RS3PE Syndrome Research Articles

THU0251 How to PREDICT That Early Arthritis without Rheumatoid Factor and ACPA May Develop into Rheumatoid Arthritis According to Acr/Eular 2010 after A 3-Years Follow-Up? Results from the ESPOIR Cohort

ObjectivesTo describe disease course of patients without rheumatoid factor (RF) and anti-citrullinated protein auto-antibodies (ACPA) in an inception cohort of early arthritis patients. To determine baseline predictors of fulfilling 2010...

RS3PE syndrome: A case-report and review of literature

We describe a case of remitting symmetrical seronegative synovitis with pitting edema (RS3PE) syndrome in the elderly. We receive a patient of 82 years, followed for blood pressure, for pain in both hands sudden onset in 72 hours. He complains of schedule inflammatory pain in the hands and wrists. We find edematous swelling at the two wrists and the dorsal surface of the fingers, without paresthesia, without deformation of fingers. We do not notice the presence of arthritic nodules. These inflammatory pains remain isolated: he does not complain of back pain, or in other peripheral joint sites, like knees or at the shoulder and pelvic area. Laboratory tests show inflammatory syndrome, with an increase in CRP (80 mmol/L), but without elevation of the erythrocyte sedimentation rate, which is consistent with an acute impairment. Antibodies related to rheumatoid arthritis are negative. In fact, the immunological tests with rheumatoid serology are negative. Anti-CCP antibodies are negative also. Antibodies, relative to other autoimmune diseases, like Sjogren’s syndrome, are negative. X-rays of both hands and wrists do not show bone involvement, such as erosion. A RS3PE syndrome is diagnosed. Search for a causal neoplasia is normal. We note a real improvement with corticosteroids for our patient: clinical and biological improvement obtained in 1 week with corticosteroids, in low doses to 20 mg per day. Regression of corticosteroids has been made progressively. At 3 months, the patient had no pain in 5 mg of corticosteroids. At 6 months, the patient had no complaints without corticosteroids. RS3PE syndrome is an inflammatory arthritis affecting the elderly electively 65% of patients over 70 years [1]. It is more common in men. The onset is typically sudden, all symptoms settling in 24–48 hours. It has been described by McCarty et al. with ten observations, which, although meeting the criteria for rheumatoid arthritis, is distinguished by a unique clinical

RS3PE syndrome: Report of two cases

RS3PE syndrome: Report of two cases

AB0571 The study of the clinical characteristics between late-onset and early-onset spondyloarthritis

BackgroundSpondyloarthritis (SpA), including ankylosing spondylitis (AS), psoriatic arthritis (PsA), reactive arthritis (ReA), enteropathic arthritis, undifferentiated spondyloarthritis (uSpA), and synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, often involves the entheses. The differences between early-onset and...

Two Cases of RS3PE Syndrome Complicated by Type 2 Diabetes with Preceding Deterioration of Glycemic Control

Two Cases of RS3PE Syndrome Complicated by Type 2 Diabetes with Preceding Deterioration of Glycemic Control

High serum matrix metalloproteinase 3 is characteristic of patients with paraneoplastic remitting seronegative symmetrical synovitis with pitting edema syndrome

Recently, it was reported that remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome could be complicated with solid tumors. In a retrospective, multicenter study between October, 2003 and September, 2010, we investigated the characteristics of patients with paraneoplastic RS3PE syndrome who fulfilled following criteria: (1) bilateral pitting edema of hands or feet or both, (2) sudden onset of polyarthritis, and (3) age >50 years, (4) seronegativity for rheumatoid factor (RF). A total of 33 cases fulfilled the above criteria. Eight patients (seven men and one woman) developed cancer within 2 years of RS3PE syndrome onset. There was no significant difference between the neoplastic and nonneoplastic groups in the proportions of patients with fever, symmetrical polyarthritis, pitting edema, and good response to corticosteroids. Serum matrix metalloproteinase 3 (MMP-3) level (median 437.3 ng/ml) in the paraneoplastic RS3PE patients was significantly higher than that in patients without neoplasia (median 114.7 ng/ml) (p < 0.05). We found that high serum MMP-3 is characteristic of patients with paraneoplastic RS3PE syndrome.

Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome in a Chronic Hemodialysis Patient

A 75-year-old male who was undergoing chronic hemodialysis developed abrupt-onset pitting edema and pain in the dorsum of both hands and feet. Biochemical analysis disclosed increased C-reactive protein, and negative rheumatoid factor and antinuclear antibody. Radiological examination showed no bony erosion. Computed tomography and gallium scintigraphy revealed no active infection or neoplasms. The clinical diagnosis was remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. The pitting edema and inflammatory response quickly subsided after low-dose prednisolone therapy. This case demonstrates that RS3PE syndrome could be a differential diagnosis in elderly patients undergoing dialysis who develop pitting edema and joint pain.

High serum matrix metalloproteinase 3 is characteristic of patients with paraneoplastic remitting seronegative symmetrical synovitis with pitting edema syndrome

Recently, it was reported that remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome could be complicated with solid tumors. In a retrospective, multicenter study between October, 2003 and September, 2010, we investigated the characteristics of patients with paraneoplastic RS3PE syndrome who fulfilled following criteria: (1) bilateral pitting edema of hands or feet or both, (2) sudden onset of polyarthritis, and (3) age >50years, (4) seronegativity for rheumatoid factor (RF). A total of 33 cases fulfilled the above criteria. Eight patients (seven men and one woman) developed cancer within 2years of RS3PE syndrome onset. There was no significant difference between the neoplastic and nonneoplastic groups in the proportions of patients with fever, symmetrical polyarthritis, pitting edema, and good response to corticosteroids. Serum matrix metalloproteinase 3 (MMP-3) level (median 437.3ng/ml) in the paraneoplastic RS3PE patients was significantly higher than that in patients without neoplasia (median 114.7ng/ml) (p<0.05). We found that high serum MMP-3 is characteristic of patients with paraneoplastic RS3PE syndrome.

The rate of polymyalgia rheumatica (PMR) and remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome in a clinic where primary care physicians are working in Japan

We analyzed the rate of polymyalgia rheumatica (PMR) and remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome, both characterized as seronegative inflammatory arthritis in elderly, in an outpatient unit where primary care physicians are working in Japan to better understand the epidemiological characteristics of the diseases in Japan. Consecutive outpatients who newly visited at Department of General Medicine, Asahikawa Medical University Hospital, Japan, between April 2004 and March 2010 were analyzed. Each parameter such as age, sex, diagnosis, and biochemical examination was investigated. During the 6 years, 10 or 3 patients were diagnosed as PMR or RS3PE syndrome, respectively. The patients with PMR were 7 women and 3 men, and the average age at diagnosis was 69. Out of all patients aged over 50 (n = 3,347), the rate of PMR was 0.22% in men or 0.36% in women, respectively. On the other hand, RS3PE syndrome was diagnosed in 3 men (76, 76, and 81 years old). The rate of patients with RS3PE syndrome was 0.09% among outpatients aged over 50 indicating that the rate of PMR in an outpatient clinic in Japan is not far from previous findings reported from western countries. When compared with PMR, the rate of RS3PE syndrome was approximately one-third, providing for the first time the rate of RS3PE syndrome when compared with PMR. These epidemilogical data might help us pick up the diseases in primary care setting in Japan.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: A case report

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare form of paraneoplastic tenosynovitis, which is more prone to occur in elderly males. A 78-year-old male had a past history of gastric cancer 23 years ago and underwent subtotal gastrectomy then. He led a fair life after the surgery. However, fever, edema in four extremities, gait disturbance, and liver function impairment bothered him for 5 months, which caused significant physical functional decline. Despite of extensive laboratory and imaging examinations, no definite diagnosis and treatment were provided. He was referred to Kyoto University hospital and RS3PE was diagnosed. After the diagnosis of RS3PE syndrome was made, systemic steroid was given and fever, edema and liver function impairment improved dramatically within 7 days. The patient was well managed by oral prednisolone 30mg per day after discharge. RS3PE should be considered when an elderly man with aforementioned presentations in order to prevent significant functional decline in daily living.

Case of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) showing dermatomyositis‐like eruption

A 73-year-old woman developed linear erythema at the sites of scratching-induced scars on the bilateral thighs 2 weeks before the initial consultation. Subsequently, edematous erythema developed in the upper eyelids, dorsum of the nose and the face, and pitting edema in the dorsum of the bilateral hands and feet. The C-reactive protein (CRP) level was 8.2 mg/dL and erythrocyte sedimentation rate (ESR) 121 mm/h. The antinuclear antibody titer was 1:160, and rheumatoid factor (RF) and anti-Jo-1 antibody were negative. X-ray examination of the bilateral hands showed neither narrowing of the joint spaces nor bone erosion. Ga scintigraphy showed synovitis of the bilateral wrists. A diagnosis of remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE) was made. The erythema disappeared after diclofenac sodium administration. However, because the joint swelling and pitting edema did not improve, p.o. administration of prednisolone (20 mg/day) was initiated. The CRP and ESR levels normalized 2 months after the initiation of administration, and pitting edema disappeared after 3 months. We report this case because linear erythema like that observed in dermatomyositis has not been described as eruptions associated with RS3PE.

