A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta; Smith, Vanessa; Cutolo, Maurizio; Foeldvari, Ivan; Airò, Paolo; Sancho, Juan José Alegre; Allanore, Yannick; Ananieva, L.; Ancuta, C.; Andrade, Luís Eduardo Coelho; Aringer, Martin; Bečvář, Radim; Bojincă, Mihai; Butrimienė, İrena; Cantatore, Francesco Paolo; Caporali, Roberto; Caramaschi, Paola; Carreira, Patrícia; Chirieac, Rodica; Corrado, A; Cosentino, Vanesa; Cuomo, Giovanna; Czirják, László; Silva, José António Pereira da; Lefebvre, Paloma García de la Peña; Keyser, Filip De; Müller, Carolina de Souza; Damgaard, Kirsten; Damjanov, Nemanja; Denisov, L N; Distler, Oliver; Doube, A; Dumitrascu, Alina; Engelhart, Merete; Expósito, M Valero; Eyerich, Kilian; Farge‐Bancel, Dominique; Azevedo, Valderílio Feijó; Foti, Rosario; Frerix, Marc; Gabrielli, Armando; Garen, Torhild; Gottschalk, Paola; Groşeanu, L.; Guiducci, Serena; Günther, Claudia; Hachulla, É.; Hein, Rüdiger; Heitmann, Stefan; Henes, Jörg; Hesselstrand, Roger; Highton, John; Iannone, Florenzo; Ionescu, Ruxandra; Kayser, Cristiane; Karpec, Diana; Kerzberg, Eduardo; Kotulska, Anna; Kopeć-Mędrek, Magdalena; Kucharz, Eugene J.; Krummel-Lorenz, Brigitte; Lauffer, Felix; Launay, David; Li, Mengtao; Litinsky, Ira; Loyo, Esthela; Cerinic, Marco Matucci; Meroni, Pier Luigi; Midtvedt, Øyvind; Mihai, Carmen Marina; Montecucco, Carlomaurizio; Montoya, Fabiana; Morgiel, Ewa; Mouthon, Luc; Müller‐Ladner, Ulf; Nielsen, Henrik; Opriş, D.; Ortiz-Santamaría, Vera; Otsa, Kati; Pileckytė, Margarita; Pisarri, Simonetta; Popescu, Monica; Pozzi, Maria Rosa; Puppo, Francesco; Radominski, Sebastião Cézar; Riccieri, Valeria; Rosato, Edoardo; Rozman, B; Rubio, Silvia; Rugienė, Rita; Saar, Petra; Salvador, Maria João; Seidel, Matthias; Sokolik, Renata; Solanki, Kamal; Stamenković, Bojana; Stanković, Aleksandra; Stebbings, Simon; Strauss, Gitte; Sulli, Alberto; Szamosi, Szilvia; Szechiński, Jacek; Szmyrka−Kaczmarek, Magdalena; Szücs, Gabriella; Tănăseanu, Cristina; Tiglea, Isabela; Tyndall, Alan; Ughi, Nicola; Üprus, Maria; Vacca, Alessandra; Varjú, Cecília; Venalis, Algirdas; Venalis, Paulius; Walker, Ulrich A.; Widuchowska, Małgorzata; Wiland, Piotr; Wuttge, Dirk M.; Zeni, S.; Zénone, Thierry

doi:10.1016/j.mvr.2015.07.007

Cited by 16 publications

(5 citation statements)

References 16 publications

(24 reference statements)

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“…Cutaneous changes characteristically evolve in a sequence beginning with oedema, followed by induration and eventually atrophy. Nailfold capillary abnormalities are common, and include capillary dropout, tortuous dilated loops, and occasionally distorted capillary architecture (14, 15). Musculoskeletal symptoms are common in jSSc and characteristically occur at or near onset of the disease in one-third of children (6, 13).…”

Section: Clinical Aspectsmentioning

confidence: 99%

Immunopathogenesis of Juvenile Systemic Sclerosis

Stevens

Torok

et al. 2019

Front. Immunol.

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Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ from women with SSc, resembling instead the genes of adult males with SSc, with additional HLA genes uniquely associated with childhood-onset disease. Current treatments are aimed at inhibiting the inflammatory aspect of disease, but important mechanisms of fibrosis regulated by dermal white adipose tissue dendritic cells may provide an avenue for targeting and potentially reversing the fibrotic stage.

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Section: Clinical Aspectsmentioning

confidence: 99%

Immunopathogenesis of Juvenile Systemic Sclerosis

Stevens

Torok

et al. 2019

Front. Immunol.

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“…A recent article presenting data on capillaroscopy found the characteristic scleroderma (SD) pattern in 93% of 30 juvenile-onset and 88% of 2.108 adult-onset SSc patients from the EUSTAR database; the frequency of active and late SD pattern was similar in the two groups. 23 Many studies have found a significantly lower frequency of ACA in the juvenile group: 7.1% in the multinational cohort, 9 8% in the USA cohort, 10 2% in the UK cohort, 11 and 5% in the EUSTAR cohort. 15 The present study also found a lower frequency of ACA (16%), but it was not statistically significant when compared to the adult-onset (21%) patients.…”

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort

Sampaio-Barros

Bortoluzzo

Rio

et al. 2018

Journal of Scleroderma and Related Disorders

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To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

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“…Data on the capillaroscopic pattern of children with connective tissue disease (CTD) are limited by its low incidence, but adults usually present with a comparable pattern to elderly patients [58]. Children with CTD are in general characterised by lower capillary densities, higher capillary width, abnormal capillaries, and capillary disarrangement [21,59].…”

Section: Connective Tissue Disease In Childrenmentioning

confidence: 99%

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal

Klein-Weigel

Sunderkötter

Sander

2016

Vasa

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Summary: Nailfold capillaroscopy is a method of great diagnostic value in the differential diagnosis of primary versus secondary Raynaud´s phenomenon, of systemic sclerosis versus other so called connective tissue diseases and of additional diagnostic value in other entities. Rheumatologists, dermatologists, and angiologists in Germany have convened in an interdisciplinary working group in which they synergistically combined their expertise to develop a common nomenclature and standards for the technical performance of nailfold capillary microscopy. The article gives an overview of historical and technical aspects of capillaroscopy, morphologic fi ndings, and disease-specifi c patterns. It also provides a critical appraisal of its signifi cance in the diagnosis and sequelae of these interdisciplinarily-managed diseases including its performance in children and gives an excursion in the potential perspectives of capillaroscopy in less common indications.

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A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Cited by 16 publications

References 16 publications

Immunopathogenesis of Juvenile Systemic Sclerosis

Immunopathogenesis of Juvenile Systemic Sclerosis

Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal

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