Pietro Francesco Bica, Elvis Hysa, Rosanna Campitiello, Alberto Sulli, Carmen Pizzorni, Emanuele Gotelli, Sabrina Paolino, Ana Rebollo Gimenez, Clara Malattia, Marco Gattorno, Silvia Rosina, Vanessa Smith, Maurizio Cutolo
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Clinical, laboratory, and NVC parameters were collected at first visit and after 1 year again in a subgroup of patients (n = 9).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.8000,"publicationDate":"2025-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Nailfold videocapillaroscopy in juvenile dermatomyositis: detailed correlations between microvascular abnormalities with clinical and laboratory parameters from an observational retrospective single-center study.\",\"authors\":\"Pietro Francesco Bica, Elvis Hysa, Rosanna Campitiello, Alberto Sulli, Carmen Pizzorni, Emanuele Gotelli, Sabrina Paolino, Ana Rebollo Gimenez, Clara Malattia, Marco Gattorno, Silvia Rosina, Vanessa Smith, Maurizio Cutolo\",\"doi\":\"10.1007/s10067-025-07561-2\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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. 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引用次数: 0
摘要
青少年皮肌炎(jDM)是一种主要影响骨骼肌和皮肤的系统性自身免疫性疾病。本研究的目的是探讨jDM患者的甲襞视频毛细血管镜(NVC)检查结果与临床和实验室参数之间的可能相关性。方法:2019 - 2023年间,28例jDM患者(平均年龄7.1±3岁,平均病程3.1±3年)与健康对照者年龄、性别匹配,按照Bohan和Peter标准进行分类。第一次就诊时收集临床、实验室和NVC参数,1年后再次收集亚组患者(n = 9)的NVC参数。结果:jDM患者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,msa阳性患者出现巨毛细血管的频率明显更高(p = 0.04)。疾病活动度评分的皮肤成分(VAS和DAS皮肤评分)与毛细血管密度呈显著负相关(r = -0.53, p = 0.007),而巨毛细血管患者的皮肤成分明显更高(中位数5.5 vs 1, p = 0.005)。甲周毛细血管扩张和Gottron丘疹与平均毛细血管计数减少和巨毛细血管的存在显著相关(p值均< 0.05)。有趣的是,接受免疫抑制治疗1年后,NVC参数没有明显变化。结论:NVC分析中毛细血管损失和巨毛细血管的存在与jDM中皮肤病活动性的临床和实验室标志物显著相关。这些发现表明,尽管需要进一步的前瞻性研究来证实其在临床实践中的作用,但NVC可能作为一种有前途的非侵入性工具来支持疾病评估。•甲襞视频毛细血管镜检查(NVC)异常,特别是毛细血管丢失和巨毛细血管,与青少年皮肌炎(jDM)的皮肤病活动显著相关。•肌炎特异性抗体(MSA),尤其是抗tif1γ抗体,与更严重的微血管损伤相关,提示其在患者分层中的潜在作用。•NVC参数在1年免疫抑制治疗期间保持稳定,强调需要更长期的研究来评估jDM的微血管变化进展。•NVC可能是一种很有前途的非侵入性生物标志物,可以支持jDM患者的临床评估和随访,但需要在更大规模的前瞻性研究中进一步验证。
Nailfold videocapillaroscopy in juvenile dermatomyositis: detailed correlations between microvascular abnormalities with clinical and laboratory parameters from an observational retrospective single-center study.
Introduction: 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.
Methods: 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).
Results: 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.
Conclusions: 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.
期刊介绍:
Clinical Rheumatology is an international English-language journal devoted to publishing original clinical investigation and research in the general field of rheumatology with accent on clinical aspects at postgraduate level.
The journal succeeds Acta Rheumatologica Belgica, originally founded in 1945 as the official journal of the Belgian Rheumatology Society. Clinical Rheumatology aims to cover all modern trends in clinical and experimental research as well as the management and evaluation of diagnostic and treatment procedures connected with the inflammatory, immunologic, metabolic, genetic and degenerative soft and hard connective tissue diseases.