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Chronic polyarthritis as the first manifestation of juvenile systemic lupus erythematosus patients
E G N Cavalcante1, N E Aikawa, R G Lozano
1Pediatric Rheumatology Unit, Children's Hospital, São Paulo, Brazil.
Insights
Chronic polyarthritis is a rare manifestation in juvenile systemic lupus erythematosus (JSLE). This study highlights the "rhupus" overlap, a distinct JSLE subgroup with poor response to NSAIDs alone.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Systemic Autoimmune Diseases
Background:
- Juvenile Systemic Lupus Erythematosus (JSLE) is a complex autoimmune condition affecting children.
- Chronic polyarthritis is an uncommon but significant manifestation in JSLE patients.
- Understanding JSLE's diverse clinical presentations is crucial for effective management.
Purpose of the Study:
- To determine the prevalence of chronic polyarthritis in juvenile systemic lupus erythematosus (JSLE).
- To characterize the clinical features, treatment strategies, and outcomes of JSLE patients with chronic polyarthritis.
- To investigate the 'rhupus' overlap syndrome in the context of JSLE.
Main Methods:
- Retrospective analysis of 5419 patients from a Pediatric Rheumatology Unit (1983-2010).
- Identified 271 patients with JSLE (ACR criteria) and evaluated for chronic polyarthritis.
- Defined 'rhupus' as overlap between juvenile idiopathic arthritis (ILAR criteria) and JSLE; assessed clinical, laboratory, and radiographic data.
Main Results:
- Chronic polyarthritis occurred in 2.6% (7/271) of JSLE patients, presenting initially in all cases.
- Three patients exhibited 'rhupus' with chronic polyarthritis, rheumatoid factor, autoantibodies, and radiographic abnormalities.
- Treatment involved NSAIDs, prednisone, antimalarials; three 'rhupus' patients required immunosuppressants due to non-responsiveness.
Conclusions:
- Chronic polyarthritis is a rare but distinct manifestation in pediatric lupus.
- 'Rhupus' in JSLE may represent a unique clinical subgroup with a poorer prognosis and limited response to NSAIDs.
- Further research into potential genetic factors for this JSLE subtype is warranted.
Objective:
To evaluate the prevalence of chronic polyarthritis in juvenile systemic lupus erythematosus (JSLE) and to describe the manifestations, treatments, and outcomes in these patients.
Methods:
From January 1983 to July 2010, 5419 patients were followed up at the Pediatric Rheumatology Unit of the University Hospital and 271 (5%) of them had JSLE (American College of Rheumatology [ACR] criteria). 'Rhupus' was classified as the overlap of juvenile idiopathic arthritis (International League of Associations for Rheumatology [ILAR] criteria) and JSLE. We evaluated demographic data, polyarthritis and other clinical manifestations, disease activity and damage, laboratory exams, radiographic findings, treatments, and outcomes.
Results:
The prevalence of chronic polyarthritis in this JSLE population was 2.6% (7/271). This articular involvement was the initial manifestation in all seven JSLE patients. The median duration of chronic polyarthritis was 11 months (range 2-15 months). Interestingly, rhupus with chronic polyarthritis and limitation of movement, presence of rheumatoid factor, autoantibodies, and/or radiographic abnormalities (juxtaarticular osteopenia, joint-space narrowing, or erosions) was evidenced in three patients. No patient had deformities of hands and feet associated with Jaccoud's arthropathy or osteonecrosis. All patients were treated with nonsteroidal anti-inflammatory drugs (NSAIDs, naproxen 10-15 mg/kg/day) when polyarthritis diagnosis was established. Prednisone and antimalarials were administered at JSLE diagnosis. The three non-responsive rhupus patients were treated in conjunction with immunosuppressive drugs (methotrexate, azathioprine, and/or cyclosporine).
Conclusions:
Chronic polyarthritis was a rare lupus manifestation in active pediatric patients. The interesting overlap between chronic arthritis and lupus, called rhupus suggests a new entity with a different clinical profile and a poor response to treatment with NSAIDs alone. In addition, the occurrence of this association in JSLE patients could be classified as a clinical sub-group of JSLE with possible specific genetic determinants.
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