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1.
OBJECTIVE: To determine the clinical characteristics of patients with "pure" remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome, and to investigate its relation with polymyalgia rheumatica (PMR). Magnetic resonance imaging (MRI) was used to describe the anatomical structures affected by inflammation in pure RS3PE syndrome. METHODS: A prospective follow up study of 23 consecutive patients with pure RS3PE syndrome and 177 consecutive patients with PMR diagnosed over a five year period in two Italian secondary referral centres of rheumatology. Hands or feet MRI, or both, was performed at diagnosis in 7 of 23 patients. RESULTS: At inspection evidence of hand and/or foot tenosynovitis was present in all the 23 patients with pure RS3PE syndrome. Twenty one (12%) patients with PMR associated distal extremity swelling with pitting oedema. No significant differences in the sex, age at onset of disease, acute phase reactant values at diagnosis, frequency of peripheral synovitis and carpal tunnel syndrome and frequency of HLA-B7 antigen were present between patients with pure RS3PE and PMR. In both conditions no patient under 50 was observed, the disease frequency increased significantly with age and the highest frequency was present in the age group 70-79 years. Clinical symptoms for both conditions responded promptly to corticosteroids and no patient developed rheumatoid arthritis during the follow up. However, the patients with pure RS3PE syndrome were characterised by shorter duration of treatment, lower cumulative corticosteroid dose and lower frequency of systemic signs/symptoms and relapse/recurrence. Hands and feet MRI showed evidence of tenosynovitis in five patients and joint synovitis in three patients. CONCLUSION: The similarities of demographic, clinical, and MRI findings between RS3PE syndrome and PMR and the concurrence of the two syndromes suggest that these conditions may be part of the same disease and that the diagnostic labels of PMR and RS3PE syndrome may not indicate a real difference. The presence of distal oedema seems to indicate a better prognosis.  相似文献   

2.
OBJECTIVE: To assess the role of -174 G/C promoter polymorphism of interleukin 6 (IL-6) in the susceptibility to polymyalgia rheumatica (PMR). We also investigated whether this polymorphism modulates the circulating level of IL-6 and the risk of relapse/recurrence in a series of patients with PMR followed up prospectively. METHODS: A prospective study of 112 consecutive, untreated patients with isolated PMR (i.e., without evidence of giant cell arteritis) who were followed up for at least 24 months. This cohort represented all patients diagnosed over a 5-year period in one Italian rheumatological secondary referral center. Patients were monitored for clinical signs/symptoms and acute-phase reactants. All PMR patients and 112 population-based controls from the same geographic area were genotyped for IL-6 polymorphism at position -174 by molecular methods. IL-6 serum levels were measured in 67 PMR patients and 43 population-based controls. RESULTS: The distribution of the G/C 174 genotype was similar in PMR patients and controls. No significant associations with IL-6 promoter polymorphism at position -174 were found when PMR patients with and without relapse/recurrence were compared. Controls homozygous for the C allele had higher serum IL-6 levels than the carriers of the G allele (4.5 +/- 3.7 pg/ml vs 1.8 +/- 2.1 pg/ml, p = 0.01). Patients homozygous for the allele C had significantly higher values of IL-6 during followup than patients carrying GC or GG genotypes. CC homozygosity was significantly more frequent in patients with persistently elevated levels of IL-6 than in those without. The presence of persistently elevated IL-6 levels, but not the CC genotype, was associated with an increased frequency of relapse/recurrence. CONCLUSION: Our findings show that the 174 G/C promoter IL-6 polymorphism is not implicated in susceptibility to PMR. However, CC genotype characterized PMR patients with persistently elevated levels of IL-6 who are at higher risk of developing relapse/recurrence. A genetically modulated pattern of IL-6 production could affect the longterm outcome of patients with PMR.  相似文献   

