Repa Argyro, Avgoustidis Nestor, Kougkas Nikos, Bertsias George, Zafiriou Michalis, Sidiropoulos Prodromos
Department of Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Heraklion, Greece.
Mediterr J Rheumatol. 2019 Mar 28;30(1):48-50. doi: 10.31138/mjr.30.1.48. eCollection 2019 Mar.
Systemic Sclerosis (SSc) is a rare, multisystemic connective tissue disease associated with significant morbidity. Early recognition of patients at risk for adverse prognosis may help towards optimized monitoring and treatment, thus improving disease outcome.
To correlate nailfold videocapillaroscopy (NVC) findings ('early', 'active', 'late' scleroderma patterns and non-specific capillary abnormalities) with major organ involvement and prognosis in patients with systemic sclerosis (SSc).
Patients from the Scleroderma cohort followed at the Rheumatology clinic of the University Hospital of Heraklion will be included. The study will include a prospective and a retrospective part. : All newly diagnosed patients will undergo NVC at baseline and subsequently every six months. We will review demographics, clinical features and autoantibodies status. Major organ involvement will be monitored (Pulmonary Function Test, DLCO, heart echocardiogram, chest XR, modified Rodnan skin score) at baseline and then every 6-12 months. : Existing SSc patients with available NVC data at diagnosis will be included. We will correlate the NVC findings at the time of diagnosis with disease outcomes such as major organ involvement, end stage organ failure, need for hospitalization, and death. We will also correlate longitudinal changes of the NVC patterns with treatment responses and outcomes.
系统性硬化症(SSc)是一种罕见的多系统结缔组织疾病,会导致严重的发病率。早期识别有不良预后风险的患者可能有助于优化监测和治疗,从而改善疾病结局。
将甲襞视频毛细血管镜检查(NVC)结果(“早期”、“活动期”、“晚期”硬皮病模式和非特异性毛细血管异常)与系统性硬化症(SSc)患者的主要器官受累情况及预后相关联。
将纳入在伊拉克利翁大学医院风湿病诊所随访的硬皮病队列中的患者。该研究将包括前瞻性和回顾性两部分。:所有新诊断的患者将在基线时接受NVC检查,随后每六个月进行一次。我们将审查人口统计学、临床特征和自身抗体状态。在基线时以及随后每6 - 12个月监测主要器官受累情况(肺功能测试、一氧化碳弥散量、心脏超声心动图、胸部X线、改良Rodnan皮肤评分)。:将纳入诊断时具有可用NVC数据的现有SSc患者。我们将把诊断时的NVC结果与疾病结局相关联,如主要器官受累、终末期器官衰竭、住院需求和死亡。我们还将把NVC模式的纵向变化与治疗反应和结局相关联。