From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands.
A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen.
J Rheumatol. 2019 Sep;46(9):1109-1116. doi: 10.3899/jrheum.180615. Epub 2018 Dec 15.
To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT).
NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Abnormal PFT was defined as forced vital or diffusion capacity < 70%. Patients were classified as primary RP (n = 245), or secondary: no definite diagnosis (n = 391), SSc (n = 40), primary Sjögren syndrome (pSS; n = 30), systemic lupus erythematosus (SLE; n = 30), mixed connective tissue disease (MCTD; n = 7), rheumatoid arthritis (RA; n = 15).
An SSc pattern on NCM was frequently observed in most patients with a definite diagnosis: SSc (88%), pSS (33%), SLE (17%), MCTD (71%), and RA (13%). In patients without definite diagnosis, 17% had a normal NCM pattern, 35% nonspecific, and 48% SSc pattern. Abnormal PFT was more frequent in patients with an SSc pattern (35.9% vs 19.5%, p = 0.002), even when corrected for SSc diagnosis (p = 0.003). Absence of an SSc pattern had high negative predictive value (88%); positive predictive values were low.
SSc pattern on NCM is common in patients with RP, and in those with connective tissue diseases other than SSc. It is associated with a higher prevalence of abnormal PFT, independent of the presence of an SSc diagnosis. Although these data need validation in a prospective setting, they underline the importance of NCM in RP and putative value to stratify the risk of pulmonary involvement in early stages of disease.
评估甲襞毛细血管显微镜（NCM）在雷诺现象（RP）患者中是否存在系统性硬化症（SSc）模式，并探讨其与异常肺功能测试（PFT）的关系。
对 759 例连续 RP 患者进行 NCM 模式评估。模式分为正常（n=354）、非特异性（n=159）或 SSc 模式（n=246）。异常 PFT 定义为用力肺活量或弥散量<70%。患者分为原发性 RP（n=245）或继发性：无明确诊断（n=391）、SSc（n=40）、原发性干燥综合征（pSS；n=30）、系统性红斑狼疮（SLE；n=30）、混合性结缔组织病（MCTD；n=7）、类风湿关节炎（RA；n=15）。
在大多数有明确诊断的患者中，NCM 上的 SSc 模式经常出现：SSc（88%）、pSS（33%）、SLE（17%）、MCTD（71%）和 RA（13%）。在无明确诊断的患者中，17%有正常的 NCM 模式，35%非特异性，48%为 SSc 模式。SSc 模式患者异常 PFT 更常见（35.9%比 19.5%，p=0.002），即使校正 SSc 诊断后仍如此（p=0.003）。缺乏 SSc 模式具有高阴性预测值（88%）；阳性预测值较低。
在 RP 患者中，尤其是在结缔组织病患者中，NCM 上的 SSc 模式很常见。它与异常 PFT 的发生率较高相关，与 SSc 诊断无关。尽管这些数据需要在前瞻性研究中验证，但它们强调了 NCM 在 RP 中的重要性，并可能具有在疾病早期分层肺受累风险的价值。
