Rheumatoid arthritis
RS3PE Presenting in a Unilateral Pattern: Case Report and Review of the Literature

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Objectives

To review the clinical features and pathophysiologic implications of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) presenting in a unilateral manner.

Methods

We identified and characterized an index case of RS3PE presenting in a unilateral pattern. We subsequently performed a systematic literature search to identify other reports of patients with unilateral RS3PE.

Results

The index case was a 76-year-old male with a prior history of right hemiparesis owing to a cerebrovascular accident 25 years prior, who developed a classic picture of RS3PE involving hand (metacarpophalageal and wrist joint) arthritis and dorsal pitting edema, accompanied by an elevated erythrocyte sedimentation rate, but only in the nonhemiparetic hand. The condition responded rapidly to low-dose prednisone. Our literature search identified 5 other cases of unilateral RS3PE, including 2 presented only in the Italian or German literature. Of the 5 cases, 2 were in patients with preexisting neurologic disease, in which the neurologically affected side was spared. One additional case initially presented as unilateral disease but rapidly progressed to bilaterality. Two cases presented in a fully unilateral manner despite no reported neurologic abnormalities on the unaffected sides.

Conclusions

While RS3PE is almost always a symmetric disease of the upper extremities, it may rarely present in a unilateral fashion. The apparent ability of neuropathic changes to protect against the expression of RS3PE in an extremity suggests a role for neural and possibly other local factors in the genesis/modulation of the onset or maintenance of RS3PE.

Section snippets

Methods

When an index case of unilateral RS3PE was identified, we conducted a literature search for additional cases previously reported. Search terms included “remitting seronegative symmetrical synovitis with pitting edema AND unilateral presentation,” “RS3PE AND unilateral,” “asymmetrical remitting seronegative symmetrical synovitis with pitting edema,” and “asymmetrical RS3PE.” Subsequently, a literature search for all review articles on RS3PE was conducted, using the terms “remitting seronegative

Index Case

A 76-year-old male was admitted to the hospital with 5 weeks of left hand and wrist swelling and pain. The patient had a past medical history of hypertension and right-sided hemiparesis secondary to a cerebrovascular accident at age 41 years. He first noted pain and swelling in his left hand and wrist during a physical therapy session but did not recall any injury during the session. He also denied trauma to the affected hand at any time before the onset of his symptoms. Subsequent to the onset

Discussion

In 1985, McCarty and coworkers reported the first series of 10 patients with RS3PE (21), defining a syndrome, primarily seen in elderly men, consisting of pain and swelling of the fingers and wrists, with pitting edema of the dorsa of the hands. The flexor and extensor tendon sheaths of the hands are also involved. The syndrome typically presents acutely and, according to McCarty and coworkers, patients can frequently pinpoint the onset of their condition “almost to the hour” (21). Laboratory

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    This study was unfunded. The authors have no conflicts of interest regarding the content of this manuscript.

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