Document Detail


Serum autoantibodies and their clinical associations in patients with childhood- and adult-onset linear scleroderma. A single-center study.
MedLine Citation:
PMID:  19004036     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To determine the frequency of selected serum autoantibodies and their clinical associations in patients with childhood-onset (ChO) or adult-onset (AO) linear scleroderma (LiScl) evaluated at a single institution. METHODS: Seventy-two patients (ChO = 40, AO = 32), including 12 with en coup de sabre, were studied. All ChO patients had disease onset before age 16 years. Clinical features (extent of cutaneous disease, activity, and joint contractures) were recorded. Antinuclear antibodies (ANA) were identified by indirect immunofluorescence (HEp-2 cells), and anti-single-stranded DNA (anti-ssDNA), antihistone (AHA), and antichromatin (AChA) autoantibodies were detected by ELISA. RESULTS: There were no significant differences between groups in regard to gender, proportion with LiScl/E, or clinical features except joint contractures (ChO > AO; p = 0.04). There were no differences in the frequency of ANA or other autoantibodies between the groups except for AHA (ChO > AO). AHA was more frequently found with anti-ssDNA (p < 0.0001). LiScl patients with positive anti-ssDNA and/or AHA had more extensive cutaneous involvement and more often had joint contractures (p < 0.05). Anti-ssDNA was present more frequently in AO than in ChO patients with active lesions (p = 0.04). ANA and AChA were not associated with any clinical features. Both AHA and anti-ssDNA levels showed good correlation with disease severity. CONCLUSION: Over two-thirds of LiScl patients had ANA. Patients with ChO were similar to those with AO with regard to the frequency of selected serum autoantibodies. Anti-ssDNA and AHA were frequently found together and both were associated with more extensive skin disease with joint contractures. LiScl disease severity correlated with the serum levels of both these antibodies.
Authors:
Thaschawee Arkachaisri; Noreen Fertig; Sally Pino; Thomas A Medsger
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-11-01
Journal Detail:
Title:  The Journal of rheumatology     Volume:  35     ISSN:  0315-162X     ISO Abbreviation:  J. Rheumatol.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-12-01     Completed Date:  2009-03-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7501984     Medline TA:  J Rheumatol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  2439-44     Citation Subset:  IM    
Affiliation:
Division of Rheumatology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania 15213, USA. arkatx@chp.edu
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Autoantibodies / analysis,  immunology*
Child
Child, Preschool
Contracture / immunology
Female
Humans
Male
Middle Aged
Scleroderma, Localized / immunology*
Severity of Illness Index
Young Adult
Chemical
Reg. No./Substance:
0/Autoantibodies

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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