RHUPUS Syndrome in Children: A Case Series and Literature Review

Abstract

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.

Characterization of chronic arthritis in a multicenter study of 852 childhood-onset systemic lupus erythematosus patients

“…Arthritis features were also evaluated: duration, number and type of joints with arthritis, number and type of joints with limitation in range of motion and deformity, and radiographic changes (juxta-articular osteopenia, joint-space narrowing and erosion). Rhupus was characterized as the association of cSLE and chronic inflammatory arthritis with erosive damage with or without positive rheumatoid factor [3,5,6,8]. Jaccoud's arthropathy was diagnosed as a nonerosive arthropathy with marked articular dislocation or subluxation leading to severe deformity of the hands and feet.…”

“…The comparison of last visit of patients On the other hand, chronic arthritis (CA) is an unusual condition in aSLE [4,8] and in cSLE [3,6]. Data in children has been limited mostly to case reports [3,[5][6][7] and to a large single center study comparing adults and children with CA focusing solely on distinct features in these two age brackets [8]. There are, however, no data regarding the possible clinical associations of CA in cSLE patients with and without this condition.…”

Rhupus syndrome: a case report

“…Overlap of RA and SLE was primarily named "rhupus syndrome" in 1974 by Schur (2,10). Rhupus syndrome is generally diagnosed based on complaints such as chronic symmetric polyarthritis, butterfly-like rash, alopecia, mucosal involvement, serositis, myositis, and hematologic findings of SLE (4). The skin biopsy is not diagnostic in rhupus syndrome, but it may be necessary for differential diagnosis of other erythematous diseases such as rosacea and allergic contact dermatitis.…”

“…It generally starts with joint involvement, and its most frequently observed symptoms are arthritis that does not lead to deformity and myalgia. Proximal interphalangeal and metacarpophalangeal joints are primarily involved (3,4).…”

An uncommon overlap of two common rheumatological disorders

“…2 KD is also the commonest aetiology of acquired heart disease in children in the developed world including Japan, the United States (US) and Europe. 3 While overlap of childhood SLE with other rheumatological disorders (such as scleroderma and juvenile idiopathic arthritis) has been described, 4–6 it is extremely unusual in the context of KD. 7–9 We report two children who had such an overlap.…”