Monitoring of therapeutic efficacy in a patient with RS3PE syndrome by serologic variables and radiographic methods

We describe a typical case of a patient with remitting seronegative symmetrical synovitis and pitting edema (RS(3)PE) syndrome. He underwent a successful clinical course monitored by serologic variables and radiographic methods. Serum levels of interleukin-6 (IL-6), vascular endothelial growth factor (VEGF), matrix metalloproteinase-3 and serum amyloid A were remarkably elevated. Accumulation of inflammatory cells into the multiple joints was found by gallium-67 scintigraphy. Multiple and symmetrical tenosynovitis with hypervascularity in the presence of subcutaneous edema of the hands and feet were determined by magnetic resonance imaging (MRI) and ultrasonography. These serologic and radiographic abnormalities immediately improved after treatment with a low-dose steroid. Our present case supports a previous observation that synovial tissue is a major inflammatory source of RS(3)PE syndrome. IL-6 (and VEGF), probably produced from the synovial tissues, are considered to be essential factors in the development of RS(3)PE syndrome.

Clinical, radiological, and biochemical characteristics in patients with diseases mimicking polymyalgia rheumatica

To find out clues to differentiate between polymyalgia rheumatica (PMR) and other diseases that mimic PMR. We studied Japanese patients with PMR (n = 7), pseudogout (n = 1), remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome (n = 1), and post-infectious polyarthritis (n = 1). The distribution of inflammation in patients was evaluated using a gallium-67 scintigraphy. We measured serum C-reactive protein (CRP), matrix metalloproteinase-3 (MMP-3), and vascular endothelial growth factor (VEGF) in patients before and after treatment. Further, we compared the clinical course of PMR with that of other diseases that mimic PMR. Patients with pseudogout, RS3PE syndrome, post-infectious polyarthritis manifested similar changes in scintigraphic findings and serum CRP, MMP-3, and VEGF levels to PMR before the treatment. A significant reduction in serum CRP levels at one week after use of nonsteroidal anti-inflammatory drugs (NSAIDs) is a good clue to differentiate pseudogout and post-infectious polyarthritis from PMR. Chondrocalcinosis in the radiographs of joints is also effective to differentiate pseudogout from PMR. A small reduction of CRP levels after NSAIDs use and promptly ameliorated CRP and symptoms by a low-dose steroid therapy, which was commonly observed in patients with PMR, were also found in a patient with RS3PE syndrome. Pitting edema of the back of hands and gallium uptake in metacarpophalangeal (MCP) joints were useful to differentiate RS3PE syndrome from PMR. In conclusion, pseudogout, RS3PE syndrome, post-infectious polyarthritis should be included in the spectrum of diseases mimicking PMR. A promptly decreased serum CRP level by NSAIDs is a good clue to differentiate pseudogout and post-infectious polyarthritis from PMR. Pitting edema of the back of hands and symmetric gallium uptake in MCP joints are characteristic for RS3PE syndrome.

Gerontorheumatologie

Gerontorheumatology deals with the particular features of onset, course and treatment of rheumatic diseases in patients of advanced age. The initial diagnosis of inflammatory rheumatic disease after the age of 60 is hindered by the frequency of non-specific general disease symptoms. The most important gerontorheumatological diseases include rheumatoid arthritis first occurring at advanced age ("late onset rheumatoid arthritis", LORA), rheumatic polymyalgia and giant cell arteritis. Important differential diagnoses in older rheumatology patients are RS3PE syndrome, polyarticular chondrocalcinosis and paraneoplastic rheumatic syndrome. In principle, all specific basic therapeutics, immunosuppressants and biologics can be used for the therapy of these diseases. However, careful dose selection is particularly necessary in the initial phase, taking age-specific limitations in organ function and metabolism into consideration; moreover, close-meshed tolerance tests should be carried out.

