Systemic sclerosis in childhood: Clinical and immunologic features of 153 patients in an international database

Giorgia Martini, Ivan Foeldvari, Ricardo Russo, Ruben Cuttica, Anne Eberhard, Angelo Ravelli, Thomas J A Lehman, Sheila Knupp Feitosa De Oliveira, Gordana Susic, Galina Lyskina, Dana Nemcova, Robert Sundel, Fernanda Falcini, Herman Girschick, Ana Paula Lotito, Antonella Buoncompagni, Flavio Sztajnbok, Sulaiman M. Al-Mayouf, Ilonka Orbàn, Clodoveo FerriBalu H. Athreya, Patricia Woo, Francesco Zulian

Research output: Contribution to journalArticle

Abstract

Objective. To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group of children and describe the clinical evolution of the disease and compare it with the adult form. Methods. Data on 153 patients with juvenile SSc collected from 55 pediatric rheumatology centers in Europe, Asia, and South and North America were analyzed. Demographic, clinical, and immunologic characteristics of children with juvenile SSc at the onset, at diagnosis, and during the disease course were evaluated. Results. Raynaud's phenomenon was the most frequent symptom, followed by skin induration in ∼75% of patients. Musculoskeletal symptoms were present in one-third of patients, and the most frequently involved internal organs were respiratory and gastrointestinal, while involvement of renal, cerebral, and cardiovascular systems was extremely rare. Antinuclear antibodies were present in the sera of 81% of patients. Anti-topoisomerase I (Scl-70) and anticentromere antibodies were found to be positive in 34% and 7.1% of patients, respectively. Involvement of the respiratory, gastrointestinal, and cardiovascular systems was more frequent and occurred earlier in patients who died than in those who survived. Compared with the adult form, juvenile SSc appears to be less severe, with the involvement of fewer internal organs, particularly at the time of diagnosis, and has a less characterized immunologic profile. Conclusion. This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome.

Original languageEnglish
Pages (from-to)3971-3978
Number of pages8
JournalArthritis and Rheumatism
Volume54
Issue number12
DOIs
Publication statusPublished - Dec 2006

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Systemic Scleroderma
Databases
Cardiovascular System
Type I DNA Topoisomerase
Raynaud Disease
South America
Antinuclear Antibodies
Rheumatology
North America
Respiratory System
Autoantibodies
Demography
Pediatrics
Kidney
Skin
Serum

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

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Systemic sclerosis in childhood : Clinical and immunologic features of 153 patients in an international database. / Martini, Giorgia; Foeldvari, Ivan; Russo, Ricardo; Cuttica, Ruben; Eberhard, Anne; Ravelli, Angelo; Lehman, Thomas J A; De Oliveira, Sheila Knupp Feitosa; Susic, Gordana; Lyskina, Galina; Nemcova, Dana; Sundel, Robert; Falcini, Fernanda; Girschick, Herman; Lotito, Ana Paula; Buoncompagni, Antonella; Sztajnbok, Flavio; Al-Mayouf, Sulaiman M.; Orbàn, Ilonka; Ferri, Clodoveo; Athreya, Balu H.; Woo, Patricia; Zulian, Francesco.

In: Arthritis and Rheumatism, Vol. 54, No. 12, 12.2006, p. 3971-3978.

Research output: Contribution to journalArticle

Martini, G, Foeldvari, I, Russo, R, Cuttica, R, Eberhard, A, Ravelli, A, Lehman, TJA, De Oliveira, SKF, Susic, G, Lyskina, G, Nemcova, D, Sundel, R, Falcini, F, Girschick, H, Lotito, AP, Buoncompagni, A, Sztajnbok, F, Al-Mayouf, SM, Orbàn, I, Ferri, C, Athreya, BH, Woo, P & Zulian, F 2006, 'Systemic sclerosis in childhood: Clinical and immunologic features of 153 patients in an international database', Arthritis and Rheumatism, vol. 54, no. 12, pp. 3971-3978. https://doi.org/10.1002/art.22207
Martini, Giorgia ; Foeldvari, Ivan ; Russo, Ricardo ; Cuttica, Ruben ; Eberhard, Anne ; Ravelli, Angelo ; Lehman, Thomas J A ; De Oliveira, Sheila Knupp Feitosa ; Susic, Gordana ; Lyskina, Galina ; Nemcova, Dana ; Sundel, Robert ; Falcini, Fernanda ; Girschick, Herman ; Lotito, Ana Paula ; Buoncompagni, Antonella ; Sztajnbok, Flavio ; Al-Mayouf, Sulaiman M. ; Orbàn, Ilonka ; Ferri, Clodoveo ; Athreya, Balu H. ; Woo, Patricia ; Zulian, Francesco. / Systemic sclerosis in childhood : Clinical and immunologic features of 153 patients in an international database. In: Arthritis and Rheumatism. 2006 ; Vol. 54, No. 12. pp. 3971-3978.
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AU - Martini, Giorgia

AU - Foeldvari, Ivan

AU - Russo, Ricardo

AU - Cuttica, Ruben

AU - Eberhard, Anne

AU - Ravelli, Angelo

AU - Lehman, Thomas J A

AU - De Oliveira, Sheila Knupp Feitosa

AU - Susic, Gordana

AU - Lyskina, Galina

AU - Nemcova, Dana

AU - Sundel, Robert

AU - Falcini, Fernanda

AU - Girschick, Herman

AU - Lotito, Ana Paula

AU - Buoncompagni, Antonella

AU - Sztajnbok, Flavio

AU - Al-Mayouf, Sulaiman M.

AU - Orbàn, Ilonka

AU - Ferri, Clodoveo

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AU - Woo, Patricia

AU - Zulian, Francesco

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N2 - Objective. To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group of children and describe the clinical evolution of the disease and compare it with the adult form. Methods. Data on 153 patients with juvenile SSc collected from 55 pediatric rheumatology centers in Europe, Asia, and South and North America were analyzed. Demographic, clinical, and immunologic characteristics of children with juvenile SSc at the onset, at diagnosis, and during the disease course were evaluated. Results. Raynaud's phenomenon was the most frequent symptom, followed by skin induration in ∼75% of patients. Musculoskeletal symptoms were present in one-third of patients, and the most frequently involved internal organs were respiratory and gastrointestinal, while involvement of renal, cerebral, and cardiovascular systems was extremely rare. Antinuclear antibodies were present in the sera of 81% of patients. Anti-topoisomerase I (Scl-70) and anticentromere antibodies were found to be positive in 34% and 7.1% of patients, respectively. Involvement of the respiratory, gastrointestinal, and cardiovascular systems was more frequent and occurred earlier in patients who died than in those who survived. Compared with the adult form, juvenile SSc appears to be less severe, with the involvement of fewer internal organs, particularly at the time of diagnosis, and has a less characterized immunologic profile. Conclusion. This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome.

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