Institute for Experimental Medical Research and K. G. Jebsen Center for Cardiac Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway, Institute for Translational Medicine, Medical School, University of Pécs, Pécs, Hungary, and Bjørknes University College, Oslo, Norway.
Institute for Experimental Medical Research and K. G. Jebsen Center for Cardiac Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway.
Arthritis Care Res (Hoboken). 2019 Apr;71(4):492-497. doi: 10.1002/acr.23687.
To explore the associations between microvascular abnormalities as assessed by nailfold capillaroscopy (NFC) and pulmonary and cardiac involvement in patients with juvenile dermatomyositis (DM) who are assessed after medium- to long-term follow-up.
Fifty-eight patients with juvenile DM were examined a mean ± SD of 17.0 ± 10.6 years after symptom onset. Nailfold capillary density (NCD) and a neovascular pattern (defined as an active or late scleroderma pattern) were analyzed, with blinding to clinical data. Pulmonary involvement was assessed by pulmonary function tests including spirometry, diffusing capacity for carbon monoxide (DLco), and body plethysmography. High-resolution computed tomography (HRCT) was also performed. Cardiac involvement was assessed by electrocardiography, Holter monitoring (heart rate variability), and echocardiography.
Patients with low NCD (<6 capillaries/mm) (n = 21), compared to patients with normal NCD (≥6 capillaries/mm) (n = 37) had lower forced vital capacity (89.7% versus 98.5% predicted), total lung capacity (87.8% versus 94.5% predicted), and more often had low DLco values (15 [71%] of 21 patients versus 14 [38%] of 37 controls) (all P < 0.05). Use of HRCT to assess airway disease was more frequent in the group with low NCD (6 [30%] of 20 patients versus 3 [8%] of 36 patients in the normal NCD group; P = 0.034). No associations between NCD and cardiac parameters or between neovascular pattern and pulmonary or cardiac parameters were observed.
In patients with juvenile DM, low NCD was associated with lung involvement, which was mostly subclinical. No significant associations with cardiac involvement were observed. These results shed light on possible mechanisms underlying organ involvement, but further and preferably larger studies are needed to identify NCD as a potential biomarker for lung and cardiac involvement in juvenile DM.
探讨经甲襞毛细血管显微镜检查（NFC）评估的微血管异常与中至长期随访后青少年皮肌炎（DM）患者的肺部和心脏受累之间的关系。
对 58 例青少年 DM 患者在症状出现后平均（±标准差）17.0 ± 10.6 年进行了检查。分析了甲襞毛细血管密度（NCD）和新生血管模式（定义为活跃或晚期硬皮病模式），并对临床数据进行了盲法分析。通过肺功能检查（包括肺活量计、一氧化碳弥散量（DLco）和体描法）评估肺部受累情况。还进行了高分辨率计算机断层扫描（HRCT）。通过心电图、动态心电图监测（心率变异性）和超声心动图评估心脏受累情况。
与正常 NCD（≥6 个毛细血管/mm）（n = 37）相比，NCD 低（<6 个毛细血管/mm）（n = 21）的患者的用力肺活量（预测值的 89.7%）、总肺活量（预测值的 87.8%）较低，且低 DLco 值更为常见（21 例患者中有 15 例[71%]，37 例对照中有 14 例[38%]）（均 P < 0.05）。使用 HRCT 评估气道疾病的患者在 NCD 低的患者中更为常见（20 例患者中有 6 例[30%]，36 例正常 NCD 患者中有 3 例[8%]；P = 0.034）。NCD 与心脏参数之间或新生血管模式与肺部或心脏参数之间均无关联。
在青少年 DM 患者中，NCD 低与肺部受累有关，且主要为亚临床受累。未观察到 NCD 与心脏受累之间有显著关联。这些结果揭示了可能导致器官受累的潜在机制，但需要进一步的、最好是更大规模的研究来确定 NCD 是否可以作为青少年 DM 肺部和心脏受累的潜在生物标志物。
