Remitting seronegative symmetrical synovitis with pitting oedema

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What is remitting seronegative symmetrical synovitis with pitting oedema?

Remitting seronegative symmetrical synovitis with pitting oedema/edema (RS3PE) is an uncommon rheumatic disease. It is also referred to as puffy oedematous hand syndrome and distal extremity swelling with pitting oedema.

 

 

Who gets remitting seronegative symmetrical synovitis with pitting oedema?

The typical patient diagnosed with RS3PE is an older man in his 70s or 80s. Most reported cases have been over 50 years of age, with a male to female predominance of 2:1.

There is a well-recognised association with haematological malignancies such as chronic lymphoid leukaemia and non-Hodgkin lymphoma, and many solid tumours.

What causes remitting seronegative symmetrical synovitis with pitting oedema?

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

• Elevated levels of vascular endothelial growth factor and matrix metalloproteinase 3
• Paraneoplastic syndrome
• HLA-B7, HLA-CW7, and HLA-DQW
• Parvovirus B19 and other infections including tuberculosis.

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

The distinguishing feature of RS3PE is pitting oedema unresponsive to diuretics.

• Oedema is usually of sudden onset.
• Oedema is typically localised to distal extremities resulting in a 'boxing glove' appearance of the hands.

Joints related to the oedema are painful with a reduced range of movement due to tenosynovitis. The skin may feel warm over the involved joints.

A recurring evening fever has been documented.

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

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

• Carpal tunnel syndrome
• Pleural effusions
• Cardiac complications.

How is remitting seronegative symmetrical synovitis with pitting oedema diagnosed?

Diagnosis of RS3PE is largely clinical. Diagnostic criteria have been proposed:

• Pitting oedema of all four limbs
• Age over 50 years
• Sudden onset
• Negative serology for rheumatoid arthritis.

Investigations may include:

• Ultrasound or magnetic resonance imaging (MRI) to demonstrate the symmetrical synovitis
• Absence of erosive arthritis on radiological examination
• An acutely elevated inflammatory response (raised C-reactive protein)
• Negative rheumatoid factor and anti-CCP.

As up to 50% of patients with RS3SE have been reported to have an underlying malignancy, age-appropriate screening should be considered.

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

Other conditions that may present with rapid peripheral oedema and symmetrical synovitis include the following:

• 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 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 with a rapid response within days.

Prognosis is reported to be good, although many patients are still requiring low-dose oral steroids and/or disease-modifying anti-rheumatic drugs such as methotrexate a year or more after diagnosis.

Treatment of an associated malignancy has been reported to result in resolution of the RS3PE signs and symptoms.