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Case Report
Autoantibody to Signal Recognition Particle in African American Girls with Juvenile Polymyositis
KELLY A. ROUSTER-STEVENS and LAUREN M. PACHMAN 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. (First Release Mar 1 2008; J Rheumatol 2008;35:927–9) Key Indexing Terms:
JUVENILE POLYMYOSITIS From Wake Forest University School of Medicine, Department of Pediatrics/Pediatric Rheumatology, Winston-Salem, North Carolina; and Feinberg School of Medicine, Northwestern University, Division of Pediatric Rheumatology, Chicago, Illinois, USA. Supported by Cure Juvenile Myositis Foundation. K.A. Rouster-Stevens, MD, Wake Forest University School of Medicine, Department of Pediatrics/Pediatric Rheumatology; L.M. Pachman, MD, Feinberg School of Medicine, Northwestern University, Division of Pediatric Rheumatology. Address reprint requests to Dr. K.A. Rouster-Stevens, WFUBMC Department of Pediatrics, One Medical Center Blvd., Winston-Salem, NC 27157. E-mail: krouster@wfubmc.edu Accepted for publication December 1, 2007. |
