Remitting seronegative symmetrical synovitis with pitting edema M67.-

Last updated on: 13.11.2022

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History
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McCarty DJ et al,1985

Definition
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Remitting seronegative symmetrical synovitis with pitting edema, also called RS3PE syndrome, is a rare syndrome characterized by symmetrical polyarthritis, synovitis, acute soft, misshapen (comparable to boxer's gloves) swelling on the dorsum of the hands and/or feet, and a negative serum rheumatoid factor. It was first described in 1985 in a series of ten patients by McCarty et al (McCarty DJ et al 1985).

Occurrence/Epidemiology
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Men> Women (ratio 2:1)

Etiopathogenesis
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Unknown, autoimmunological?

Paraneoplastic RS3PE: associations with lung and prostate cancers have been described (Yajima S et al. 2020).

Manifestation
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The syndrome is more common in older adults, with the average age in most studies ranging from 70 to 80 years.

Clinical features
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Clinically, patients present with usually acute, bilateral, massive, pillow-like swellings of the dorsum of the hands and/or feet, with associated synovitis of the hand and finger joints (the rheumatologic components are often masked). The edema is deeply depressible and shows prompt improvement on glucocorticoids and NSAIDs. Other joints affected, but less commonly, include the MTP and small toe joints, elbow, shoulder, hip, knee, and ankle joints.

Diagnosis
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Diagnostic criteria for RS3PE (McCarty DJ et al 1985):

  • localized edema, especially on the backs of the hands and/or forearms
  • acute onset of polyarthritis
  • age> 50 years
  • Seronegativity for RF.

Differential diagnosis
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Mixed connective tissue disease

Systemic scleroderma (Li H et al 2015; Tabeya T et al 2016).

Therapy
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If no underlying disease can be identified (idiopathic RS3PE), this entity is well treated with antiphlogistic therapeutic measures.

Case report(s)
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An 82-year-old Asian man presents with a 2-month history of symmetrical swelling in both hands. Despite treatment with the loop diuretic furosemide 40 mg/day, his condition did not respond to medication. His quality of life deteriorated. There was evidence of tenderness of the proximal interphalangeal joints, suggestive of synovitis with limited motion. Laboratory results showed hyperglycemia, elevated ESR (118 mm Hg), and elevated C-reactive protein of 6.58 mg/dL.

Radiographs of both hands showed soft tissue swelling, changes suggestive of osteoarthritis, but no erosions.

Therapy: prednisolone 15 mg daily initiated.

Literature
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  1. Bucaloiu ID et al (2007) Remitting seronegative symmetrical synovitis with pitting edema syndrome in a rural tertiary care practice: a retrospective analysis. Mayo Clin Proc 82:1510-1515.
  2. Li H et al (2015) RS3PE: clinical and research development. Curr Rheumatol Rep ;17:49.
  3. McCarty DJ et al (1985) Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome. JAMA. 1985;254:2763-7.
  4. O'Brien JG et al (2005), Chennubhotla SA, Chennubhotla RV. Treatment of edema. Am Fam Phys. 2005;71:2111-7.
  5. Tabeya T et al (2016) A case of angioimmunoblastic T-cell lymphoma with high serum VEGF preceded by RS3PE syndrome. Mod Rheumatol 26:281-285.
  6. Yajima S et al (2020) Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema (RS3PE), improved following surgical resection of prostatic carcinoma: A case report. Urol Case Rep 32:101232.
  7. Yasushi Tanaka et al (2022) Remitting seronegative symmetrical synovitis with pitting edema: a case report. Journal of Medical Case Reports 16: 125

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Last updated on: 13.11.2022