Bernero E, Sulli A, Ferrari G, Ravera F, Pizzorni C, Ruaro B, Zampogna G, Alessandri E, Cutolo M
Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa.
Reumatismo. 2013 Oct 31;65(4):186-91. doi: 10.4081/reumatismo.2013.186.
The objective of this prospective study was to investigate the transition from primary (PRP) to secondary (SRP) Raynaud's phenomenon (RP), in a large cohort of patients affected by isolated RP. A total of 2065 patients with RP were investigated by clinical interview, laboratory examinations, and nailfold videocapillaroscopy (NVC). Patients with negative NVC at first visit were yearly followed to monitor either the appearance of specific morphological alterations at NVC, or clinical manifestations of an underlying disease. Capillary abnormalities at NVC were scored, as well as the qualitative patterns of microangiopathy (Early, Active and Late). NVC was found negative at first visit in 1500 subjects; among them, 412 patients were evaluable and they were followed for a mean time of 5±4 years (range 2-13 years). Sixty-eight patients (16%) achieved a diagnosis of SRP during follow-up, showing normal or not specific capillary alterations at NVC 4% of patients (the diagnosis was undifferentiated connective tissue diseases), Early scleroderma-pattern 57%, Active scleroderma-pattern 7%, Late scleroderma-pattern 12%, and scleroderma-like pattern 18% of patients. The time of transition from normal/not specific capillary alterations to Early scleroderma-pattern was 4.4±3.8 years. Enlarged capillaries (diameter between 20 and 50 microns) and mild reduction of capillary density were found the more frequent markers at first NVC visit in patients who progressed to a scleroderma pattern (P=0.01). This study demonstrates in a large cohort, that almost 16% of patients initially diagnosed as affected by RP with negative NVC may transit to SRP during a mean follow-up of 4.4 years. PRP patients showing major notspecific alterations of nailfold capillaries at first NVC should be strictly monitored at least once a year since at higher risk of transition to SRP.
这项前瞻性研究的目的是，在一大群孤立性雷诺现象（RP）患者中，调查原发性雷诺现象（PRP）向继发性雷诺现象（SRP）的转变情况。通过临床访谈、实验室检查和甲襞视频毛细血管镜检查（NVC）对总共2065例RP患者进行了调查。首次就诊时NVC结果为阴性的患者每年接受随访，以监测NVC是否出现特定形态学改变或潜在疾病的临床表现。对NVC的毛细血管异常进行评分，以及微血管病变的定性模式（早期、活动期和晚期）。1500名受试者首次就诊时NVC结果为阴性；其中，412例患者可进行评估，他们的平均随访时间为5±4年（范围2 - 13年）。68例患者（16%）在随访期间确诊为SRP，NVC显示正常或非特异性毛细血管改变的患者占4%（诊断为未分化结缔组织病），早期硬皮病模式占57%，活动期硬皮病模式占7%，晚期硬皮病模式占12%，硬皮病样模式占18%。从正常/非特异性毛细血管改变转变为早期硬皮病模式的时间为4.4±3.8年。在进展为硬皮病模式的患者中，首次NVC检查时发现较大的毛细血管（直径在20至50微米之间）和毛细血管密度轻度降低是较常见的指标（P = 0.01）。这项研究在一大群患者中表明，最初诊断为NVC阴性的RP患者中，近16%可能在平均4.4年的随访期间转变为SRP。首次NVC检查时甲襞毛细血管出现主要非特异性改变的PRP患者，由于转变为SRP的风险较高，应至少每年进行一次严格监测。
