Localized scleroderma in childhood is not just a skin disease

bjective. Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. Methods. Data from a mult...

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Autores principales: Zulian, Francesco, Vallongo, Cristina, Woo, Patricia, Russo, Ricardo, Ruperto, Nicolino, Harper, John, Espada, Graciela, Corona, Fabrizia, Mukamel, Masha, Vesely, Richard, Musiej Nowakowska, Elzbieta, Chaitow, Jeff
Formato: Artículo
Lenguaje:Español
Publicado: 2005
Materias:
R Medicina (General)
Acceso en línea:http://pa.bibdigital.ucc.edu.ar/3988/1/A_Zulian_Vallengo_Woo.pdf
Aporte de:
Producción Académica Universidad Católica de Córdoba (UCCor) de Universidad Católica de Córdoba
id I38-R144-3988
record_format dspace
spelling I38-R144-39882025-04-14T20:32:25Z http://pa.bibdigital.ucc.edu.ar/3988/ Localized scleroderma in childhood is not just a skin disease Zulian, Francesco Vallongo, Cristina Woo, Patricia Russo, Ricardo Ruperto, Nicolino Harper, John Espada, Graciela Corona, Fabrizia Mukamel, Masha Vesely, Richard Musiej Nowakowska, Elzbieta Chaitow, Jeff R Medicina (General) bjective. Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. Methods. Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement. Results. Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflex. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased. Conclusion. Extracutaneous manifestations of juvenile localized sclerodenna developed in almost one-fourth of the children in this study. These extracotaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully. 2005-12-31 info:eu-repo/semantics/article info:eu-repo/semantics/closedAccess application/pdf spa http://pa.bibdigital.ucc.edu.ar/3988/1/A_Zulian_Vallengo_Woo.pdf Zulian, Francesco, Vallongo, Cristina, Woo, Patricia, Russo, Ricardo, Ruperto, Nicolino, Harper, John, Espada, Graciela, Corona, Fabrizia, Mukamel, Masha, Vesely, Richard, Musiej Nowakowska, Elzbieta and Chaitow, Jeff (2005) Localized scleroderma in childhood is not just a skin disease. Arthritis and Rheumatism, 52 (9). pp. 2873-2881. ISSN 0004-3591 info:eu-repo/semantics/altIdentifier/doi/10.1002/art.21264
institution Universidad Católica de Córdoba
institution_str I-38
repository_str R-144
collection Producción Académica Universidad Católica de Córdoba (UCCor)
language Español
orig_language_str_mv spa
topic R Medicina (General)
spellingShingle R Medicina (General)
Zulian, Francesco
Vallongo, Cristina
Woo, Patricia
Russo, Ricardo
Ruperto, Nicolino
Harper, John
Espada, Graciela
Corona, Fabrizia
Mukamel, Masha
Vesely, Richard
Musiej Nowakowska, Elzbieta
Chaitow, Jeff
Localized scleroderma in childhood is not just a skin disease
topic_facet R Medicina (General)
description bjective. Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. Methods. Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement. Results. Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflex. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased. Conclusion. Extracutaneous manifestations of juvenile localized sclerodenna developed in almost one-fourth of the children in this study. These extracotaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.
format Artículo
author Zulian, Francesco
Vallongo, Cristina
Woo, Patricia
Russo, Ricardo
Ruperto, Nicolino
Harper, John
Espada, Graciela
Corona, Fabrizia
Mukamel, Masha
Vesely, Richard
Musiej Nowakowska, Elzbieta
Chaitow, Jeff
author_facet Zulian, Francesco
Vallongo, Cristina
Woo, Patricia
Russo, Ricardo
Ruperto, Nicolino
Harper, John
Espada, Graciela
Corona, Fabrizia
Mukamel, Masha
Vesely, Richard
Musiej Nowakowska, Elzbieta
Chaitow, Jeff
author_sort Zulian, Francesco
title Localized scleroderma in childhood is not just a skin disease
title_short Localized scleroderma in childhood is not just a skin disease
title_full Localized scleroderma in childhood is not just a skin disease
title_fullStr Localized scleroderma in childhood is not just a skin disease
title_full_unstemmed Localized scleroderma in childhood is not just a skin disease
title_sort localized scleroderma in childhood is not just a skin disease
publishDate 2005
url http://pa.bibdigital.ucc.edu.ar/3988/1/A_Zulian_Vallengo_Woo.pdf
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