RS3PE and the role of ultrasound. A case report and brief review of the literature
Remitting seronegative symmetric synovitis with pitting edema (RS3PE) is a rare syndrome characterised by acute onset of symmetrical distal synovitis and tenosynovitis, associated with severe pitting edema of the dorsum of the hands and feet, seronegativity for autoimmunity and dramatic response to glucocorticoids. First described by McCarty et al. in 1985, RS3PE is reported mostly in elderly males, but it can also rarely occur in young people. Although it was initially regarded as a form of rheumatoid arthritis (RA), it is now considered a clinical distinct entity that can be associated with other rheumatologic conditions or be secondary to underlying diseases, such as cancer. As revealed by magnetic resonance imaging (MRI), the typical landmark of RS3PE is extensor tenosynovitis. Due to extensive subcutaneous edema of the extremities, the clinical assessment of synovitis and tenosynovitis is difficult. On the other hand, the use of MRI is expensive, time-consuming and requires experienced staffs. Musculoskeletal ultrasound (MSUS) may be a viable, reliable and cost-effective tool for evaluation of RS3PE patients. We hereby report the case of an 84 year-old woman presenting with bilateral hand and wrist swelling and morning stiffness. Clinical examination, laboratory tests and imaging led to a diagnosis of RS3PE. The aim of this report is to discuss the role of MSUS in RS3PE for the purpose of diagnosis and differential diagnosis compared with other rheumatologic conditions.
McCarty DJ, O’Duffy JD, Pearson L, et al. Remitting seronegative symmetrical synovitis with pitting edema: RS3PE syndrome. JAMA 1985;254:2763-7. DOI: https://doi.org/10.1001/jama.254.19.2763
Manger B, Schett G. Paraneoplastic syndromes in rheumatology. Nat Rev Rheumatol 2014;10:662-70. DOI: https://doi.org/10.1038/nrrheum.2014.138
Mainali NR, Schmidt TR, Alweis R, et al. Novel development of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome due to insulin therapy. Am J Case Rep 2014;15:119-22. DOI: https://doi.org/10.12659/AJCR.890318
Yao Q, Su X, Altman RD. Is remitting seronegative symmetrical synovitis with pitting edema (RS3PE) a subset of rheumatoid arthritis? Semin Arthritis Rheum 2010;40:89-94. DOI: https://doi.org/10.1016/j.semarthrit.2008.11.006
Kenzaka T. The Relationship between remitting seronegative symmetrical synovitis with pitting edema and vascular endothelial growth factor and matrix metalloproteinase 3. Intern Med Tokyo Jpn 2020;59:1021-2. DOI: https://doi.org/10.2169/internalmedicine.4239-19
Arima K, Origuchi T, Tamai M, et al. RS3PE syndrome presenting as vascular endothelial growth factor associated disorder. Ann Rheum Dis 2005;64:1653-5. DOI: https://doi.org/10.1136/ard.2004.032995
Joseph AD, Kumanan T, Aravinthan N, et al. An unusual case of remitting seronegative symmetrical synovitis with pitting edema: case report and literature review. SAGE Open Med Case Rep 2020;8:2050313X20910920. DOI: https://doi.org/10.1177/2050313X20910920
Möller I, Janta I, Backhaus M, et al. The 2017 EULAR standardised procedures for ultrasound imaging in rheumatology. Ann Rheum Dis 2017;annrheumdis-2017-211585.
Filippou G, Scanu A, Adinolfi A, et al. Criterion validity of ultrasound in the identification of calcium pyrophosphate crystal deposits at the knee: an OMERACT ultrasound study. Ann Rheum Dis 2020;annrheumdis-2020-217998.
Christiansen SN, Østergaard M, Terslev L. Ultrasonography in gout: utility in diagnosis and monitoring. Clin Exp Rheumatol 2018;7.
Filippou G, Filippucci E, Mandl P, et al. A critical review of the available evidence on the diagnosis and clinical features of CPPD: do we really need imaging? Clin Rheumatol 2020 [Epub ahead of print]. DOI: https://doi.org/10.1007/s10067-020-05516-3
Sakellariou G, Scirè CA, Adinolfi A, et al. Differential diagnosis of inflammatory arthropathies by musculoskeletal ultrasonography: a systematic literature review. Front Med 2020;7:141. DOI: https://doi.org/10.3389/fmed.2020.00141
D’Agostino M-A, Terslev L, Aegerter P, et al. Scoring ultrasound synovitis in rheumatoid arthritis: A EULAR-OMERACT ultrasound taskforce - Part 1: Definition and development of a standardised, consensus-based scoring system. RMD Open 2017;3. DOI: https://doi.org/10.1136/rmdopen-2016-000428
Origuchi T, Arima K, Umeda M, et al. Clinical outcomes in the first year of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Mod Rheumatol 2017;27:150-4. DOI: https://doi.org/10.1080/14397595.2016.1192744
Ughi N, Sebastiani GD, Gerli R, et al. The Italian Society of Rheumatology clinical practice guidelines for the management of polymyalgia rheumatica. Reumatismo 2020;72:1-15. DOI: https://doi.org/10.4081/reumatismo.2020.1268
Zayat AS, Ellegaard K, Conaghan PG, et al. The specificity of ultrasound-detected bone erosions for rheumatoid arthritis. Ann Rheum Dis 2015;74:897-903. DOI: https://doi.org/10.1136/annrheumdis-2013-204864
Filippou G, Scirè CA, Adinolfi A, et al. Identification of calcium pyrophosphate deposition disease (CPPD) by ultrasound: reliability of the OMERACT definitions in an extended set of joints - an international multiobserver study by the OMERACT calcium pyrophosphate deposition disease ultrasound subtask force. Ann Rheum Dis 2018;annrheumdis-2017-212542. DOI: https://doi.org/10.1136/annrheumdis-2017-212542
Cantini F, Salvarani C, Olivieri I, et al. Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study. Ann Rheum Dis 1999;58:230-6. DOI: https://doi.org/10.1136/ard.58.4.230
Klauser A, Frauscher F, Halpern EJ, et al. Remitting seronegative symmetrical synovitis with pitting edema of the hands: ultrasound, color doppler ultrasound, and magnetic resonance imaging findings. Arthritis Rheum 2005;53:226-33. DOI: https://doi.org/10.1002/art.21067
Agarwal V, Dabra AK, Kaur R, et al. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: ultrasonography as a diagnostic tool. Clin Rheumatol 2005;24:476-9. DOI: https://doi.org/10.1007/s10067-004-1061-x
Kawashiri S, Suzuki T, Okada A, et al. Differences in musculoskeletal ultrasound findings between RS3PE syndrome and elderly-onset rheumatoid arthritis. Clin Rheumatol 2020;39:1981-8. DOI: https://doi.org/10.1007/s10067-020-04931-w
Gutierrez M, Draghessi A, Bertolazzi C, et al. Ultrasound in psoriatic arthritis. Can it facilitate a best routine practice in the diagnosis and management of psoriatic arthritis? Clin Rheumatol 2015;34:1847-55. DOI: https://doi.org/10.1007/s10067-015-3053-4
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