Dilek TEZCAN, Semral GÜLCEMAL, Muhammet LİMON, Sema YİLMAZ

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

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.

Anahtar Kelimeler:

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

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

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,

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