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Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis
Arthritis & Rheumatism, 06/05/09

Gunawardena H et al. – Study demonstrates that anti-p140 autoantibodies represent a major autoantibody subset in juvenile dermatomyositis (DM). This specificity may identify a further immunogenetic and clinical phenotype within the juvenile myositis spectrum that includes an association with calcinosis.

• Aim was to describe autoantibodies to a 140-kd protein in children with Juvenile DM
• Clinical data and sera were collected; sera that recognized a 140-kd protein by IP were identified
• Identity of the p140 autoantigen was investigated by IP/immunodepletion, using Abs to NXP-2, reference anti-p140, and anti-p155/140, the other autoantibody recently described in juvenile DM
• DNA samples from 100 Caucasian children with myositis were genotyped for HLA class II haplotype associations and compared with 864 control subjects

• Sera from 37/162 (23%) pts with juvenile myositis were positive for anti-p140 autoantibodies
• These were detected exclusively in pts with juvenile DM vs juvenile DM-overlap syndrome or control subjects
• No anti-p140 antibody-positive pts were positive for other recognized autoantibodies
• Immunodepletion suggested that the identity of p140 was consistent with NXP-2
• In children with anti-p140 Abs, the association with calcinosis was compared with the rest of the cohort
• The clinical features of pts with anti-p140 autoantibodies were different from those of children with anti-p155/140 autoantibodies
• Presence of HLA-DRB1*08 was a possible risk factor for anti-p140 autoantibody positivity

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