The Effectiveness of Tools for Monitoring Hemophilic Arthropathy Yesim Oymak, MD,* Aysen T. Yildirim, MD,* Yontem Yaman, MD,* Muge Gurcinar, MD,w Altinay Firat, MD,z Duygu Cubuckcu, MD,z Ozgur Carti, MD,* Gulcihan Ozek, MD,* and Raziye C. Vergin, MD* Introduction: Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health. Aim: The purpose of this study was to determine the correlation between these tools when used to assess hemophilia patients. Methods: This cross-sectional study enrolled 38 hemophilia patients between 2 and 18 years of age. Hemophilia Joint Health Score (HJHS) and radiologic scores (Pettersson and Arnold-Hilgartner) were used to evaluate the joints of the patients (n = 236). Magnetic resonance imaging (MRI) was performed on 46 joints that were pathologic according to the HJHS. These joints were imaged bilaterally; therefore, 14 normal joints were imaged. In addition, the Functional Independence Score in Hemophilia (FISH) was used to evaluate the joints of 33 patients. Results: The HJHS scores were correlated with the MRI and FISH scores. The annual bleeding rate was not correlated with any scores; however, the number of painful joints was correlated with the MRI scores. The radiologic scores were correlated weakly with progressive score and HJHS. Conclusion: The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophilia patients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful. Key Words: hemophilia, FISH, HJHS, compatible scale, Pettersson, Arnold-Hilgartner (J Pediatr Hematol Oncol 2015;37:e80–e85) H emophilia is an X-linked recessive disorder that results from coagulation factor VIII and factor IX deficien- cies. This disease causes morbidity that is associated with bleeding into the muscles and joints. Recurrent bleeding may cause permanent joint damage. 1 Although 80% of bleeding occurs in the knees, elbows, and ankles, other joints can be affected. 2 Prophylactic factor replacement treatments are considerably effective in reducing the frequency and severity of bleeding and protecting joints in hemophilia patients. 3,4 Clinical and radiologic scorings are essential to assess the condition of joints, to monitor the progression of arthropathy, and to determine the effec- tiveness of therapeutic interventions. The Hemophilia Joint Health Score (HJHS) is used as the first-line assessment of joints in hemophilia patients and is recognized by the World Federation of Hemophilia. 5 Arnold-Hilgartner (AH) and Pettersson scores have been used in radiography for >30 years. 6,7 Compared with radiography, MRI is a more accurate and sensitive tool, which can display alterations in soft tissue, such as synovia, and osteochondral units. The scoring system that is used in MRI is called the compatible MRI scale and is based on radiography scoring systems. 8 The Functional Independ- ence Score in Hemophilia (FISH) is a self-evaluation tool that measures constraints in everyday life. 9 All the scales were found to be reliable. Knowing the correlation among them and using some of them together enabled us to maintain optimal hemophilic arthropathy (HA) management. The aim of this study was to determine the correlations between the HJHS score, the FISH, radi- ography scores (AH and Pettersson), and MRI scores. METHODS This cross-sectional study was conducted from July 1, 2010 to October 31, 2010 at Dr Behcet Uz Children’s Hospital. The study included 38 patients (32 hemophilia A, 6 hemophilia B) with a median age of 9 (range, 2.0 to 18.0) years, who had been diagnosed and followed up at our center. Of the patients, 22 (57.8%) were on secondary prophylaxis with a median duration of 3 (1 to 8) months, and 16 (42.2%) were treated with on-demand therapy during the time period of the study. Factor VIII and IX levels that ranged from 1% to 5% were classified as mod- erate hemophilia, whereas factor VIII and IX levels <1% were classified as severe hemophilia. The knee, elbow, ankle, and target joints represented a total of 236 joints in 38 (26 severe and 12 moderate) hemophilia patients. For each patient, the evaluations were performed when no bleeding had occurred during the prior month. The number of bleeds into the joints over the last year was defined as an Received for publication February 17, 2014; accepted May 30, 2014. From the *Clinic of Hematology; zClinic of Physical Medicine and Rehabilitation, Dr Behcet Uz Children’s Hospital; and wAlsancak Bilrad Magnetic Resonance Imaging Center, Izmir, Turkey. The diagnostic tools that were used for the patients were paid for by the health insurance that was provided by the state. Y.O. organized and performed the study, and wrote the manuscript. A.T.Y. calculated the results using SPSS. M.G. applied the radio- logic (Pettersson and Arnold-Hilgartner) scores and the MRI scores. Y.Y. applied the functional independence scores. A.F. and D.C. applied the World Federation of Hemophilia Physical Examination scores. O.C. and G.O. provided information about the study to the parents of the patients and obtained their informed consent. R.C.V. performed a final review of the written manuscript. The authors declare no conflicts of interest. Reprints: Yesim Oymak, MD, Clinic of Hematology, Dr Behcet Uz Children’s Hospital, Izmir 35210, Turkey (e-mail: yesimoymak@ hotmail.com). Copyright r 2014 Wolters Kluwer Health, Inc. All rights reserved. ORIGINAL ARTICLE e80 | www.jpho-online.com J Pediatr Hematol Oncol  Volume 37, Number 2, March 2015