Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) Syndrome: A Single-Center Experience

Year 2021, Volume: 11 Issue: 6, 804 - 810, 20.11.2021

Dilek Tezcan , Semral Gülcemal , Muhammet Limon , Sema Yilmaz

https://doi.org/10.16899/jcm.964824

Abstract

Objective: Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare disorder characterized by pitting edema, the onset of acute polyarthritis, and negative rheumatoid factor. The absence of joint erosion and good long-term response to low-dose steroids are its other characteristics. The present study aimed to evaluate the characteristics and accompanying conditions of RS3PE for contributing to the literature on the issue.
Material and method: This is cross-sectional descriptive study was carried out in a rheumatology clinic between January 2019- October 2020. Fifteen patients over 18 diagnosed with RS3PE were included in the study.
Results: Of 15 patients diagnosed with RS3PE, seven were female, and eight male. The following comorbid disorders were present: relapsing polychondritis, scleroderma, seropositive rheumatoid arthritis, gout, monoclonal gammopathy of unknown significance, and primary myelofibrosis. Most patients other than the ones who have hematological malignities and those diagnosed with relapsing polychondritis responded rapidly to steroids, and no recurrence occurred.
Conclusion: RS3PE may be associated with neoplasia, drugs, and various rheumatismal conditions, suggesting that it may be heterogeneous and considered a paraneoplastic rheumatic disease. The rare occurrence of this syndrome leads clinicians to miss it commonly. It is essential to increase awareness of this entity among clinicians.

Keywords

Remitting seronegative symmetrical synovitis with pitting edema syndrome, paraneoplastic; arthritis, pitting edema

Tekrarlayıcı Seronegatif Pitting Ödemli Simetrik Sinovit (R3SPE) Sendromu: Tek Merkez Deneyim

Abstract

Amaç: Tekrarlayıcı seronegatif pitting ödemli simetrik sinovit sendromu (R3SPE), akut poliartrit başlangıcı olan, negatif romatoid faktör ve pitting ödem ile karakterize nadir bir hastalıktır. Eklem erozyonu yapmaması ve düşük doz steroide iyi yanıt vermesi diğer özellikleridir. Bu çalışma, RSPE sendromu özelliklerini ve eşlik eden durumları değerlendirmeyi ve literature katkıda bulunmayı amaçlamaktadır.
Gereç ve Yöntem: Bu tanımlayıcı vaka bazlı retrospektif çalışma Romatoloji kliniğinde Ocak 2019- Ekim 2020 tarihleri arasında yapıldı. 18 yaş üstü on beş RS3PE hastası çalışmaya dahil edildi.
Bulgular: RS3PE tanısı konulan 15 hastanın yedisi kadın ve sekizi erkekti. Tekrarlayan polikondrit, skleroderma, seropozitif romatoid artrit, gut, önemi bilinmeyen monoklonal gamopati ve primer miyelofibroz komorbid bozuklukları mevcuttu. Hematolojik malignitesi olanlar ve tekrarlayan polikondrit tanısı konanlar dışındaki hastaların çoğu steroid tedavisine hızla yanıt verdi ve nüks gözlenmedi.
Sonuç: RS3PE sendromu, heterojen bir hastalıktır. Neoplazi, ilaçlar ve çeşitli romatizmal hastalıklar ile ilişkili olabilmekte ayrıca paraneoplastik romatizmal hastalik olabileceği düşünülmektedir. Bu sendrom nadir görülmesi nedeniyle klinisyenlerin gözünden kaçabilir ve bu yüzden klinisyenler arasında hastalık açısından farkındalığını arttırmak esastır. 

Keywords

artrit, komorbiditeler, paraneoplastik, pitting ödem, tekrarlayıcı seronegatif pitting ödemli simetrik sinovit

