Myelodysplastic Syndrome Precedes the Onset of Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) Syndrome

Abstract

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is characterized by symmetrical synovitis predominantly involving the wrists, and is associated with marked pitting edema of the dorsum of the hands. Although the etiology of RS3PE syndrome is still unknown, several putative associations with malignancies and hematological disorders have been reported. Myelodysplastic syndrome (MDS) is characterized by infective hematopoiesis with possible transformation to leukemia; however, an association between RS3PE syndrome and MDS has been rarely reported. Here, we describe a 67-year-old man with MDS with refractory anemia who developed RS3PE syndrome 3 months after the diagnosis of MDS. The patient presented with polyarthritis with pitting edema at the dorsum of the hands, the elevated serum levels of C-reactive protein and a proinflammatory cytokine, interleukin-6, and the elevated plasma levels of vascular endothelial growth factor (VEGF). VEGF has been shown to be involved in the pathogenesis of RS3PE syndrome. Treatment with low doses of corticosteroids resulted in the regression of polyarthritis and pitting edema of the dorsum of the hands, as well as a reduction in the elevated levels of plasma VEGF. Partial resolution of refractory anemia was also observed with steroid therapy. In summary, RS3PE syndrome developed shortly after MDS was identified in this patient. The sequence of clinical events suggests that MDS-mediated immunological abnormalities including inflammatory cytokine induction may be responsible for the association between MDS and RS3PE syndrome. Patients with RS3PE syndrome should be screened for hematological disorders that promote proinflammatory mediators.

Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) Syndrome: A Single-Center Experience

“…Although the relation between RP and RS3PE and MDS is well known, RS3PE cases developing after RP have not been reported. [22] In our case, only RP and RS3PE concurrence were present without any hematological malignity. Also, unlike the other case, RS3PE developed after RP findings in a young female patient RS3PE syndrome, caused by crystal arthritis, in particular gout, is quite rare.…”

Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome Complicated with Organizing Pneumonia

“…Previous studies have suggested that VEGF may contribute to the pathological changes; both synovial hypervascularity (synovitis) and an increment in vascular permeability (subcutaneous edema) may be facilitated by VEGF in patients with RS3PE syndrome (18). Several reports of pleural effusion in patients with RS3PE syndrome have been published (19)(20)(21). Among them, the pleural effusion was turbid and exudative in five patients, and one patient showed a high level of serum VEGF.…”

Idiopathic remitting seronegative symmetrical synovitis with pitting edema syndrome associated with bilateral pleural and pericardial effusions: a case report

“…Several reports suggested that serum IL-6 and VEGF are increased in RS3PE syndrome [ 2 , 10 – 12 ], suggesting that these cytokines may be associated with symptoms of RS3PE syndrome. In our patient, serum IL-6 and VEGF were also markedly elevated when pleural and pericardial effusions were increased.…”