3.
Salvarani C  Cantini F  Hunder GG 《Lancet》2008,372(9634):234-245
Polymyalgia rheumatica and giant-cell arteritis are closely related disorders that affect people of middle age and older. They frequently occur together. Both are syndromes of unknown cause, but genetic and environmental factors might have a role in their pathogenesis. The symptoms of polymyalgia rheumatica seem to be related to synovitis of proximal joints and extra-articular synovial structures. Giant-cell arteritis primarily affects the aorta and its extracranial branches. The clinical findings in giant-cell arteritis are broad, but commonly include visual loss, headache, scalp tenderness, jaw claudication, cerebrovascular accidents, aortic arch syndrome, thoracic aorta aneurysm, and dissection. Glucocorticosteroids are the cornerstone of treatment of both polymyalgia rheumatica and giant-cell arteritis. Some patients have a chronic course and might need glucocorticosteroids for several years. Adverse events of glucocorticosteroids affect more than 50% of patients. Trials of steroid-sparing drugs have yielded conflicting results. A greater understanding of the molecular mechanisms involved in the pathogenesis should provide new targets for therapy.  相似文献   

4.
Two elderly patients presented with generalized aches and pains (particularly in the shoulders and the pelvic girdle), stiffness, fatigue, anemia, and an elevated erythrocyte sedimentation rate, but there were no signs or symptoms directly referable to the joints. Two and five months later respectively, pain, swelling, and signs of synovitis appeared in several joints in a symmetrical pattern, and a diagnosis of rheumatoid arthritis was made. Rheumatoid arthritis in the elderly may resemble polymyalgia rheumatica. On the other hand, synovitis in many patients with polymyalgia rheumatica may resemble rheumatoid arthritis. In the elderly, the differentiation of these two entities may be difficult. Moreover, patients initially presenting with the signs and symptoms of polymyalgia may eventually manifest typical rheumatoid arthritis.  相似文献   

5.
Clinically detectable joint swelling was found in 10 of 13 fully evaluated patients considered to have polymyalgia rheumatica. Five patients had some joint findings at disease onset. Knees were most commonly affected. Sternoclavicular involvement was seen in 3 patients. Joint effusions in 8 patients had 300-5,700 leukocytes/mm3 with a mean of 2,900. Six synovial biopsy specimens studied by light microscopy revealed mild to moderate synovial proliferation and chronic inflammation that was generally less severe than in typical rheumatoid arthritis. Electron microscopy identified microvascular changes and large amounts of vesicular and granular debris in lining cells. In 1 patient, a "fingerprint" pattern in the granular material was suggestive of the findings in some immune complexes. This still unexplained synovitis may, as previously suggested, be important in the pathogenesis of the polymyalgia rheumatica syndrome.  相似文献   

6.
OBJECTIVE: To assess the value of gray-scale ultrasound (US), color Doppler ultrasound (CDUS), contrast-enhanced CDUS, and magnetic resonance imaging (MRI) in the diagnostic evaluation of the hands in patients with remitting seronegative symmetrical synovitis with pitting edema (RS3PE). METHODS: Eight patients (5 men, 3 women; mean +/- SD age 69.3 +/- 7.2 years) with clinical diagnosis of RS3PE syndrome underwent US, CDUS, contrast-enhanced CDUS, and MRI. US was performed with a linear array transducer operating at 12 MHz. The US contrast agent (SHU 508; Levovist, Schering, Germany) was intravenously infused in a concentration of 300 mg/ml at a rate of 1 ml/minute. RESULTS: All patients showed symmetric subcutaneous edema and synovitis of tendons and finger joints on both US and MRI. Vascularity was detected subcutaneously in tendon sheaths and in the joint synovia on CDUS and MRI. Detection of increased vascularity was improved after contrast administration compared with unenhanced CDUS (P < 0.01). CONCLUSION: Ultrasound, CDUS, contrast-enhanced CDUS, and MRI are valuable tools in the diagnostic evaluation of involved anatomic structures in patients with RS3PE. Contrast-enhanced CDUS is superior to CDUS in assessment of inflammatory edema, effusion, and synovitis.  相似文献   

7.
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.  相似文献   

8.
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.  相似文献   

9.
Seventy patients with polymyalgia rheumatica (PMR) were seen at a suburban rheumatology practice from July 1983 to December 1987. Six of these patients presented without the typical limb girdle features associated with PMR. Presenting symptoms included peripheral synovitis or unilateral shoulder pain (3 patients), lower leg pain (3 patients), carpal tunnel syndrome (1 patient), and abdominal pain (1 patient). The disease evolved into the recognizable syndrome of PMR over a period of 2-12 months. We suggest that PMR may present in a variety of guises, or have a "stuttering evolution" to the full syndrome. The presenting manifestations of these atypical cases result from peripheral synovitis and thus represent a variant of the more common subclinical proximal synovitis seen in PMR. Increased clinical awareness of atypical presentations may assist earlier diagnosis and effective treatment.  相似文献   

