RT Journal Article
SR Electronic
T1 Autoantibody to signal recognition particle in African American girls with juvenile polymyositis.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 927
OP 929
VO 35
IS 5
A1 Kelly A Rouster-Stevens
A1 Lauren M Pachman
YR 2008
UL http://www.jrheum.org/content/35/5/927.abstract
AB Anti-signal recognition particle (anti-SRP) is a myositis-specific autoantibody that is linked to a severe polymyositis (PM) associated with interstitial lung disease (ILD) and esophageal dysmotility in adults. We describe 3 African American adolescent girls with anti-SRP juvenile PM. One child required aggressive treatment to control her disease and 2 were refractory to multiple immunosuppressants. Patient 1 developed ILD and cardiac disease; Patient 2 developed ILD; Patient 3 developed esophageal dysmotility and cardiac disease. Organ system involvement was comparable to that seen in adults. We conclude that testing for anti-SRP in children with PM may facilitate diagnosis and management.