P0229 RS3PE (REMITTING SERONEGATIVE SIMETRICAL SYNOVITIS WITH PITTING EDEMA) SYNDROME ACCOMPANIED BY POLYCYTHEMIA VERA

Abstract

Introduction: Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome was originally reported by McCarty et al in 1985, and it is characterized by symmetrical arthritis with prominent pitting edema of the dorsum of the hands and/or feet. The aetiology of the syndrome is still unknown, but has been linked to infections, tumors and autoimmune diseases. The diagnosis is clinical, but must be differentiated from other rheumatic diseases. It has a fast response with steroid therapy and excellent prognosis. Objectives: Provision of a clinical case and literature review Materials & methods: Description of a case report Results: A 59-year-old man was admitted to our hospital for a three week history of fever, myalgia and arthralgia without response to treatment with antibiotics. He had a history of polycythemia vera treated with hydroxyurea. Physical examination showed the tender articular joints sacroiliac and back pain. Two weeks later, started with morning stiffness in wrists, hands, shoulders and ankles of 3-4 hours, with edema of hands and ankles. Laboratory studies revealed elevation of white blood cell count: WBC 16,000 (76% N, 6% L, M 2%, Eo 12%, 4% B), the erythrocyte sedimentation rate was increased at 55 mm in the first hour. Liver function, renal function, and electrolytes were normal. Serum ferritin 403 mg/dL, C reactive protein at 283 mg/dL. Electrophoresis demonstrated acute inflammatory pattern. Rheumatoid factor was negative. Antinuclear antibody titer was 1/160, and the rest of the serum antibodies were negative. Tumour markers were all negative. Microbiological studies (blood, cultures, stool, sputum and serology) were negative. Chest X-ray, abdominal ultrasound and thoracoabdominal CT were normal. Radiograph hands, elbows, ankles, knees, feet and shoulders showed increased density of soft parts. Echocardiogram was normal. Bone marrow aspirate showed myeloproliferative syndrome. Temporal artery biopsy was normal. A diagnosis of RS3PE was made and the patient was treated with low-dose prednisone with a rapid clinical response Discussion & conclusion: Our patient met 6 of 7 criteria for RS3PE established by Olivo (rheumatoid factor negative, polisinovitis symmetrical, edema with fovea, morning stiffness, response to steroid treatment, the exclusion of other diseases). Fever is not a frequent symptom in this syndrome, so we must rule out infectious processes. As in our case, there is often a rise of acute phase reactants and sometimes can be elevated antinuclear antibody titer low. It has described its association with solid tumors (lung, prostate, stomach, kidney) and hematologic (leukemia, lymphoma and myelodysplastic syndromes), but we have not found any literature associated with polycythemia vera described as in this patient