Abstract
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.
Original language | English (US) |
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Pages (from-to) | 927-929 |
Number of pages | 3 |
Journal | Journal of Rheumatology |
Volume | 35 |
Issue number | 5 |
State | Published - May 2008 |
Keywords
- Anti-signal recognition particle autoantibody
- Juvenile polymyositis
ASJC Scopus subject areas
- Immunology and Allergy
- Rheumatology
- Immunology