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Journal ArticleDOI

Quantitative nailfold capillaroscopy-update and possible next steps.

14 May 2021-Rheumatology (Oxford Academic)-Vol. 60, Iss: 5, pp 2054-2065
TL;DR: In this article, the potential for precise discrimination between normal and abnormal capillaries and for reliable measurement of disease progression and treatment response is discussed. And the potential of automated analysis could facilitate large-scale prospective studies using capillaroscopic parameters as possible biomarkers of systemic sclerosis spectrum disorders.
Abstract: We review the exciting potential (and challenges) of quantitative nailfold capillaroscopy, focusing on its role in systemic sclerosis. Quantifying abnormality, including automated analysis of nailfold images, overcomes the subjectivity of qualitative/descriptive image interpretation. First we consider the rationale for quantitative analysis, including the potential for precise discrimination between normal and abnormal capillaries and for reliable measurement of disease progression and treatment response. We discuss nailfold image acquisition and interpretation, and describe how early work on semi-quantitative and quantitative analysis paved the way for semi-automated and automated analysis. Measurement of red blood cell velocity is described briefly. Finally we give a personal view on 'next steps'. From a clinical perspective, increased uptake of nailfold capillaroscopy by general rheumatologists could be achieved via low-cost hand-held devices with cloud-based automated analysis. From a research perspective, automated analysis could facilitate large-scale prospective studies using capillaroscopic parameters as possible biomarkers of systemic sclerosis-spectrum disorders.
Citations
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Journal ArticleDOI
TL;DR: A review of non-invasive tools for analyzing microvascular changes is presented in this article, and the clinical roles of validated or tested imaging methods are discussed for autoimmune rheumatic diseases.
Abstract: Morphological and functional analysis of the microcirculation are objective outcome measures that are recommended for use in the presence of clinical signs of altered peripheral blood flow (such as Raynaud phenomenon), which can occur in systemic sclerosis (SSc) and other autoimmune rheumatic diseases. Several advanced non-invasive tools are available for monitoring the microcirculation, including nailfold videocapillaroscopy, which is the best-studied and most commonly used method for distinguishing and quantifying microvascular morphological alterations in SSc. Nailfold videocapillaroscopy can also be used alongside laser Doppler techniques to assist in the early diagnosis and follow-up of patients with dermatomyositis or mixed connective tissue disease. Power Doppler ultrasonography, which has been used for many years to evaluate the vascularity of synovial tissue in rheumatoid arthritis, is another promising tool for the analysis of skin and nailbed capillary perfusion in other autoimmune rheumatic diseases. Other emerging methods include raster-scanning optoacoustic mesoscopy, which offers non-invasive high-resolution 3D visualization of capillaries and has been tested in psoriatic arthritis and SSc. The principle functions and operative characteristics of several non-invasive tools for analysing microvascular changes are outlined in this Review, and the clinical roles of validated or tested imaging methods are discussed for autoimmune rheumatic diseases.

31 citations

Journal ArticleDOI
TL;DR: The growing interest in the visualization of psoriatic nail unit changes has led to the discovery of an abundance of image characteristics across various modalities.
Abstract: The growing interest in the visualization of psoriatic nail unit changes has led to the discovery of an abundance of image characteristics across various modalities.

7 citations

Journal ArticleDOI
TL;DR: An automated image analysis method for segmentation and mapping of capillary flow dynamics captured using nailfold video capillaroscopy (NVC) is developed and compared between young and middle‐aged healthy controls.
Abstract: This study aimed to develop an automated image analysis method for segmentation and mapping of capillary flow dynamics captured using nailfold video capillaroscopy (NVC). Methods were applied to compare capillary flow structures and dynamics between young and middle‐aged healthy controls.

