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

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.

• Authors: Kelly A Rouster-Stevens, Lauren M Pachman
• Journal: The Journal of rheumatology (J Rheumatol) Vol. 35 Issue 5 Pg. 927-9 (May 2008) ISSN: 0315-162X [Print] Canada
• PMID: 18322984 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Immunosuppressive Agents
• Signal Recognition Particle

Topics

• Adolescent
• Black or African American (ethnology)
• Autoantibodies (blood, immunology)
• Child
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Polymyositis (diagnosis, ethnology, immunology)
• Prognosis
• Signal Recognition Particle (immunology)