title: 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.affiliation: Wake Forest University School of Medicine, Department of Pediatrics/Pediatric Rheumatology, Winston-Salem, North Carolina 27157, USA. krouster@wfubmc.edu
authors.forename: Kelly A
authors.initials: KA
authors.lastname: Rouster-Stevens
authors.forename: Lauren M
authors.initials: LM
authors.lastname: Pachman
mesh_terms: D000293:Adolescent; D001741:Black or African American / Q000208:ethnology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D002648:Child; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017285:Polymyositis / Q000175:diagnosis* / Q000208:ethnology / Q000276:immunology*; D011379:Prognosis; D018271:Signal Recognition Particle / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; D018271:Signal Recognition Particle