3 citations

Journal ArticleDOI
TL;DR: In this article , the performance and reliability of a vision transformer (ViT)-based deep-learning model for identifying distinct signs of microangiopathy in nailfold capilloroscopy (NFC) images of patients with SSc was compared with that of practising rheumatologists.
Abstract: Abstract Objectives The first objective of this study was to implement and assess the performance and reliability of a vision transformer (ViT)-based deep-learning model, an ‘off-the-shelf’ artificial intelligence solution, for identifying distinct signs of microangiopathy in nailfold capilloroscopy (NFC) images of patients with SSc. The second objective was to compare the ViT’s analysis performance with that of practising rheumatologists. Methods NFC images of patients prospectively enrolled in our European Scleroderma Trials and Research group (EUSTAR) and Very Early Diagnosis of Systemic Sclerosis (VEDOSS) local registries were used. The primary outcome investigated was the ViT’s classification performance for identifying disease-associated changes (enlarged capillaries, giant capillaries, capillary loss, microhaemorrhages) and the presence of the scleroderma pattern in these images using a cross-fold validation setting. The secondary outcome involved a comparison of the ViT’s performance vs that of rheumatologists on a reliability set, consisting of a subset of 464 NFC images with majority vote–derived ground-truth labels. Results We analysed 17 126 NFC images derived from 234 EUSTAR and 55 VEDOSS patients. The ViT had good performance in identifying the various microangiopathic changes in capillaries by NFC [area under the curve (AUC) from 81.8% to 84.5%]. In the reliability set, the rheumatologists reached a higher average accuracy, as well as a better trade-off between sensitivity and specificity compared with the ViT. However, the annotators’ performance was variable, and one out of four rheumatologists showed equal or lower classification measures compared with the ViT. Conclusions The ViT is a modern, well-performing and readily available tool for assessing patterns of microangiopathy on NFC images, and it may assist rheumatologists in generating consistent and high-quality NFC reports; however, the final diagnosis of a scleroderma pattern in any individual case needs the judgement of an experienced observer.

2 citations

Journal ArticleDOI
TL;DR: The challenges, challenges, provides tips, and highlights the exciting potential of nailfold capillaroscopy in standard practice are presented.

1 citations

References
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Journal ArticleDOI
TL;DR: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria and should allow for more patients to be classified correctly as having the disease.
Abstract: OBJECTIVE: 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. METHODS: 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. RESULTS: 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, 7 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. CONCLUSION: 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.

2,743 citations

Journal ArticleDOI
TL;DR: In RP evolving to definite SSc, microvascular damage is dynamic and sequential, while SSc-specific autoantibodies are associated with the course and type of capillary abnormalities.
Abstract: Objective To identify in patients with Raynaud's phenomenon (RP) independent markers that predict progression to definite systemic sclerosis (SSc) and to determine in patients with progression to SSc the type and sequence of microvascular damage and its relationship to SSc-specific autoantibodies. Methods Consecutive patients referred for evaluation of RP who had no definite connective tissue disease were evaluated for microvascular damage by nailfold capillary microscopy (NCM) and for anticentromere (anti–CENP-B), anti-Th/To, anti–topoisomerase I, and anti–RNA polymerase III (anti–RNAP III) autoantibodies by specific assays. Patients were studied prospectively. Results Of the 586 patients who were followed up for 3,197 person-years, 74 (12.6%) developed definite SSc. A characteristic sequence of microvascular damage was identified, starting with enlarged capillaries, followed by capillary loss, and then by capillary telangiectases. Definite SSc was diagnosed in close temporal relationship to capillary loss. Enlarged capillaries, capillary loss, and SSc-specific autoantibodies independently predicted definite SSc. Anti–CENP-B and anti-Th/To antibodies predicted enlarged capillaries; these autoantibodies and anti–RNAP III predicted capillary loss. Each autoantibody was associated with a distinct time course of microvascular damage. At followup, 79.5% of patients with 1 of these autoantibodies and abnormal findings on NCM at baseline had developed definite SSc. Patients with both baseline predictors were 60 times more likely to develop definite SSc. The data validated the proposed criteria for early SSc. Conclusion In RP evolving to definite SSc, microvascular damage is dynamic and sequential, while SSc-specific autoantibodies are associated with the course and type of capillary abnormalities. Abnormal findings on NCM at baseline together with an SSc-specific autoantibody indicate a very high probability of developing definite SSc, whereas their absence rules out this outcome.

