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Remitting seronegative symmetrical synovitis with pitting oedema

Author: Dr Jenny Chung, Dermatology Registrar, Auckland District Health Board, Auckland, New Zealand. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. July 2020.


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What is remitting seronegative symmetrical synovitis with pitting oedema?

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) is a rare rheumatic disease. It is also referred to as puffy oedematous hand syndrome and distal extremity swelling with pitting oedema [1].

Pitting oedema

Who gets remitting seronegative symmetrical synovitis with pitting oedema?

RS3PE is generally diagnosed in older men in their 70s and 80s [2,3].

What causes remitting seronegative symmetrical synovitis with pitting oedema?

Although the cause of RS3PE is unknown, the following may contribute to the disease.

  • An elevated level of vascular endothelial growth factor [3].
  • A solid tumour or haematological malignancy.
  • Parvovirus B19 infection [2,3].

What are the clinical features of remitting seronegative symmetrical synovitis with pitting oedema?

The distinguishing feature of RS3PE is peripheral oedema.

  • Oedema is usually of sudden onset.
  • Oedema can be generalised or, typically, localised to distal extremities [3].

What are the complications of remitting seronegative symmetrical synovitis with pitting oedema?

The complications of RS3PE relate to pitting oedema, and can include:

  • Synovitis
  • The range of motion of the affected joints is reduced [3]
  • Carpal tunnel syndrome
  • Cardiac complications [4].

How is remitting seronegative symmetrical synovitis with pitting oedema diagnosed?

RS3PE is a clinical diagnosis with the following features [4,5]:

  • Symmetrical synovitis – this can be detected on ultrasound or magnetic resonance imaging (MRI)
  • Pitting oedema of the hands and feet
  • An acutely elevated inflammatory response (raised C-reactive protein)
  • Negative rheumatoid factor and anti-CCP
  • Fast response to systemic corticosteroids.

What is the differential diagnosis for remitting seronegative symmetrical synovitis with pitting oedema?

While RS3PE is rare, there are other diseases that present with rapid peripheral oedema and symmetrical synovitis. These include the following conditions.

  • Polymyalgia rheumatica (PMR) — this affects men and women in their 50s, who present with symmetrical proximal morning stiffness, affecting the neck, torso, shoulders, thighs, and hips. PMR responds rapidly to corticosteroids.
  • Hypothyroidism — in severe hypothyroidism, pretibial myxoedema (non-pitting oedema more often associated with Graves disease) and other signs develop rapidly without synovitis.
  • Rheumatoid arthritis — classically a seropositive symmetrical small joint polyarthritis that only partially responds to oral corticosteroids.

What is the treatment and outcome for remitting seronegative symmetrical synovitis with pitting oedema?

The treatment for RS3PE is oral steroids with a typical starting dose of 10–20 mg of prednisone per day. Prognosis is reported to be good [2,5].

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References

  1. McCarty DJ, O’Duffy JD, Pearson L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. JAMA 1985; 254: 2763–7. DOI: 10.4103/0022-3859.147038. PubMed Central
  2. 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: 10.1080/14397595.2016.1192744. PubMed
  3. 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; 14: 119–22. DOI: 10.1016/j.jccase.2016.06.006. PubMed Central
  4. Okuma K, Furuhata R, Kiyota Y, et al. Acute progressive bilateral carpal tunnel syndrome associated with remitting seronegative symmetrical synovitis with pitting edema syndrome: a case report. J Orthop Surg 2019; 28: 1–5. DOI: 10.1177/2309499019893079. PubMed
  5. Tani K, Kawaminami S, Okura Y, et al. Predictive factors associated with the therapeutic response in patients with polymyalgia rheumatica and remitting seronegative symmetrical synovitis with pitting edema syndrome. J Med Invest 2019; 66: 112–8. DOI: 10.2152/jmi.66.112. PubMed

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