Autoantibody to signal recognition particle in African American girls with juvenile polymyositis

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 requ...

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Bibliographic Details
Published inJournal of rheumatology Vol. 35; no. 5; p. 927
Main Authors Rouster-Stevens, Kelly A, Pachman, Lauren M
Format Journal Article
LanguageEnglish
Published Canada 01.05.2008
Subjects
Adolescent
African Americans - ethnology
Autoantibodies - blood
Autoantibodies - immunology
Child
Female
Humans
Immunosuppressive Agents - therapeutic use
Polymyositis - diagnosis
Polymyositis - ethnology
Polymyositis - immunology
Prognosis
Signal Recognition Particle - immunology
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Summary: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.
ISSN:0315-162X