Abstract | OBJECTIVES: C4 is encoded by 2 distinct but closely linked loci within the major histocompatibility complex locus on human chromosome 6. C4A deficiencies have been associated with autoimmune disease and C4B with increased frequency of infection. C4 deficiencies have rarely been associated with juvenile idiopathic arthritis (JIA). Our aim was to investigate the prevalence of deficiencies in C4 allotypes in JIA patients. METHODS: RESULTS: CONCLUSIONS: Defects in the complement system have been implicated in the pathogenesis of CTD. However, the specific role of C4 in JIA is not clear. We demonstrate partial C4 deficiencies in 5 JIA patients. Our findings suggest an association between C4 deficiency and another CTD, JIA, as well as with disease severity and recurrent infections.
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Authors | Brooke E Gilliam, Anne E Wolff, Terry L Moore |
Journal | Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
(J Clin Rheumatol)
Vol. 13
Issue 5
Pg. 256-60
(Oct 2007)
ISSN: 1076-1608 [Print] United States |
PMID | 17921792
(Publication Type: Journal Article)
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Chemical References |
- Complement C4a
- Complement C4b
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Topics |
- Adolescent
- Adult
- Arthritis, Juvenile
(genetics, physiopathology)
- Child
- Cohort Studies
- Complement C4a
(deficiency, genetics, immunology)
- Complement C4b
(deficiency, genetics, immunology)
- Female
- Haplotypes
- Humans
- Male
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