| Characteristics of patients with juvenile onset systemic sclerosis in an adult single-center cohort. | |
| | |
MedLine Citation:
|
PMID: 20843905 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
|
OBJECTIVE: Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group. METHODS: Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort. RESULTS: We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups. CONCLUSION: The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias. |
| | |
Authors:
|
Ivan Foeldvari; Svetlana I Nihtyanova; Angela Wierk; Christopher P Denton |
Related Documents
:
|
11153815 - Magnitude of sustained multiple risk factors for ischemic heart disease in japanese emp... 963635 - An unusual occurrence of multiple sclerosis in a small rural community. 8782285 - First-episode schizophrenia: ii. premorbid patterns by gender. 17888225 - Fibromyalgia syndrome in young children: onset at age 10 years and younger. 1568935 - Differential age effects for components of the adult auditory middle latency response. 19546415 - Association between late-life social activity and motor decline in older adults. |
Publication Detail:
|
Type: Journal Article Date: 2010-09-15 |
Journal Detail:
|
Title: The Journal of rheumatology Volume: 37 ISSN: 0315-162X ISO Abbreviation: J. Rheumatol. Publication Date: 2010 Nov |
Date Detail:
|
Created Date: 2010-11-02 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 7501984 Medline TA: J Rheumatol Country: Canada |
Other Details:
|
Languages: eng Pagination: 2422-6 Citation Subset: IM |
Affiliation:
|
Hamburger Zentrum für Kinder- und Jugendrheumatologie, Kompetenz-Zentrum für Sklerodermie und autoimmune Uveitis im Kindesalter, Am Klinikum Eilbek, Dehnhaide 120, D-22081 Hamburg, Germany. sprechstunde@kinderrheumatologie.de |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Prospective evaluation of clinical and ultrasound findings in ankle disease in juvenile idiopathic a...
Next Document: High DNA oxidative damage in systemic sclerosis.