An abrupt onset of seropositive polyarthritis with prominent distal tenosynovitis concomitant with bronochiolitis obliterans organizing pneumonia (BOOP): consideration of the relationship with RS3PE syndrome

A 64-year-old Japanese woman with a two-week history of polyarthralgia and persistent cough was diagnosed as seropositive polyarthritis and fulfilled the criteria of early rheumatoid arthritis (RA). In addition, inflammatory pitting edema of the distal extremities was apparent, suggestive of the remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. A number of investigations including hand MRI, bone scintigraphy and HLA typing supported a diagnosis of RS3PE syndrome rather than RA. Chest computed tomography revealed concomitant evidence of bronchiolitis obliterans organizing pneumonia (BOOP). Treatment with 30 mg of prednisolone daily immediately ameliorated the polyarthritis and the BOOP. Seropositive polyarthritis with distal pitting edema may be categorized as both RA and the RS3PE syndrome.     

Dynamic gadolinium-enhanced fat-suppressed T1-weighted MRI (chemical shift selective images) for remitting seronegative symmetrical synovitis with pitting edema

A 75-year-old man suddenly suffered from polyarthralgia and pitting edema in his distal extremities. Laboratory tests revealed inflammation, negative rheumatoid factor, and positive B7 human leukocyte antigen typing. Severe synovitis was observed in dynamic gadolinium-enhanced fat-suppressed (DGEFS) T1-weighted images of his right hand. Our diagnosis was remitting seronegative symmetrical synovitis with pitting edema (RS₃PE syndrome). He responded dramatically to low-dose corticosteroids, after which his synovitis remarkably improved as observed with DGEFS imaging. DGEFS imaging is useful for distribution of synovitis in patients with RS₃PE syndrome as well as assessing the efficacy of treatment.     

Oedematous polyarthritis and myopericarditis as presenting features of periarteritis nodosa

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 following acute intracranial hemorrhage

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is an unusual inflammatory arthritis with unknown pathogenic mechanism, and is characterized by an acute onset of symmetrical synovitis with pitting edema of the hands or feet. Numerous diseases are associated with RS3PE, including neoplasia, infection, Parkinson’s disease, and other rheumatic diseases. Neoplasia is the most common condition associated with RS3PE. We report a case of RS3PE that occurred following acute intracranial hemorrhage in an 80-year-old man with a 20-year history of hypertension.     

Remitting, seronegative (A) symmetrical synovitis with pitting edema--two cases of RS3PE syndrome

The sparing effect of neurological damage on the development and progression of several arthritic conditions has been documented. We describe the first 2 cases of unilateral remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome in individuals with neurologic disorders. Case 1 suffered from birth trauma resulting in paresis of the right upper extremity and developed RS3PE syndrome in the nonaffected extremities. Case 2 developed RS3PE syndrome on the nonparetic side 7 years after a cerebrovascular accident resulting in hemiparesis.     

Magnetic resonance imaging findings in a case of remitting seronegative symmetrical synovitis with pitting edema

We describe a case of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) in a 66-year-old man. This report discusses magnetic resonance imaging findings of RS3PE syndrome and the changes after steroid therapy.     

Seven cases of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome

Among the elderly patients with seronegative polyarthritis, McCarty et al. (1985) proposed a disease entity of "remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome", but only a few cases have been reported in Japan. Here we report 7 cases suspicious of RS3PE syndrome, 2 men and 5 women with an average age of 75.9 years, ranging from 67-82 years. Their common findings were (1) relatively acute onset, (2) polyarthritis, (3) pitting edema of the dorsum of both hands and both feet, and (4) negative rheumatoid factor and antinuclear antibody. McCarty et al. found that RS3 PE syndrome was more prevalent in men; however, in our experience, the opposite was observed. The clinical courses of all patients were good, and they were effectively treated either by small dosages of oral prednisolone, nonsteroidal antiinflammatory drugs, or Chinese herbal (Kampo) medicines. Since this syndrome might not be rare in Japan, it seems necessary to evaluate elderly patients with seronegative polyarthritis with pitting edema as RS3PE syndrome in their routine medical examinations.     

Gastric carcinoma in association with remitting seronegative symmetrical synovitis with pitting edema.

We describe an elderly woman who developed remitting seronegative symmetrical synovitis with pitting edema (RS3PE). In addition to typical findings, she had increased alkaline phosphatase; and investigations revealed metastatic gastric carcinoma. The literature relating to RS3PE in association with neoplasia is reviewed.     

