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Table 1 Clinical findings in remitting seronegative symmetrical synovitis with pitting edema, polymyalgia rheumatica, and calcium pyrophosphate (CPP) crystal arthritis [2, 4,5,6, 10, 11]

From: Idiopathic remitting seronegative symmetrical synovitis with pitting edema syndrome mimicking symptoms of polymyalgia rheumatica: a case report

Disease

RS3PE

PMR

CPP arthritis

Age (years)

Typically > 50

Over 60

typically > 60

Sex

Male > female

Female > male

=

Symptom onset

Sudden

Progressive

Sudden or progressive

Pitting edema (hands and feet)

Common, symmetrical

Synovitis small articulations

++

+ (30%)

+

Shoulder and pelvic girdle pain/stiffness

+/−

+++

+

Functional impact

High

Moderate

Moderate

Radiological erosions

Elevation of CRP, ESR

+

++

+

RF

Negative (100%)

Negative/positive (16%)

Negative (100%)

Anti-CCP antibodies

Negative

Negative/positive

Negative

Genetic factors

HLA B7 (55%)

HLA DR4

ANKH gene mutation

Response to corticoid therapy

High, spectacular

High

High

Treatment duration

Short, several weeks/months

2−3 years

Variable

Relapse

Rare

Common within 2 years after diagnosis

Frequent

Paraneoplastic syndrome

20%

2.4%

No

Up to 30% if association of RS3PE and PMR

To be considered if resistant to glucocorticoids

 
  1. CPP Calcium pyrophosphate, CRP C-reactive protein, ESR erythrocyte sedimentation rate, RF rheumatoid factor, anti-CCP antibodies anti-cyclic citrullinated peptides antibodies, HLA human leukocyte antigen, ANKH the human homolog of the protein product of the murine progressive ankylosis gene. The multipass membrane protein participates in regulating pyrophosphate levels