Bica Pietro Francesco, Hysa Elvis, Campitiello Rosanna, Sulli Alberto, Pizzorni Carmen, Gotelli Emanuele, Paolino Sabrina, Gimenez Ana Rebollo, Malattia Clara, Gattorno Marco, Rosina Silvia, Smith Vanessa, Cutolo Maurizio
Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine and Specialties (DIMI), University of Genova, Viale Benedetto XV, n°6, 16132, Genova, Italy.
IRCCS San Martino Polyclinic Hospital, Genova, Italy.
Clin Rheumatol. 2025 Jul 21. doi: 10.1007/s10067-025-07561-2.
Juvenile dermatomyositis (jDM) is a systemic autoimmune disease mainly affecting the skeletal muscle and skin. The aim of our study was to investigate the possible correlations between nailfold videocapillaroscopy (NVC) findings with clinical and laboratory parameters in patients with jDM.
Twenty-eight jDM patients (mean age 7.1 ± 3 years, mean disease duration 3.1 ± 3 years), evaluated between 2019 and 2023, classified according to Bohan and Peter criteria, were age- and sex-matched with healthy controls. Clinical, laboratory, and NVC parameters were collected at first visit and after 1 year again in a subgroup of patients (n = 9).
Positivity for myositis-specific autoantibodies (MSA) was associated with a lower mean capillary density (p = 0.02) in jDM patients. Additionally, the presence of giant capillaries was significantly more frequent in MSA-positive patients (p = 0.04). The skin component of the disease activity score (VAS and DAS skin score) showed a significant inverse correlation with capillary density (r = -0.53, p = 0.007) and was significantly higher in patients with giant capillaries (median 5.5 vs 1, p = 0.005). Periungual telangiectasias and Gottron's papules were significantly associated with a reduction of the mean capillary count and with the presence of giant capillaries (all p-values < 0.05). Interestingly, no significant changes in NVC parameters were reported after 1 year of receiving immunosuppressive treatment.
Capillary loss and the presence of giant capillaries at NVC analysis were significantly associated with clinical and laboratory markers of skin disease activity in jDM. These findings suggest that NVC may serve as a promising non-invasive tool to support disease assessment, although further prospective studies are needed to confirm its role in clinical practice. Key Points • Nailfold videocapillaroscopy (NVC) abnormalities, particularly capillary loss and giant capillaries, correlate significantly with skin disease activity in juvenile dermatomyositis (jDM). • Myositis-specific antibodies (MSA), especially anti-TIF1γ, are associated with more severe microvascular damage, suggesting their potential role in patient stratification. • NVC parameters remained stable over 1 year of immunosuppressive treatment, highlighting the need for longer-term studies to evaluate microvascular change progressions in jDM. • NVC may represent a promising non-invasive biomarker to support the clinical assessment and follow-up of patients with jDM, but further validation in larger prospective studies is warranted.
青少年皮肌炎(jDM)是一种主要影响骨骼肌和皮肤的全身性自身免疫性疾病。我们研究的目的是调查青少年皮肌炎患者甲襞视频毛细血管镜检查(NVC)结果与临床及实验室参数之间可能存在的相关性。
2019年至2023年间评估的28例青少年皮肌炎患者(平均年龄7.1±3岁,平均病程3.1±3年),根据博汉和彼得标准进行分类,在年龄和性别上与健康对照匹配。在首次就诊时以及1年后对部分患者(n = 9)再次收集临床、实验室和NVC参数。
青少年皮肌炎患者中,肌炎特异性自身抗体(MSA)阳性与较低的平均毛细血管密度相关(p = 0.02)。此外,巨大毛细血管的存在在MSA阳性患者中显著更常见(p = 0.04)。疾病活动评分的皮肤部分(视觉模拟评分法和皮肤疾病面积评分)与毛细血管密度呈显著负相关(r = -0.53,p = 0.007),并且在有巨大毛细血管的患者中显著更高(中位数5.5对1,p =