Remitting seronegative symmetrical synovitis with pitting oedema associated with rifampicin

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE syndrome) is a very rare condition incorporating a tenosynovitis of the hands and wrists, as well as the feet, ankles and shoulders. The aetiology of RS3PE syndrome is unknown, although it has been linked with infectious agents (including mycobacteria), other rheumatological conditions, HLA serotypes and malignancies. This report examines the case of a 72-year-old man with a heart transplant and infected knee prosthesis, who developed RS3PE syndrome after introducing antibiotic treatment with rifampicin. His symptoms resolved with cessation of this agent. This case demonstrates a possible direct aetiological link between rifampicin and RS3PE.

Acute onset polyarthritis in older people: Is it RS3PE syndrome?

Remitting Seronegative Symmetrical Synovitis with Pitting oedema syndrome, a rare inflammatory arthritis, commonly affects people in the older age group. It can present as an acute onset polyarthritis with associated pitting oedema of the extremities. Patients show excellent response to low dose steroids with complete and sustained remissions. It can also be a paraneoplastic manifestation of an underlying occult malignancy, hence thorough clinical evaluation is warranted.We discuss a case of Remitting Seronegative Symmetrical Synovitis with pitting oedema syndrome where the patient presented with acute onset polyarthritis and pitting oedema of the extremities without an underlying systemic cause. Patient showed dramatic response to low dose steroids.

Polyarthrite œdémateuse et myopéricardite révélatrices d’une périartérite noueuse

Remitting symmetrical seronegative synovitis with pitting edema (RS3PE) syndrome is a rare type of seronegative polyarthritis occurring in the elderly. It can be associated to various diseases. We report a case of RS3PE syndrome associated with myopericarditis, leading to the diagnosis of polyarteritis nodosa in a 71-year-old patient admitted to the hospital for a febrile acute polyarthritis with pitting edema of the hands associated with a marked inflammatory syndrome. On second day of hospitalization, a sustained chest pain led to the diagnosis of myopericarditis. Muscular biopsy showed necrotizing vasculitis, characteristic of polyarteritis nodosa. The coexistence of RS3PE and myopericarditis has never been described in the literature. Its association with polyarteritis nodosa is also very rare and only one case has been previously reported.

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome following spontaneous rupture of a gouty tophus

A 70-year-old man with a 30-year history of gout presented with a ruptured gouty tophus over the right lateral malleolus. After the debridement of the tophus, bilateral arthralgia and pitting edema were observed in his extremities. Treatments with antibiotics and nonsteroidal antiinflammatory drugs were ineffective. However, prednisolone therapy was highly effective, and the patient's symptoms were rapidly ameliorated. Thus, we presume that rupture of a gouty tophus or its surgical treatment might contribute to the occurrence of RS3PE syndrome; however, in our case, the etiology of the syndrome remained unknown.

Remitting seronegative syndrome with pitting edema as the presenting symptom in a young, male patient with sarcoidosis: A case report

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a clinical diagnosis based on an abrupt onset of pitting edema of the dorsum of the hands or feet associated with synovitis of the wrist carpus and/or ankles, small joints, and tendon sheaths. RS3PE typically occurs in elderly patients, especially in men. A specific laboratory test for RS3PE syndrome is not yet available. However, recently published data provide evidence that color Doppler ultrasound (CDUS), contrast-enhanced CDUS, and magnetic resonance imaging are valuable tools in the diagnostic evaluation of involved anatomic structures in patients with RS3PE (1,2). Although idiopathic RS3PE has a benign prognosis with rapid and sustained response to low-dose steroid therapy, RS3PE may also be associated with different rheumatic and neoplastic diseases including myelodysplastic syndrome, non-Hodgkin’s lymphoma, T cell lymphoma, and chronic lymphocytic leukemia (3–11). The coincidence of RS3PE and sarcoidosis has been suggested in only one 62-year-old patient so far (12). Sarcoidosis is a chronic multisystemic disorder histologically characterized by noncaseating epithelioid cell granuloma (13). The lung is the most frequently affected organ ( 90%), but the skin, eyes, liver, and lymph nodes are also commonly involved (14). In this report, we describe the case of a 32-year-old man presenting with RS3PE syndrome. Further examinations led to the diagnosis of underlying sarcoidosis.

Remitting seronegative symmetrical synovitis with pitting oedema associated with lung malignancy

RS3PE syndrome is an inflammatory disease which affects mainly males and responds rapidly to low-dose steroids. We describe the concurrence of RS3PE and lung malignancy in a 78-year-old woman. We recommend that an underlying malignancy should always be excluded in patients with RS3PE syndrome, even when general signs and symptoms such as weight loss, anorexia and fever are absent or uncertain.