival analysis, severe interstitial lung disease (ILD) (HR: 8.61, 95%CI 3.40 - 21.81), pulmonary arterial hypertension (PAH) (HR: 4.05, 95%CI 1.42 - 11.61) and SRC (HR: 17.27, 95%CI 3.36 - 88.97) were the only factors associated with poor prognosis.
CONCLUSIONS
In this cohort anti-RNAP III antibodies are related with dcSSc subset, shorter diagnostic delay and higher prevalence of musculoskeletal involvement, arterial hypertension and SRC. ILD, PAH and SRC were independent prognostic factors.
目的
评估西班牙单一中心抗 RNA 聚合酶 III（抗-RNAP III）阳性患者的临床特征和生存情况。
方法
我们根据 LeRoy 和 Medsger 标准分析了 221 例 SSc 患者。将 26 例抗-RNAP III 抗体阳性患者与 195 例阴性患者进行比较。分析了流行病学、临床、免疫学特征和生存情况。
结果
抗-RNAP III 阳性患者中弥漫性皮肤 SSc（dcSSc）亚组最为常见（20 例，76.9% vs. 35 例，17.9%，p < 0.001），诊断延迟时间更短（4.11 ± 7.34 年 vs. 6.77 ± 9.22 年，p = 0.005）。抗-RNAP III 抗体阳性患者高血压（13 例，50% vs. 55 例，28.2%，p = 0.024）、硬皮病肾危象（SRC）（3 例，11.5% vs. 3 例，1.5%，p = 0.023）、关节炎（9 例，34.6% vs. 35 例，17.9%，p = 0.046）、肌腱摩擦音（4 例，15.4% vs. 1 例，0.5%，p = 0.001）和挛缩（5 例，19.2% vs. 10 例，5.1%，p = 0.02）更为常见。两组癌症发生率或总体生存率无差异。在多变量生存分析中，严重间质性肺病（ILD）（HR：8.61，95%CI 3.40 - 21.81）、肺动脉高压（PAH）（HR：4.05，95%CI 1.42 - 11.61）和 SRC（HR：17.27，95%CI 3.36 - 88.97）是唯一与预后不良相关的因素。
结论
在本队列中，抗-RNAP III 抗体与 dcSSc 亚型、诊断延迟时间更短、肌肉骨骼受累、高血压和 SRC 发生率较高有关。ILD、PAH 和 SRC 是独立的预后因素。
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