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Published on: September 20, 2018
Rhupus syndrome in children: A multi-center retrospective cohort study and literature review
Seher Sener1, Ezgi Deniz Batu1, Sezgin Sahin2
1Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine Ankara, Turkey.
Insights
This study analyzed pediatric rhupus patients, finding juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE) are common. Key features include polyarticular, asymmetric, erosive arthritis and skin, blood, and kidney involvement.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Systemic Lupus Erythematosus
- Juvenile Idiopathic Arthritis
Background:
- Rhupus syndrome, a rare condition, presents overlapping features of Systemic Lupus Erythematosus (SLE) and Rheumatoid Arthritis.
- Understanding the characteristics of pediatric rhupus is crucial for timely diagnosis and management.
Conclusions:
- This study represents the largest cohort of pediatric rhupus cases to date.
- Pediatric rhupus commonly involves polyarticular, asymmetric, and erosive arthritis.
- Significant SLE-related organ involvement, particularly skin, hematological, and kidney, is characteristic.
Objective:
In this study, we aimed to evaluate the characteristics of pediatric rhupus patients including all the related series in the literature.
Methods:
Thirty pediatric patients with rhupus syndrome from 12 different centers in Turkey were included in this study. The literature was also reviewed for pediatric patients with rhupus syndrome.
Results:
The most prominent phenotype of these 30 patients was juvenile idiopathic arthritis (JIA) (60%) at the disease onset and SLE (73.3%) at the last visit. Major SLE-related organ involvements were skin (80%), hematological system (53.3%), and kidney (23.3%). Arthritis was polyarticular (73.3%), asymmetric (66.7%), and erosive (53.3%) in most patients. Hydroxychloroquine (100%), glucocorticoids (86.7%), and mycophenolate mofetil (46.7%) were mostly used for SLE, while glucocorticoids (76.6%), methotrexate (73.3%), and nonsteroidal anti-inflammatory drugs (NSAIDs) (57.6%) were mainly preferred for JIA. Our literature search revealed 20 pediatric patients with rhupus syndrome (75% were RF positive). The most prominent phenotype was JIA (91.7%) at the disease onset and SLE (63.6%) at the last visit. Major SLE-related organ involvements were skin (66.7%), hematological system (58.3%), and kidney (58.3%). Arthritis was polyarticular (77.8%), asymmetric (63.6%), and erosive (83.3%) in most patients. Glucocorticoid (100%), hydroxychloroquine (76.9%), and azathioprine (46.2%) were mostly used for SLE, while methotrexate (76.9%) and NSAIDs (46.2%) were mainly preferred for the JIA phenotype.
Conclusion:
Our study is the largest cohort in the literature evaluating pediatric rhupus cases. Most of the pediatric patients had polyarticular, asymmetric, and erosive arthritis, as well as organ involvements associated with SLE, including the skin, hematological system, and kidney.
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