Remitting seronegative symmetrical synovitis with pitting edema associated with subcutaneous Streptobacillus moniliformis abscess

We describe an 84-year-old woman who developed remitting seronegative symmetrical synovitis with pitting edema (RS3PE) associated with a subcutaneous abscess of the hand due to Streptobacillus moniliformis. Polyarthritis and edema were relieved after therapy with corticosteroids. We review the association of RS3PE to different rheumatologic, neoplastic, or infectious diseases.     

Remitting seronegative symmetrical synovitis with pitting edema following intravesical bacillus Calmette-Guérin instillation

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare syndrome of undetermined etiology occurring in the elderly. We describe the first case of RS3PE in a HLA-B27 positive 65-year-old man following intravesical bacillus Calmette-Guérin (BCG) instillation for bladder carcinoma. He developed symmetrical arthritis and synovitis involving wrists, knees, ankles, and metatarsophalangeal joints, with marked pitting edema of the dorsa of both hands and feet, fever, and elevated acute phase reactants. Right knee effusion revealed nonspecific sterile inflammatory fluid. He responded dramatically to nonsteroidal antiinflammatory drugs. BCG instillation may have triggered active symmetrical synovitis via local T cell activation and a T-helper-1 (Th-1)/Th-2 inflammatory profile.     

Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome): a report of two cases and review of the literature

We report two cases of paraneoplastic remitting seronegative symmetrical synovitis with pitting edema (RS3PE) associated with prostatic adenocarcinoma. One of the patients was positive for Helicobacter pylori and the other had secondary bone metastases. In the latter, the clinical picture of RS3PE developed after surgical intervention for the primary lesion. On physical examination, while the hands and feet were swollen in the first patient, pitting edema was present only at the feet of the other. All joints of the affected hands and feet were painful. Serological tests including rheumatoid factor, antinuclear antibody, and human leukocyte antigen B27 were all negative. Response to low-dose corticosteroid treatment was delayed in the first patient, but the symptoms were relieved better in the second one.     

Parvovirus B19: another agent associated with remitting seronegative symmetrical synovitis with pitting edema

A number of conditions have been associated with remitting seronegative symmetrical synovitis with pitting edema (RS3PE) and the controversy of whether this should be considered a syndrome rather than a disease continues. There are few reports on the role of infectious agents in the etiology of RS3PE, and human parvovirus B19 has not previously been linked to this syndrome. We describe a patient with RS3PE syndrome in association with positive serology and viremia for parvovirus B19. A 9-year followup failed to uncover another cause for RS3PE.     

Paraneoplastic remitting seronegative symmetrical synovitis with pitting edema

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a syndrome that may be associated with many conditions, including malignancy. Three further cases of paraneoplastic RS3PE are described and the literature is reviewed. Paraneoplastic RS3PE is more frequently associated with solid tumors, in particular adenocarcinoma. The two clinical characteristics suggestive of paraneoplastic RS3PE are systemic sign/symptoms and the poor response to corticosteroid therapy.     

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?相似文献   

RS3PE syndrome presenting as vascular endothelial growth factor associated disorder

OBJECTIVES: To characterise serum concentrations of various cytokines and detection by magnetic resonance imaging (MRI) of synovial hypervascularity in patients with remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome before and after corticosteroid treatment. METHODS: Vascular endothelial growth factor(165) (VEGF(165)), tumour necrosis factor alpha (TNFalpha), and interleukin 1beta (IL1beta) were measured by enzyme linked immunosorbent assay (ELISA) in serum samples from three patients with RS3PE syndrome. As controls, serum samples from 26 healthy volunteers, 12 patients with rheumatoid arthritis, 10 patients with systemic lupus erythematosus, 13 patients with polymyositis/dermatomyositis, 13 patients with vasculitis syndrome, and 6 patients with mixed connective tissue disease were also analysed. Synovial hypervascularity of patients with RS3PE syndrome was estimated by rate of enhancement (E-rate) in a dynamic MRI study. RESULTS: Serum concentrations of VEGF(165) (mean (SD) 2223.3 (156.3) pg/ml) were significantly higher in patients with active RS3PE syndrome than in controls before corticosteroid treatment. TNFalpha and IL1beta levels were similar in patients and controls. Synovial hypervascularity in affected joints and subcutaneous oedema decreased during corticosteroid treatment, in parallel with the fall in serum VEGF(165). CONCLUSIONS: VEGF promotes synovial inflammation and vascular permeability in patients with RS3PE syndrome, suggesting that RS3PE can be classified as a VEGF associated disorder.     

