ARTHRITIS & RHEUMATISM Vol. 56, No. 9, September 2007, pp 3087–3095 DOI 10.1002/art.22835 © 2007, American College of Rheumatology Adapted Versions of the Sharp/van der Heijde Score Are Reliable and Valid for Assessment of Radiographic Progression in Juvenile Idiopathic Arthritis Angelo Ravelli, 1 Maka Ioseliani, 2 Ximena Norambuena, 2 Juliana Sato, 2 Angela Pistorio, 2 Federica Rossi, 2 Nicolino Ruperto, 2 Silvia Magni-Manzoni, 3 Nicola Ullmann, 2 and Alberto Martini 1 Objective. To develop adapted versions of the Sharp/van der Heijde radiographic scoring system for use in juvenile idiopathic arthritis (JIA), and to inves- tigate their validity in JIA patients with polyarticular disease. Methods. The study group comprised 177 patients with polyarticular JIA. Radiographs of the wrist/hand of each patient were obtained at baseline (first observa- tion) and then at 1, 3, 5, 7/8, and 10 years and were assessed independently by 2 pediatric rheumatologists according to different adaptations of the Sharp/van der Heijde method. To facilitate score assignment, the ra- diograph for each patient was compared with a bone age–related standard. Validation procedures included analysis of reliability, construct validity, and score progression over time. Results. Interobserver and intraobserver agree- ment on longitudinal score values and score changes was good for all of the adapted scoring versions (intra- class correlation coefficient >0.85). Score changes over time were moderately to strongly correlated with the clinical indicators of long-term joint damage and with the amount of long-term radiographic damage as mea- sured with the carpo:metacarpal ratio, thereby demon- strating good construct validity. A steady increase in scores over time was observed, with joint space narrow- ing being the most common form of damage throughout the disease course. The inclusion of 5 new areas ap- peared to increase the overall construct validity of erosion scores. Conclusion. Our results show that the adapted versions of the Sharp/van der Heijde score are reliable and valid for the assessment of radiographic progres- sion in patients with JIA. Juvenile idiopathic arthritis (JIA) is a chronic and heterogeneous disease characterized by prolonged syno- vial inflammation that may lead to destructive lesions of joint structures (1). Because the prevention or retarda- tion of joint changes is a major objective of treatment of chronic arthritis, evaluation of radiographic joint dam- age has become an important tool for assessing disease severity and progression in patients with JIA. The assessment of structural joint damage is considered the gold standard of treatment efficacy studies in patients with chronic arthritis (2) and is now required by the US Food and Drug Administration to be used as a measure of disease progression in clinical trials of potential disease-modifying drugs (3). The evaluation of radio- graphic progression has never been included in con- trolled trials in JIA, reflecting primarily the paucity of established radiographic scoring systems for use in the pediatric age group. However, because new potent ther- apeutic agents are now available for children with JIA (4), there is a growing need for a reliable radiographic assessment standard to investigate thoroughly the effec- tiveness of these new agents. Dr. Ioseliani is recipient of a bursary from the European League Against Rheumatism. Drs. Norambuena and Sato are recipi- ents of an Alpha Scholarship from the European Union (contract AML/B7-311/970666/II-0246-FI). 1 Angelo Ravelli, MD, Alberto Martini, MD: Universita ` degli Studi di Genoa, and IRCCS, Istituto G. Gaslini, Genoa, Italy; 2 Maka Ioseliani, MD, Ximena Norambuena, MD, Juliana Sato, MD, Angela Pistorio, MD, PhD, Federica Rossi, MD, Nicolino Ruperto, MD, MPH, Nicola Ullmann, MD: IRCCS, Istituto G. Gaslini, Genoa, Italy; 3 Silvia Magni-Manzoni, MD: IRCCS, Policlinico S. Matteo, Pavia, Italy. Drs. Ioseliani and Norambuena contributed equally to this work. Address correspondence and reprint requests to Angelo Ravelli, MD, Pediatria II, Istituto G. Gaslini, Largo G. Gaslini 5, 16147 Genoa, Italy. E-mail: angeloravelli@ospedale-gaslini.ge.it. Submitted for publication December 29, 2006; accepted in revised form May 16, 2007. 3087