518 citations

Journal ArticleDOI
Jérôme Avouac1, Jaap Fransen2, Ulrich A. Walker3, Valeria Riccieri4, Vanessa Smith5, Carolina de Souza Müller6, I. Miniati7, Ingo H. Tarner8, S. Bellando Randone6, Maurizio Cutolo9, Yannick Allanore1, Oliver Distler10, Gabriele Valentini11, L. Czirják12, Ulf Müller-Ladner8, Daniel E. Furst13, A Tyndall3, Marco Matucci-Cerinic7, F De Keyser5, Alberto Sulli9, Carmen Pizzorni9, Britta Maurer10, Stanislaw Sierakowsky14, Otylia Kowal-Bielecka14, P. Coelho, G. Riemekasten15, Simona Rednic16, Ileana Nicoara16, Roberto Caporali, Jiri Stork17, Murat Inanc18, Patricia Carreira19, Srdan Novak, Cecília Varjú12, Carlo Chizzolini20, Camillo Ribi20, Eugeniusz J. Kucharz21, AT Kotulska21, Małgorzata Widuchowska21, Jutta G Richter22, A. Sipek-Dolnicar23, Blaž Rozman23, Armando Gabrielli24, Gianluca Moroncini24, Dominique Farge1, C. Durant1, Hans P. Kiener25, E. Rath25, Paolo Airò, Frank A. Wollheim26, Nicolas Hunzelmann27, Stefano Bombardieri28, A. Della Rossa28, Laura Bazzichi28, Raffaele Pellerito, M. Saracco, Christopher P. Denton29, Madelon C. Vonk, F.H.J. van den Hoogen, Nemanja Damjanov, Ina Kötter30, Stefan Heitmann, Matthias Seidel, Paul Hasler, J.M. van Laar31, Maria João Salvador32, J.A. Pereira da Silva32, Søren Jacobsen33, Margitta Worm15, Annegret Kuhn34, Tatiana Nevskaya35, Evgeny Nasonov35, Raffaella Scorza, Henrik Nielsen, Richard M. Silver, Eric Hachulla, D. Launay, Guido Valesini4, Ruxandra Ionescu36, Daniela Opris36, N. Del Papa, Wanda Maglione, D. Comina, G. Udrea, Coziana Ciurtin, R. Ionitescu, C. Mihai, Cord Sunderkötter34, Jae Bum Jun37, Chris T. Derk38, S. Alhasani, L. Alhajjar, Evelien Ton39, James R. Seibold40, Peter Nash, Luc Mouthon1, C. A. Von Mühlen, Brigitte Krummel-Lorenz, P. Eilbacher, Rene Westhovens41, E. De Langhe41, Miroslav Mayer42, Branimir Anić42, M. Baresic42, F. Stoeckl43, Maria Uprus, S. Popa, M. Buslau, B. Granel, Thierry Zenone, Alessandro Mathieu44, Alessandra Vacca44, Paolo Amerio, T. Tourinho, L. Lonzetti, M. Lemos Lopes, R. E. de Souza45, D. Vealex46, Paola Caramaschi47, Alexandra Balbir-Gurman, Y. Braun, Susanne Ullman33, Magdalena Szmyrka-Kaczmarek48, Ewa Morgiel48, Marie Vanthuyne49, M. Meurer50, P. Rehberger50, Percival D. Sampaio-Barros45 
TL;DR: A core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis were identified in a Delphi exercise among 110 experts in the field of SSc.
Abstract: Objective: To identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc). Methods: A list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores. Results: Physicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily);vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting. Conclusion: The three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.

334 citations

Journal ArticleDOI
TL;DR: The fingers of 75 patients with connective tissue disorders were examined by “wide-field” capillary microscopy and 3 distinct groups were recognized among these patients: increased visibility of nailfold subpapillary plexus in rheumatoid arthritis, massive capillary dilatation in scleroderma-dermatomyositis, and focal loss of capillaries in systemic lupus erythematosus.
Abstract: The fingers of 75 patients with connective tissue disorders were examined by “wide-field” capillary microscopy Four diagnostic groups were included in this study: rheumatoid arthritis—28, scleroderma—22, dermatomyositis—8, and systemic lupus erythematosus—17On the basis of different patterns of elementary microvascular abnormalities and their distribution, 3 distinct groups were recognized among these patients: 1) increased visibility of nailfold subpapillary plexus in rheumatoid arthritis, 2) massive capillary dilatation in scleroderma-dermatomyositis, and 3) focal loss of capillaries and prominence of subpapillary vessels with “punched-out” lesions in systemic lupus erythematosus

308 citations