Tohoku J. Exp. Med., 2015 January, 235(1)

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

TAKAFUMI MATSUNAGA,¹ YASUMORI IZUMI,¹ NOZOMI IWANAGA,² CHIEKO KAWAHARA,¹ YOSHIKA SHIGEMITSU,¹ SHINICHIRO YOSHIDA,³ ATSUSHI KAWAKAMI,⁴ DAISUKE OGAWA⁵ and KIYOSHI MIGITA²

¹Department of General Internal Medicine, Nagasaki Medical Center, Omura, Nagasaki, Japan
²Department of Rheumatology, Nagasaki Medical Center, Omura, Nagasaki, Japan
³Department of Hematology, Nagasaki Medical Center, Omura, Nagasaki, Japan
⁴Department of Rheumatology, Nagasaki University Hospital, Nagasaki, Nagasaki, Japan
⁵Department of Hematology, Nagasaki Prefecture Shimabara Hospital, Shimabara, Nagasaki, Japan

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.

keywords —— corticosteroid; interleukin-6; myelodysplastic syndrome; remitting seronegative symmetrical synovitis with pitting edema syndrome; vascular endothelial growth factor

© 2015 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2015, 235, 47-52

Correspondence: Kiyoshi Migita, Nagasaki Medical Center, 2-1001-1 Kubara, Omura, Nagasaki 856-8652, Japan.

e-mail: migita@nagasaki-mc.com