References

• REFERENCES: 1. McCarty DJ, O'Duffy JD, Pearson L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome JAMA 1983; 254:2763–2767
• 2. Lakhmalla M, Dahiya DS, Kichloo A, Fatima T, Edigin E, Wani F. Remitting seronegative symmetrical synovitis with pitting edema: a review. J Investig Med 2021 Jan; 69(1):86-90.
• 3. Kenzaka T. The Relationship between Remitting Seronegative Symmetrical Synovitis with Pitting Edema and Vascular Endothelial Growth Factor and Matrix Metalloproteinase 3. Intern Med 2020; 59(8):1021-1022
• 4. Oide T, Ohara S, Oguchi K, et al. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome in Nagano, Japan: clinical, radiological, and cytokine studies of 13 patients. Clin Exp Rheumatol 2004 Jan-Feb; 22(1):91-8.
• 5. Özşahin M, Ataoğlu S, Turan H. Unilateral RS3PE with young-onset rheumatoid arthritis. Seminars in Arthritis and Rheumatism 2011; 40(4):e1.doi: 10.1016/j.semarthrit.2010.09.001.
• 6. Pariser KM, Canosa JJ. Remitting seronegative symmetrical synovitis with pitting edema-two cases of RS3PE syndrome. J Rheumatol 1991; 18(8):1260-2.
• 7. Finnell JA, Cuesta IA. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: a review of the literature and a report of three cases. J Foot Ankle Surg 2000; 39:189–93.
• 8. Joseph AD, Kumanan T, Aravinthan N, Suganthan N. An unusual case of remitting seronegative symmetrical synovitis with pitting edema: Case report and literature review. SAGE Open Med Case Rep 2020 Mar 18; 8:2050313X20910920. DOI: 10.1177/2050313X20910920.
• 9. 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 Apr 15; 53(2):226-33.
• 10. Eguia HA, Parodi Garcia JF, Ramas Diez C, Eguia A EA. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) case presentation and comparison with other polyarthritides affecting older people. Age Ageing 2017 Mar 1; 46(2):333-334. 11. Karmacharya P, Donato AA, Aryal MR, et al. RS3PE revisited: a systematic review and meta-analysis of 331 cases. Clin Exp Rheumatol 2016 MayJun; 34(3):404-15.
• 12. Paira S, Graf C, Roverano S, Rossini J. Remitting seronegative symmetrical synovitis with pitting edema: a study of 12 cases. Clin Rheumatol 2002 May; 21(2):146-9.
• 13. Varshney AN, Singh NK. Syndrome of remitting seronegative symmetrical synovitis with pitting edema: a case series. J Postgrad Med 2015; 61(1):38-41
• 14. Pittau E, Passiu G, Mathieu A. Remitting asymmetrical pitting edema in systemic lupus erythematosus: Two cases studied with magnetic resonance imaging. Joint Bone Spine 2000; 67: 544–549
• 15. Günaydin, I, Daikeler T, Mohren M. Lower limb pitting edema in systemic lupus erythematosus. Rheumatol Int 1999; 18: 159–160
• 16. Alpigiani MG, Giampietri L, Emmanuele V, Salvati P, Valle M, Lorini R. Remitting symmetrical pitting edema of hands and feet at onset of pediatric systemic lupus erythematosus: a case report. Clin Exp Rheumatol 2008 Nov-Dec; 26(6):1166.
• 17. Hegazi MO, Saleh F, Al Rashidi A, Yaktien MM. Synovitis with pitting edema as the presenting manifestation of systemic lupus erythematosus. Lupus 2014 Sep; 23(10):1069-72.
• 18. Choi YM, Sheen DH, Lee YJ, Lee EB, Song YW. Sjogren's syndrome presenting as remitting seronegative symmetric synovitis with pitting edema (RS3PE). J Korean Med Sci 2003 Aug; 18(4):606-8.
• 19. Manganelli P, Delsante G, Bianchi G, Fietta P, Quaini F. Remitting seronegative symmetrical synovitis with pitting edema in a patient with myelodysplastic syndrome and relapsing polychondritis. Clin Rheumatol 2001; 20(2):132-5.
• 20. Matsunaga T, Izumi Y, Iwanaga N, et al. Myelodysplastic syndrome precedes the onset of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Tohoku J Exp Med 2015 Jan; 235(1):47-52.
• 21. Palazzi C, Olivieri I, D'Amico E, Pace-Palitti V, Petricca A. Symmetrical pitting edema resembling RS3PE in gout. Clin Rheumatol 2003 Dec; 22(6):506-7.
• 22. Sugisaki K, Hirose T. Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome following spontaneous rupture of a gouty tophus. Mod Rheumatol 2008; 18:630–3.
• 23. Horai Y, Origuchi T, Iwanaga N, et al. Clinical analysis of pre-existing diabetes mellitus and dipeptidyl peptidase-4 inhibitors in patients with remitting seronegative symmetrical synovitis and pitting edema syndrome. Mod Rheumatol 2020 Jul; 30(4):703-707.
• 24. Yamauchi K, Sato Y, Yamashita K, Funase Y, Kaneko T, Hashimoto T, et al. RS3PE in association with dipeptidyl peptidase-4 inhibitor: report of two cases. Diabetes Care 2012 Feb; 35(2):e7. DOI: 10.2337/dc11-1995.
• 25. Yokota K, Igaki N. Sitagliptin (DPP-4 inhibitor)-induced rheumatoid arthritis in type 2 diabetes mellitus: a case report. Intern Med 2012; 51(15):2041-4.
• 26. Mainali NR, Schmidt TR, Alweis R, George DL. Novel Development of Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) Syndrome due to Insulin Therapy. Am J Case Rep 2014 Mar 21; 15: 119-22.
• 27. Oyama K, Taniguchi JI, Goto R, Matsui K. Remitting seronegative symmetrical synovitis with pitting edema syndrome in individuals with type 2 diabetes mellitus or impaired glucose tolerance. Diabetes Res Clin Pract 2015 Oct; 110(1):e5-e8.
• 28. Manzo C, Natale M. Polymyalgia Rheumatica in Association with Remitting Seronegative Synovitis with Pitting Edema: a Neoplastic Warning. Can Geriatr J 2017; 20(2):94-96.
• 29. Tunc SE, Arslan C, Ayvacioglu NB, Sahin M, Akkus S, Yorgancigil H. Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome): a report of two cases and review of the literature. Rheumatol Int 2004 Jul; 24(4):234-7.
• 30. Nojima Y, Ihara M, Adachi H, Kurimoto T, Nanto S. Impact of low-dose prednisolone on refractory pitting edema manifesting remitting seronegative symmetrical synovitis with pitting edema syndrome. J Cardiol Cases 2016 Jul 15; 14(4):119-122.