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      Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study

      research-article
      , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , for the EULAR Study Group on Microcirculation in Rheumatic Diseases
      Rheumatology (Oxford, England)
      Oxford University Press
      nailfold capillaroscopy, scleroderma pattern, juvenile rheumatic and musculoskeletal diseases, children, microcirculation

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          Abstract

          Objectives

          To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs).

          Material and methods

          In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis.

          Results

          A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively.

          Conclusions

          This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD.

          Graphical Abstract

          Graphical Abstract

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          Most cited references39

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          Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus

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            Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.

            The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE. The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios. Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001). The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies. Copyright © 2012 by the American College of Rheumatology.
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              2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative.

              The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by (1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and (2) validating against the combined view of a group of experts on a set of cases with or without SSc. It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, seven additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.
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                Author and article information

                Contributors
                Journal
                Rheumatology (Oxford)
                Rheumatology (Oxford)
                brheum
                Rheumatology (Oxford, England)
                Oxford University Press
                1462-0324
                1462-0332
                April 2023
                25 August 2022
                25 August 2022
                : 62
                : 4
                : 1605-1615
                Affiliations
                Department of Rheumatology, Ghent University Hospital , Ghent, Belgium
                Department of Internal Medicine, Ghent University , Ghent, Belgium
                Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova , Genoa, Italy
                IRCCS San Martino Polyclinic , Genoa, Italy
                Department of Pediatric Immunology, Rheumatology and Infectious diseases, Amsterdam University Medical Centres , Amsterdam, Netherlands
                Centre for Paediatric and Adolescent Rheumatology, An der Schön Klinik , Hamburg, Germany
                Department of Rheumatology, Ghent University Hospital , Ghent, Belgium
                Medical and Rheumatological Clinic, S. Maria Hospital , Terni, Italy
                Department of Immunology-Allergology, CHU Brugmann, Université Libre de Bruxelles , Brussels, Belgium
                Department of Dermatology, CHU Brugmann, Université Libre de Bruxelles , Brussels, Belgium
                Department of Rheumatology, CHU Brugmann, Université Libre de Bruxelles , Brussels, Belgium
                Pediatric Rheumatology Unit, Gaetano Pini Hospital, Department of Clinical Sciences and Community Health, Research Centre for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano , Milan, Italy
                Department of Pediatric Rheumatology, Ghent University Hospital , Ghent, Belgium
                Department of Public Health and Primary Care, Biostatistics Unit, Ghent University , Ghent, Belgium
                Department of Internal Medicine, Division of Rheumatology, University of Utah , Salt Lake City, UT, USA
                Salt Lake Veterans Affair Medical Centre, Utah Vascular Research Laboratory , Salt Lake City, UT, USA
                Department of Pediatrics, Universidad de Antioquia , Medellín, Colombia
                Clinical Rheumatology Unit , Gaetano Pini Hospital, Department of Clinical Sciences and Community Health, Research Centre for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano , Milan, Italy
                Department of Pediatric Rheumatology, SRCC Children’s Hospital , Mumbai, India
                Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin , Lublin, Poland
                Department of Pediatrics, University Medicine Gießen , Gießen, Germany
                Division of Rheumatology, Mayo Clinic , Rochester, MN, USA
                Rheumatology Section, Clínica Universitaria Universidad Pontificia Bolivariana , Medellín, Colombia
                Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin , Lublin, Poland
                Department of Rheumatology and Clinical Immunology, Campus Kerckhoff, Justus-Liebig University , Gießen, Germany
                Kerckhoff-Klinik , Bad Nauheim, Germany
                Division of Rheumatology, Hospital La Paz , Madrid, Spain
                Department of Internal Medicine, Division of Rheumatology, University of Utah , Salt Lake City, UT, USA
                University of Utah, Department of Pediatrics, Division of Pediatric Rheumatology , Salt Lake City, UT, USA
                Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova , Genoa, Italy
                IRCCS San Martino Polyclinic , Genoa, Italy
                Department of Internal Medicine, Division of Rheumatology, University of Utah , Salt Lake City, UT, USA
                University Hospital Split , Split, Croatia
                Department of Pediatric Rheumatology, SRCC Children’s Hospital , Mumbai, India
                Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini , Genoa, Italy
                University of Genoa , Genoa, Italy
                Sechenov First Moscow State Medical University , Moscow, Russian Federation
                Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini , Genoa, Italy
                Pediatric Rheumatology Department, La Paz Children’s Hospital , Madrid, Spain
                Department of Pediatric Immunology, Rheumatology and Infectious diseases, Amsterdam University Medical Centres , Amsterdam, Netherlands
                Centre for Musculoskeletal Research, University of Manchester , Manchester, UK
                Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester, UK
                Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova , Genoa, Italy
                IRCCS San Martino Polyclinic , Genoa, Italy
                Department of Rheumatology, Ghent University Hospital , Ghent, Belgium
                Department of Internal Medicine, Ghent University , Ghent, Belgium
                Unit for Molecular Immunology and Inflammation, Inflammation Research Centre, VIB-Ghent University , Ghent, Belgium
                Author notes
                Correspondence to: Vanessa Smith, Department of Rheumatology, Ghent University Hospital, Corneel Heymanslaan 10, 9000 Ghent, Belgium. E-mail: vanessa.smith@ 123456ugent.be

                Shared first authorship.

                See acknowledgements section for a list of the EULAR Study Group on Microcirculation in Rheumatic Diseases.

                Author information
                https://orcid.org/0000-0003-4125-7163
                https://orcid.org/0000-0002-6727-1273
                https://orcid.org/0000-0002-7190-1215
                https://orcid.org/0000-0003-4941-7926
                https://orcid.org/0000-0003-3674-4880
                https://orcid.org/0000-0001-6271-7945
                Article
                keac487
                10.1093/rheumatology/keac487
                10070071
                36005889
                81d99cb4-ee23-4e7f-8b87-5a2e25ca7f44
                © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License ( https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, [br]distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 28 March 2022
                : 17 July 2022
                : 22 September 2022
                Page count
                Pages: 11
                Funding
                Funded by: Research Foundation–Flanders;
                Award ID: 1.8.029.20N
                Categories
                Clinical Science
                AcademicSubjects/MED00360

                Rheumatology
                nailfold capillaroscopy,scleroderma pattern,juvenile rheumatic and musculoskeletal diseases,children,microcirculation

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