Remitting seronegative symmetrical synovitis with pitting edema syndrome: followup for neoplasia

OBJECTIVE: To investigate whether the remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome may represent a paraneoplastic disorder in a significant percentage of cases. METHODS: Patients diagnosed with RS3PE syndrome at the Medical College of Wisconsin before 1995 were telephoned and asked about their rheumatologic course since initial diagnosis of RS3PE and whether they had been diagnosed with any cancer. If so, permission was obtained to review tissue pathology. Criteria used for diagnosis of RS3PE syndrome included sudden onset of painful diffuse swelling of both hands associated with pitting edema of the dorsa of the hands without other synovitis or evidence of disease, negative rheumatoid factor, absence of radiologic abnormalities, and resolution within 6-12 months without sequelae. Data from the national SEER databank on population incidence of cancers in the appropriate sex, age, and ethnic groups for the years under study were used to assess relative risk for cancer. RESULTS: There were 10 patients for whom followup data were available. Four had a cancer diagnosed following recognition of the RS3PE syndrome; 1 patient developed non-Hodgkin's lymphoma initially diagnosed as hairy cell leukemia after 4 years; 1 developed acute lymphocytic leukemia with hyperdiploidy after 14 years; 1 was diagnosed with male breast cancer after 2.5 years; and 1 developed squamous cell lung cancer 12 months after RS3PE diagnosis. SEER data project an estimated rate of 2-3 cancers in a similar group of 10 patients of the same sex, age, and time period for this geographic area. CONCLUSION: The small sample size in this longterm followup precludes extrapolation to larger populations but suggests that there may be a slightly higher than expected rate of neoplasia in patients diagnosed with RS3PE syndrome. The interval between onset of RS3PE syndrome and diagnosis of cancer was fairly long, indicating that patients should be monitored for neoplasia with prudent age and sex specific surveillance for an extended period after diagnosis with RS3PE.     

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome: ultrasonography as a diagnostic tool

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome is characterized by symmetrical synovitis and swelling of both the upper and lower extremities. The anatomical determinant of RS3PE is predominantly extensor tenosynovitis as revealed by magnetic resonance imaging (MRI). Given the cost constraints, time, and expertise required in carrying out MRI and ease in diagnosing tenosynovitis by ultrasound, we utilized high-frequency ultrasonography (USG) for evidence of tenosynovitis of the distal tendons in patients with RS3PE. Diagnosis of tenosynovitis was made on the basis of anechoic or hypoechoic signals around the tendon sheaths in both transverse and longitudinal planes. Flexor and extensor tendons at the wrist and metacarpal heads and extensor digitorum longus (EDL) tendons at the ankle were evaluated with a 7.5–10-MHz linear probe. There were ten patients (seven males) with a mean age of 59.5 years (range: 52–78 years) and mean disease duration of 6.1 months (range: 1.5–12 months). Disease onset was acute in all of the cases. Pitting edema of the hands was present in all except two patients whereas four patients, in addition, had edema of the feet. Edema was symmetrical in seven patients. Inability to make a complete fist was noted in all. Tenosynovitis of extensor and flexor tendons at the wrist and the metacarpal heads was documented in all patients with edema of the hands. In seven cases extensor tendon tenosynovitis was more prominent compared to the flexor tendons. Tenosynovitis of EDL tendons was detected in six cases. Dramatic relief with low-dose prednisolone was noted in all patients within 6 weeks of therapy. At a mean follow-up of 10.1 months all patients had marked relief in edema of extremities and improvement in the grip strength. Our study confirms that tenosynovitis of both flexor and extensor tendons at the wrist and extensor tendons of the feet is the hallmark of RS3PE syndrome. USG is a reliable and cost-effective modality for evaluation of patients with suspected RS3PE.     

Pitting edema of the four extremities of rapid appearance

We report the case of a 70 year old white man with rapid onset edema of the four extremities without any other systemic symptoms. Cardiac failure, nephrotic syndrome and hepatic dysfunction were ruled out. Rheumatoid factor was slightly elevated at 33 UI/ml and sedimentation rate was within normal range. Ultrasound examination of the upper limb revealed increased blood flow (400 ml/min) at the level of the brachial artery and edema of the tenosynovial sheaths. A diagnosis of remitting seronegative symmetrical synovitis and pitting edema (RS3PE) was made and prednisone 15 mg/day was prescribed with rapid disappearance of the edema and reduction of the forearm blood flow.     

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome accompanied by Parkinson's disease

We encountered two cases of RS3PE (remitting seronegative symmetrical synovitis with pitting edema) syndrome accompanied by Parkinson's disease (PD). Although the etiology of RS3PE syndrome is still unknown, several possible associations, such as malignancies and viral infections, have been reported; RS3PE syndrome is thought to be an autoimmune-mediated disorder. The present patients did not have any factors which are reported to be associated with RS3PE. Whether or not the complication of PD and RS3PE syndrome is incidental needs to be further examined, and we discuss here the possible cause of association between PD and RS3PE syndrome, including dopamine agonists one of the anti-PD medications.     

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

Abstract

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.