University of Modena and Reggio Emilia, Italy.
University of Florence, Italy.
Clin Exp Rheumatol. 2020 May-Jun;38 Suppl 125(3):40-47. Epub 2020 Apr 14.
Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation).
The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc.
Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features.
Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.
系统性硬化症（SSc）是一种严重的多器官疾病，其特征为临床病程不可预测、治疗反应不足和预后不良。全国性 SSc 登记处可能为描述性和预后研究提供所需的大量具有代表性的患者队列。因此，意大利风湿病学会推动了登记处 SPRING（系统性硬化症进展研究）。
SPRING 是一项多中心风湿病队列研究，涵盖了广泛的硬皮病谱，即原发性雷诺现象（pRP）、疑似继发性 RP、早期系统性硬化症诊断（VEDOSS）和明确的 SSc。在这里，我们描述了初始招募阶段的 2028 名意大利患者的人口统计学和临床特征，主要集中在 1538 名明确 SSc 患者的队列上。
明确的 SSc 显示出更高的发生率，如手指溃疡、毛细血管镜“晚期”模式、食管和心肺受累，与 VEDOSS 相比，这是基于所遵循的分类标准预期的。对明确的 SSc 进行深入分析发现，男性、弥漫性皮肤亚型和抗 Scl70 阳性与更严重疾病表现的发生率增加显著相关。同样，在 SSc 诊断时 RP 持续时间（≤1 年）非常短的患者，具有统计学上增加的不良临床血清学特征发生率。
全国性登记处对患者进行适当的亚组划分，并从疾病前驱期开始进行随访研究，可能会为我们提供有关 SSc 自然史和主要预后因素的宝贵见解。
