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. (J Rheumatol First Release Mar 1 2008)

Key Indexing Terms:

JUVENILE POLYMYOSITIS
ANTI-SIGNAL RECOGNITION PARTICLE AUTOANTIBODY

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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.