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Rheumatology 2001; 40: 1279-1284
© 2001 British Society for Rheumatology


Paediatric Rheumatology

Persistent maternally derived peripheral microchimerism is associated with the juvenile idiopathic inflammatory myopathies

Paediatric Rheumatology/Series Editor: P. Woo

C. M. Artlett, F. W. Miller1,2, L. G. Rider1,2 and for the Childhood Myositis Heterogeneity Collaborative Study Group ,*

Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107,
1 Division of Monoclonal Antibodies, Center for Biologics Evaluation and Research, Food and Drug Administration, Bethesda, MD and
2 Arthritis and Rheumatism Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA

Abstract

Objective. Fetal cells have been demonstrated in the active lesions of adult women with systemic sclerosis. Because the juvenile idiopathic inflammatory myopathies (JIIM) share clinical and histopathological features with systemic sclerosis and graft-vs-host disease, we explored the possibility that maternal cells persist and play a role in the pathogenesis of JIIM.

Methods. DNA samples extracted from peripheral blood of 28 JIIM patients (14 females, 14 males) and 23 healthy controls were assessed for microchimerism by the HLA Cw polymerase chain reaction method. HLA Cw alleles from eight mothers and three healthy siblings of JIIM patients were also examined.

Results. A microchimeric allele was identified in 19 of 26 JIIM patients whose data were able to be interpreted, compared with two of 21 healthy controls (P<0.001). Subjects with microchimerism ranged in age from 4 to 28 yr. In eight cases in which maternal peripheral blood was available, the additional Cw allele present in the patients was confirmed to be identical to a maternal allele. Three healthy siblings of JIIM patients did not have evidence of a microchimeric Cw allele.

Conclusion. Maternal cells can persist in the peripheral blood of their children up to three decades after birth, and are found in a higher proportion in JIIM patients compared with controls. These findings, with other data, suggest that maternal cells may be involved in the immunopathogenesis of JIIM.

KEY WORDS: Microchimerism, Maternal–fetal exchange, Dermatomyositis, Polymyositis, Graft versus host reaction, Inflammatory myopathy.

Notes

Correspondence to: C. M. Artlett, Division of Rheumatology, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA.

* A complete list of members is given in the Appendix.


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