Diagnosis and treatment of enthesitis-related arthritis

Abstract

Juvenile idiopathic arthritis (JIA) is a chronic, inflammatory disease of unknown etiology. The enthesitis-related arthritis (ERA) JIA category describes a clinically heterogeneous group of children including some who have predominately enthesitis, enthesitis and arthritis, juvenile ankylosing spondylitis, or inflammatory bowel disease-associated arthropathy. ERA accounts for 10%-20% of JIA. Common clinical manifestations of ERA include arthritis, enthesitis, and acute anterior uveitis. Axial disease is also common in children with established ERA. Treatment regimens for ERA, many of them based on adults with rheumatoid arthritis and ankylosing spondylitis, include the use of nonsteroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs, and biologic agents either individually or in combination.

Figure 1

Radiographic findings of spinal and sacroiliac involvement. (A) Axial T2-weighted image of the sacroiliac joints demonstrate fluid within both sacroiliac joints with widening of the left sacroiliac joint. There is bone marrow edema within the sacral ala and adjacent iliac wings (arrows). (B) Sagittal T2-weighted image of the lumbar spine demonstrates triangular-shaped regions of edema along the corners of the vertebral bodies (arrows) consistent with magnetic resonance corner lesions. (C) Axial T1-weighted postcontrast image shows left hip synovitis (black arrow). There is enhancing edema within both greater trochanters and at the hip flexor entheses (white arrows) with mild surrounding soft tissue inflammatory changes. Courtesy of Dr Nancy Chauvin, The Children’s Hospital of Philadelphia, Philadelphia, PA.

Figure 2

Enthesitis-related arthritis-associated acute anterior uveitis. Recurrent HLA-B27-associated anterior uveitis with hypopyon and extensive posterior synechiae. Courtesy of Dr JP Dunn, The Wilmer Eye Institute, The Johns Hopkins School of Medicine, Baltimore, MD.