Department of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers (Amsterdam UMC), University of Amsterdam, Amsterdam, the Netherlands.
Department of Pediatric Rheumatology, Leiden University Medical Centre (LUMC), Leiden, the Netherlands.
Lupus. 2021 Apr;30(5):818-827. doi: 10.1177/0961203321998750. Epub 2021 Mar 3.
For selection of high-risk systemic lupus erythematosus (SLE) patients it is necessary to obtain indicators of disease severity that predict disease damage. As in systemic sclerosis, nailfold capillary abnormalities could be such a biomarker in SLE. The primary objective of this cross-sectional study is to describe capillary abnormalities in childhood-onset SLE (cSLE) cohort (onset < 18 years) and compare them with matched healthy controls. The secondary objective is to correlate the observed capillary abnormalities with demographical variables in both cohorts and with disease-specific variables in cSLE patients.
Healthy controls were matched for ethnic background, age and gender. Videocapillaroscopy was performed in eight fingers with 2-4 images per finger. Quantitative and qualitative assessments of nailfold capillaroscopy images were performed according to the definitions of the EULAR study group on microcirculation in Rheumatic Diseases.
Both groups (n = 41 cSLE-patients and n = 41 healthy controls) were comparable for ethnic background (p = 0.317). Counted per mm, cSLE-patients showed significantly more 'giants' (p = 0.032), 'abnormal capillary shapes' (p = 0.003), 'large capillary hemorrhages' (p < 0.001) and 'pericapillary extravasations' (p < 0.001). Combined 'abnormal capillary shapes and pericapillary extravasations' (in the same finger) were detected in 78% (32/41 patients). By qualitative analysis, 'microangiopathy' was detected in 68.3% (28/41) and a 'scleroderma pattern' in 17.1% (7/41) of the cSLE-patients (without scleroderma symptoms). The difference of percentage positive anti-RNP antibodies in the group with or without a scleroderma pattern was not significant (p = 0.089). The number of 'abnormal capillary shapes per mm' was significantly correlated with treatment-naivety. The number of 'large pathological hemorrhages per mm' was significantly correlated with SLEDAI score and presence of nephritis. Compared to healthy controls, 'pericapillary extravasations' were found in significantly higher numbers per mm (p < 0.001) as well as in percentage of patients (p < 0.001).
Our observations confirm that giants, abnormal capillary morphology and capillary hemorrhages are also observed in cSLE, as was already known for adults with SLE. Number of capillary hemorrhages in cSLE was significantly correlated with disease activity. A high frequency and total amount of "pericapillary extravasations" was observed in cSLE patients, possibly revealing a new subtype of capillary hemorrhage that might reflect endothelial damage in these pediatric patients.
为了选择高危系统性红斑狼疮（SLE）患者，有必要获得预测疾病损害的疾病严重程度指标。在系统性硬皮病中，甲褶毛细血管异常可能是 SLE 的此类生物标志物。本横断面研究的主要目的是描述儿童发病的 SLE（cSLE）队列（发病年龄<18 岁）中的毛细血管异常，并将其与匹配的健康对照组进行比较。次要目的是将观察到的毛细血管异常与两组患者的人口统计学变量以及 cSLE 患者的疾病特异性变量相关联。
健康对照组按种族背景、年龄和性别进行匹配。在 8 个手指上进行视频毛细血管镜检查，每个手指有 2-4 张图像。根据 EULAR 研究小组在风湿病中小血管的定义，对甲褶毛细血管镜图像进行定量和定性评估。
两组（n=41 例 cSLE 患者和 n=41 例健康对照组）在种族背景方面具有可比性（p=0.317）。按每毫米计算，cSLE 患者的“巨细胞”（p=0.032）、“异常毛细血管形态”（p=0.003）、“大毛细血管出血”（p<0.001）和“毛细血管周围渗出”（p<0.001）明显更多。在 78%（32/41 例患者）的同一手指中同时检测到“异常毛细血管形态和毛细血管周围渗出”。通过定性分析，在 68.3%（28/41）的 cSLE 患者中检测到“微血管病”，在 17.1%（7/41）的患者中检测到“硬皮病样模式”（无硬皮病症状）。有或没有硬皮病样模式的组中抗 RNP 抗体阳性的百分比差异无统计学意义（p=0.089）。每毫米的“异常毛细血管形态数”与治疗初治呈显著正相关。每毫米的“大病理性出血数”与 SLEDAI 评分和肾炎的存在显著相关。与健康对照组相比，cSLE 患者的毛细血管周围渗出数量（p<0.001）和患者百分比（p<0.001）明显更高。
我们的观察结果证实，巨细胞、异常毛细血管形态和毛细血管出血在 cSLE 中也存在，这在成人 SLE 中已经得到证实。cSLE 中毛细血管出血的数量与疾病活动度显著相关。在 cSLE 患者中观察到“毛细血管周围渗出”的频率和总量均较高，这可能揭示了一种新的毛细血管出血亚型，这可能反映了这些儿科患者的内皮损伤。
