Codullo Veronica, Cavazzana Ilaria, Bonino Claudia, Alpini Claudia, Cavagna Lorenzo, Cozzi Franco, Del Papa Nicoletta, Franceschini Franco, Guiducci Serena, Morozzi Gabriella, Ruffatti Amelia, Ferri Clodoveo, Giacomelli Roberto, Matucci-Cerinic Marco, Valentini Gabriele, Montecucco Carlomaurizio
Division of Rheumatology, University of Pavia, IRCCS Policlinico San Matteo Foundation, Pavia, 27100, Italy.
J Rheumatol. 2009 Jul;36(7):1464-9. doi: 10.3899/jrheum.080806. Epub 2009 Jun 1.
To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc).
A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit III, and immunoprecipitation (IP) were performed.
Forty-six cases (38 female; 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years +/- 13.2 and 55.4 years +/- 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%), anti-RNAP I-III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I-III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being lower in the anti-topo I than in the anti-RNAP I-III group (p = 0.034).
SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I-III in our patients with SRC and was associated with delayed onset and high mortality rates.
分析意大利系统性硬化症（SSc）患者中硬皮病肾危象（SRC）患者的临床和血清学特征。
对9个意大利风湿病转诊中心的病历进行回顾性分析。纳入所有发生SRC且在危象发生时可获得血清样本的患者。采用间接免疫荧光法检测抗核抗体（ANA），酶联免疫吸附测定（ELISA）检测抗拓扑异构酶（topo）I，ELISA检测抗RNA聚合酶（RNAP）亚基III，并进行免疫沉淀（IP）检测。
共识别出46例患者（38例女性；40例弥漫性皮肤型SSc）。SSc和SRC发病时的平均年龄分别为52.8岁±13.2岁和55.4岁±11.8岁。44例患者（96%）存在ANA。30例（65%）检测到抗topo I抗体，7例（15%）检测到抗RNAP I-III抗体。这两组患者的主要临床特征无差异。抗RNAP I-III组患者在SSc病程中早期（<18个月）发生SRC的比例显著更高（p = 0.03）。随访1年、2年和10年时的累积生存率分别为64%、53%和35%。SSc患者的生存率根据其自身抗体谱有显著差异，抗topo I组低于抗RNAP I-III组（p = 0.034）。
在意大利，SRC是SSc的一种罕见表现，但仍与严重预后相关。在我们的SRC患者中，抗topo I反应比抗RNAP I-III更常见，且与发病延迟和高死亡率相关。
