Anti-centromere autoantibody in a patient evolving from a lupus/Sjögren's overlap to the CREST variant of scleroderma.
We characterized the development of the anti-centromere antibody in a patient prior to the development of CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) symptoms. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis followed by immunoblotting (IgG and IgM) of cellular extracts enriched for centromere antigens and indirect immunofluorescence were used to study the anti-centromere immune response. The sera recognized 3 centromere antigens with molecular masses 18,000 (CENP-A), 50,000 (CENP-D), and 80,000 (CENP-B). For CENP-A, IgM was present before the appearance of the IgG response. Anti-CENP-D revealed an IgM response that decreased over time but no IgG, while CENP-B showed an IgG response that strengthened and then weakened over time. The appearance of an anti-centromere nuclear fluorescence pattern correlated with the appearance of IgG anti-CENP-A. Signs and symptoms typical of CREST began about 4 years after antibodies to centromere antigens were found. The development of the CREST syndrome in our patient was preceded by the appearance of anti-centromere autoantibodies. For at least one of the antigens (CENP-A), there was an immunoglobulin class switch from IgM to IgG.
Department of Medicine, University of Oklahoma Health Science Center, Department of Veterans Affairs Medical Center, Oklahoma City, USA.
A L
AL
Ford
B T
BT
Kurien
J B
JB
Harley
R H
RH
Scofield
D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D017675:CREST Syndrome / Q000150:complications / Q000276:immunology*; D002503:Centromere / Q000276:immunology*; D005260:Female; D006801:Humans; D015151:Immunoblotting; D008177:Lupus Vulgaris / Q000150:complications / Q000276:immunology*; D008856:Microscopy, Fluorescence; D012859:Sjogren's Syndrome / Q000150:complications / Q000276:immunology*
D001323:Autoantibodies