Objective. Overlap of juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) is a rare clinical condition in children. This 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 after 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 different in children due to difference 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 affect joints in any part of the body. Organs involvement is common in systemic onset type but it decreases after control of inflammation [1]. Systemic lupus erythematosus (SLE) is the most important, multisystem, autoimmune rheumatic disease characterized by both clinical and immunological abnormalities [2]. The peak incidence of childhood SLE occurs around puberty and predominantly involves young women in reproductive age, and in 10%–20% of SLE patients, diagnosis is made for the first 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 proliferative, 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 defined 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 first time, RHUPUS syndrome was described by Schur in 1971 [7], but first clinical observation of this syndrome has been described in 1960 by Toone. He identified
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