From the Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia, Modena, Italy; ACURA Rheumatology Center, Bad Kreuznach, Germany; Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria, Santander, Spain; Rheumatology Division, University Hospital of Frankfurt, Frankfurt, Germany; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico; Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain; Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy; Rheumatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria (IIS) Princesa, Madrid; Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group, Barcelona, Spain; Department of Experimental and Clinical Medicine, and Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence, Florence; Unità Operativa Complessa (UOC) Reumatologia, Ospedale San Camillo-Forlanini, Rome; ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany; Rheumatology Department, Città Della Salute e della Scienza, Torino; Division of Rheumatology, University and Institute for Research and Health Care (IRCCS) Policlinico S. Matteo Foundation, Pavia, Italy; Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain; Dermatology Clinic, University Hospital of Trieste; Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara, Trieste; Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti di Trieste, Trieste, Italy; Medizinische Klinik A, Klinikum der Stadt, Ludwigshafen, Germany; Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa, Genoa, Italy; Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany; UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Italy; Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany; Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa; Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany; University of Ghent, Ghent University Hospital, Ghent, Belgium.
M. Sebastiani, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; A. Manfredi, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; G. Cassone, MD, Rheumatology Unit, Azienda Policlinico of Modena, University of Modena and Reggio Emilia; K. Triantafyllias, MD, ACURA Rheumatology Center; A. Schwarting, MD, Professor, ACURA Rheumatology Center; M.A. González-Gay, MD, Professor, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; N. Palmou-Fontana, MD, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDiVAL, University of Cantabria; U. Drott, MD, Rheumatology Division, University Hospital of Frankfurt; C. Delbrück, MD, Rheumatology Division, University Hospital of Frankfurt; J. Rojas-Serrano, MD, Professor, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; C. Bertolazzi, MD, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas; L. Nuño, MD, Servicio de Reumatología, Hospital Universitario La Paz; M. Giannini, MD, DIM, Rheumatology Unit, University of Bari; F. Iannone, MD, Professor, DIM, Rheumatology Unit, University of Bari; E.F. Vicente, MD, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; S. Castañeda, MD, Professor, Rheumatology Department, Hospital Universitario de la Princesa, IIS Princesa; A. Selva-O'Callaghan, MD, Professor, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; E. Trallero Araguas, MD, Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Universidad Autonoma de Barcelona, on behalf of the GEAS group; G. Emmi, MD, Department of Experimental and Clinical Medicine, University of Florence; A. Iuliano, MD, UOC Reumatologia, Ospedale San Camillo-Forlanini; J. Bauhammer, MD, ACURA Centre for Rheumatic Diseases; N. Miehle MD, ACURA Centre for Rheumatic Diseases; S. Parisi, MD, Rheumatology Department, Città Della Salute e della Scienza; L. Cavagna, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; V. Codullo, MD, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; C. Montecucco, MD, Professor, Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation; F.J. Lopez-Longo, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J. Martínez-Barrio, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; J.C. Nieto-González, MD, Servicio de Reumatología, Hospital General Universitario Gregorio Marañón; S. Vichi, MD, Dermatology Clinic, University Hospital of Trieste; M. Confalonieri, MD, Department of Pneumology and Respiratory Intermediate Care Unit, University Hospital of Cattinara; P. Tomietto, MD, Rheumatology Unit, Azienda Ospedaliero-Universitaria Ospedali Riuniti; R. Bergner, MD, Professor, Medizinische Klinik A, Klinikum der Stadt; A. Sulli, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; M. Cutolo, MD, Professor, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, San Martino Polyclinic Hospital IRCCS Genoa; F. Bonella, MD, Interstitial and Rare Lung Disease Unit, Ruhrlandklinik University Hospital, University of Duisburg-Essen; F. Furini, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C.A. Scirè, MD, Associate Professor, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; A. Bortoluzzi, MD, UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara; C. Specker, MD, Professor, Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic; S. Barsotti, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; R. Neri, MD, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Mosca, MD, Professor, Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa; M. Caproni, MD, Department of Medical and Surgical Critical Care, Section of Dermatology, University of Florence; J. Weinmann-Menke, MD, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; A. Schwarting, MD, Professor, Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg; V. Smith, MD, Professor, University of Ghent, Ghent University Hospital.
J Rheumatol. 2019 Mar;46(3):279-284. doi: 10.3899/jrheum.180355. Epub 2018 Nov 15.
To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease.
We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures.
NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP.
NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.
描述抗合成酶综合征（AS）患者的甲襞毛细血管显微镜检查（NVC）特征，并探讨其与疾病的临床和血清学特征之间的可能相关性。
我们回顾性分析了 190 例 AS 患者的 NVC 图像[女性/男性 3.63，平均年龄 49.7±12.8 岁，中位疾病病程 53.7 个月（四分位间距 82），133 例抗-Jo1 阳性和 57 例非抗-Jo1 阳性患者]。对于每位患者，我们检查了毛细血管数量、巨毛细血管、微出血、无血管区、分枝状毛细血管以及是否存在硬皮病样模式。最后，我们将 NVC 特征与 AS 患者的临床和血清学发现相关联。同时，我们还将一组 75 例抗核抗体阴性的原发性雷诺现象（RP）和长期随访的患者作为对照组（女性/男性比例 4.13/1，平均年龄 53.9±17.6 岁）进行 NVC 测量。
与原发性 RP 组的 29.3%相比，AS 患者的 NVC 异常发生率为 62.1%（p<0.001）。在 67 例患者中检测到硬皮病样模式（35.3%），它与抗-Jo1 抗体相关（p=0.002），并且与疾病病程较长相关（p=0.004）。有趣的是，硬皮病样模式的存在与 RP 之间没有显著相关性，并且只有 47%的硬皮病样模式患者存在 RP。
NVC 异常在 AS 中很常见，与 RP 的发生无关。硬皮病样模式的存在可能可以确定更明确的 AS 亚型，前瞻性研究可以证实其与 AS 的临床和血清学特征之间的关联。
