Hindawi Publishing Corporation Case Reports in Rheumatology Volume 2013, Article ID 819629, 4 pages http://dx.doi.org/10.1155/2013/819629 Case Report RHUPUS Syndrome in Children: A Case Series and Literature Review Vahid Ziaee, 1,2,3 Mohammad Hassan Moradinejad, 1,3,4 and Reyhaneh Bayat 4 1 Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran 2 Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran 3 Division of Pediatric Rheumatology, Children’s Medical Center, Pediatrics Center of Excellence, Tehran 14194, Iran 4 Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran Correspondence should be addressed to Vahid Ziaee; ziaee@tums.ac.ir Received 24 March 2013; Accepted 21 April 2013 Academic Editors: H. Alexanderson, C. Orellana, and G. J. Tsay Copyright © 2013 Vahid Ziaee et al. his is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Objective. Overlap of juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) is a rare clinical condition in children. his condition has been described as RHUPUS syndrome. Prevalence of this syndrome and 3 cases are reported in this paper. Cases Presentation. During 10 years, 3 patients with SLE had chronic arthritis before or ater diagnosis of SLE. Prevalence of this disorder in JSLE was 2.5%. Two patients were females and one of them was a male. According to our review, mean delay between chronic joint involvement and JSLE diagnosis was 50.1 months. In our case report, two females had joint erosion and one of them died due to heart failure, but in the literature review, just 45% cases had joint erosion and 70% cases were polyarticulare form. Conclusion. RHUPUS is unusual presentation of lupus in children. It seems that clinical feature and outcome of RHUPUS syndrome are diferent in children due to diference between RA and JIA. We suggest juvenile RHUPUS for overlap of JIA and JSLE. 1. Introduction Juvenile idiopathic arthritis (JIA) is the most common type autoimmune rheumatic disease in children. It is a chronic non infectious synovitis that can afect joints in any part of the body. Organs involvement is common in systemic onset type but it decreases ater control of inlammation [1]. Systemic lupus erythematosus (SLE) is the most impor- tant, multisystem, autoimmune rheumatic disease character- ized by both clinical and immunological abnormalities [2]. he peak incidence of childhood SLE occurs around puberty and predominantly involves young women in repro- ductive age, and in 10%–20% of SLE patients, diagnosis is made for the irst time in childhood onset [2]. Arthritis is one of the earliest symptoms in juvenile SLE (JSLE) but it is characteristically short in duration and can be migratory [2]. Although the synovitis of JSLE may be minimally prolifera- tive, it is only occasionally erosive and usually does not result in permanent deformity. JSLE can mimic JIA, especially when it is presented as chronic and erosive arthritis [3]. Overlapping JIA and JSLE is a rare clinical condition in children [4, 5]. RHUPUS syndrome has been deined as an overlap between RA and SLE in adult patients [6]. Although serological overlap between RA and SLE is common in adult patients, clinical overlap between RA and SLE is a rare manifestation of rheumatologic disorders. For the irst time, RHUPUS syndrome was described by Schur in 1971 [7], but irst clinical observation of this syndrome has been described in 1960 by Toone. He identiied this clinical manifestation with this entity [8]. For diagnosis of RHUPUS syndrome, erosive polyarthri- tis and clinical symptoms of SLE as well as positive dsDNA or anti-Sm autoantibodies are necessary [9]. In this paper, we described the presence of three children with RHUPUS syndrome. Finally we reviewed all cases that have been reported in the literature with clinical picture of JIA as irst presentation and eventually turned out to be typical systemic lupus erythemaosus, during follow-up. 2. Case Report During 10 years, 120 patients with systemic lupus erythemato- sus who had been referred to our tertiary pediatric medical