Background/Purpose

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome is a rare inflammatory arthritis, first described by McCarty et al in 1985 characterised by abrupt onset of symmetrical distal synovitis, marked pitting edema of the dorsum of the hands and/or feet, absence of rheumatoid factor (RF), and favorable response to corticosteroids. The aim of our study is to further delineate the clinical and laboratory features, their correlation with various rheumatologic diseases and malignancies; and response to treatment.

Methods

A systematic electronic search of Medline, PubMed, and EMBASE for case reports, case series, and related articles of RS3PE published from 1985 till Feb 2014 was carried out. Statistical analysis was done using EXCEL and SPSS version 20.0, comparing categorical variables with Chi-square tests and frequencies of means via t-tests.

 

Results

Two hundred fifty cases of RS3PE were identified from 91 articles. RS3PE was found predominantly in males (70%) and the age at onset was 69±11 years. It was symmetrical in most cases (98%) and majority involved dorsum of the hands. Patients had a mean white cell count of 8,800/mm3 and elevated erythrocyte sedimentation rate (63±35mm/hr) and C-reactive protein (569±2687mg/dl). RF was negative in most cases (97%). Vascular endothelial growth factor (VEGF) was found to be significantly elevated (2-3 times above normal) in the 5 cases reporting it. Radiographic joint erosions were found in 2%. Most patients responded to low dose prednisone (16.39±10 mg/day). Prednisone dose was not associated with reported presence of malignancy (16 vs 18 mg, p=not significant). NSAIDs were useful in the acute setting in addition to the steroids. Other therapies such as hydroxychloroquine and methotrexate were used with variable efficacy.  Concurrent malignancy was present in 13.6% (3.6% hematological, 10% solid organ malignancies). These patients were found to have more severe disease requiring higher dose of prednisone (16 vs. 22 mg). Other rheumatological diseases were present in 2.8%. CRP was significantly higher in those with underlying malignancy (3293 vs. 374, p<0.001), but ESR was not (65 vs 62 mm, p=0.4).

Conclusion

RS3PE seems to be a distinct entity rather than a subset of other rheumatologic diseases or a paraneoplastic syndrome. Although we cannot establish an association between malignancy and RS3PE from this study, clinicians should be aware that patients with concurrent cancer tend to have more severe presentation and resistance to low-dose steroids. The cost effectiveness of aggressive cancer screening for patients with RS3PE compared to age-appropriate screening will need further research. Serum VEGF levels may be associated with its pathogenesis and it might be useful for the diagnosis and monitoring of disease activity. Uncomplicated cases of RS3PE usually have an excellent prognosis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/revisiting-rs3pe-after-twenty-five-years-a-systematic-review-of-250-cases/