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Remitting seronegative symmetrical synovitis with pitting edema ( RS3PE)

Remitting seronegative symmetrical synovitis with pitting edema

Remitting seronegative symmetrical synovitis with pitting edema ( RS3PE) is a rare syndrome identified by symmetric polyarthritis, synovitis, acute pitting edema of the back of the hands and/or feet, and a negative serum rheumatoid factor.

 It has an excellent prognosis and responds well to treatment.

RS3PE typically involves the joints of the extremities, specifically the metacarpophalangeal and proximal interphalangeal joints, wrists, shoulders, elbows, knees and ankles: arms and hands are more commonly affected than the legs and feet.

It is more common in older adults, with the mean age between 70 and 80 years.

It occurs more often in men than in women with a 2:1 ratio.

With RS3PE typically have repeated episodes of inflammation of the lining of their synovial joints and swelling of the end portion of the limbs.

RS3PE is  usually bilateral.

RS3PE can be caused by many other conditions.

Has no definitive diagnostic test,

Other conditions have to be ruled out before this rare condition can be diagnosed.

Differential diagnosis: polymyalgia rheumatica (PMR), late onset (seronegative) rheumatoid arthritis, acute sarcoidosis, ankylosing spondylitis and other spondyloarthropathies such as psoriatic arthropathy, mixed connective tissue disease, chondrocalcinosis and arthropathy due to amyloidosis, and vasculitides such as polyarteritis nodosa.

With PMR pain, stiffness and weakness at the level of the shoulders and pelvic girdle with associated systemic symptoms of fever, malaise, fatigue, weight loss is typical.

PMR typically requires protracted courses of steroids, whereas corticosteroids can be tapered more quickly with persisting remission in RS3PE.

RS3PE has been documented in patients with malignancy:Non-Hodgkin’s lymphoma, gastric cancer, pancreatic cancer, lung cancer, breast cancer, colon cancer, prostate cancer and bladder cancer, among others.

Other causes of edema, don’t tend to manifest with pitting edema at the back of the hands.

Its pathophysiology) remains unknown. 

Possibly related  to vascular endothelial growth factor, or tenosynovitis of the extensors of the hands and feet.

Ultrasonography and magnetic resonance imaging of the hands and/or feet useful for diagnosis.

May be linked to HLA-B27.

Treatment: 

Responds to low dose corticosteroids, with sustained and often complete remission. 

Non-steroidal anti-inflammatory drugs , and hydroxychloroquine have proven effective in some cases.

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