Fig. 11.1
(a, b) Severe symmetrical lymphedema with development of elephantiasis in both lower limbs in a patient with the familial form of articular chondrocalcinosis (RS3PE)
Oedema may have both an acute and subacute form; it is often asymmetrical and affects mostly women. General inflammatory manifestations are usually slightly increased. Examination of synovial effusion under the polarising microscope may show calcium pyrophosphate dihydrate crystals, and radiological diagnosis may confirm calcification of hyaline cartilage. These findings together with a good response to non-steroidal antirheumatic drugs may contribute to proper diagnosis.
11.6 Reflex Sympathetic Dystrophy (Sudeck’s Disease)
Oedema on the hands may result in bilateral reflex sympathetic dystrophy. Marked pain limits active and passive motion; it is associated with vasomotor disorders, usually without arthritis. The predisposing factors include a history of myocardial infarction and sudden stroke; systemic inflammatory manifestations are not among the factors. The diagnosis of Sudeck’s atrophy may be supported by radiographic examination, bone scintigraphy and MRI.
11.7 Amyloid Arthropathy
Amyloid arthropathy is also often associated with pitting oedema of hands; sometimes it occurs in light chain disease within multiple myeloma. Oedema develops slowly and is rarely accompanied by short-term episodes of morning stiffness. It is a pseudoedema that has slightly inflammatory parameters and is relatively rigid by palpation. Diagnosis is based on clinical findings, including renal insufficiency with proteinuria.
Bioptic examination of the synovial membrane or synovial fluid may reveal the presence of amyloid which shows apple-green birefringence in polarised light when Congo red stained.
11.8 Late-Onset Spondyloarthropathy
Pitting oedema may be a manifestation of late-onset peripheral spondyloarthropathy [19]. It affects mainly lower extremities, less frequently hands, and is often asymmetrical [19, 20]. The patients are mostly middle-aged men, while RS3PE syndrome affects primarily individuals at the age of about 60 years. The patients present with mild oligoarthritis in lower extremities. The axial skeleton remains as a rule intact; the response to non-steroidal antirheumatic drugs and glucocorticoids is weak, and the disease usually evolves over the years. HLA-B27 is usually positive [21].
11.9 Sarcoidosis
Cantini et al. [22] studied RS3PE syndrome in lower extremities in acute sarcoidosis. Remitting distal extremity swelling with pitting oedema was the presenting manifestation in five (29 %) of 17 consecutive patients with acute sarcoidosis seen during a 2-year period. The swelling and pitting oedema were most prominent over the dorsum of both feet and ankles. In perimalleolar areas the oedema followed the distribution of tibial and peroneal tendons. MRI confirmed in three patients a severe tenosynovitis of peroneal, tibial and extensor tendons that was primarily responsible for the oedema in the subcutaneous and peritendinous ankle and foot soft tissues.
11.10 Whipple’s Disease
Olivieri et al. [3] reported five cases of RS3PE associated with the Whipple’s disease, manifested by pitting oedema over the dorsum of the hands and feet.
Conclusion
RS3PE syndrome is a sub-entity of seronegative symmetrical synovitis that occurs in elderly men with a marked pitting oedema, affecting primarily the hands. The disease is highly sensitive to the glucocorticoid therapy ensuring long-term remission after withdrawal of drugs. However, it should be pointed out that RS3PE syndrome may be also part of the paraneoplastic syndrome. Such a syndrome should be suspected particularly in cases where the response to glucocorticoid treatment is inadequate.
Differential diagnosis has to take into account that RS3PE syndrome does not always occur as an isolated disorder but may be part of the clinical features of other inflammatory and metabolic rheumatic diseases.
References
1.
McCarty DJ, O’Duffy JD, Person L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome. JAMA. 1985;254(19):2763–77.CrossRefPubMed