10.
Myalgias most commonly occur in polymyalgia rheumatica (PMR). About 45% of patients with giant cell arteritis present with symptoms of PMR. Other vasculitides may also lead to arthralgia and myalgia. While shoulder and pelvic pain is characteristic for PMR pain often also occurs in the back of the neck and in the region of the thoracic spine. In addition, patients often present with malaise, morning stiffness and weight loss. CRP and ESR are elevated. Ultrasound and MRI delineate minor synovitis, tenosynovitis and bursitis in the shoulder. Hip joint synovitis and trochanteric bursitis are also commonly seen. PMR should be distinguished from rheumatoid arthritis. The initial treatment comprises a prednisolone dose of 15-25 mg/day, followed by a weekly decrease of 1-2.5 mg. Once 10 mg/day has been reached the dose should be reduced more slowly.  相似文献   

11.
OBJECTIVE: To determine the relationship between the antiinflammatory molecule interleukin 10 (IL-10) and disease symptoms, IL-1beta, tumor necrosis factor (TNF), and IL-1 receptor antagonist (IL-1ra) in patients with polymyalgia rheumatica (PMR). METHODS: In 102 patients with PMR, we determined the severity of the disease by the presence of typical clinical symptoms (symptom score with a maximum of 10 points). IL-10, IL-1beta, TNF, and IL-1ra were measured in all patients and 31 age matched healthy controls by enzyme immunometric assays. RESULTS: Compared to patients with elevated serum levels, patients with normal serum levels of IL-10 (below the mean + 3 SD of controls, 7.79 pg/ml) more often had adynamia (p = 0.045), bilateral muscular pain in shoulders, upper arms or neck (p = 0.045), bilateral muscular pain in the pelvic girdle (p < 0.001), headache (p = 0.014), morning stiffness (p < 0.001), symptoms of depression (p = 0.013), and initial weight loss (p = 0.011), and had a higher symptom score (5.5+/-0.4 vs 3.7+/-0.3; p < 0.001). The overall symptom score correlated negatively with IL-10 serum levels (Rrank = -0.421, p < 0.001). IL-10 correlated negatively with IL-1beta (p = 0.013) and TNF-alpha (p = 0.039). The association between elevated serum levels of IL-10 and low serum levels of IL-1beta and TNF was observed only in patients with corticosteroid treatment. In these patients, elevated serum levels of IL-10 were positively associated with an increased ratio of IL-1ra to IL-1beta. CONCLUSION: Elevated serum levels of IL-10 were associated with a more mild form of PMR. This study indicates a favorable role of IL-10 in patients with PMR.  相似文献   

12.
OBJECTIVE: To study fluorodeoxyglucose (FDG) uptake in the different vascular beds and in the large joints of patients with giant cell arteritis (GCA) at diagnosis, during steroid treatment, and at relapse. METHODS: All consecutive patients admitted to our department with a diagnosis of GCA underwent FDG-positron emission tomography (PET) scan before treatment with methylprednisolone was started. PET scans were repeated at 3 and 6 months, if the initial PET scans showed vascular FDG uptake. PET scans were scored at 7 different vascular areas and a total vascular score (TVS) was calculated, ranging from 0 to 21. RESULTS: A total of 35 patients entered the study. At diagnosis, vascular FDG uptake was noted in 29 patients (83%), especially in the subclavian arteries (74%), but also in the aorta (>50%) and up to the femoral arteries (37%). TVS decreased from a mean +/- SD score of 7.9 +/- 5.5 at baseline to 2.4 +/- 3.5 on repeat PET scan at 3 months (P < 0.0005), but did not further decrease at 6 months. The patients who relapsed had similar earlier decreases of TVS compared with those who did not relapse. FDG uptake in the shoulders at diagnosis correlated significantly with the presence of polymyalgia rheumatica (P = 0.005). CONCLUSION: FDG uptake in the large vessels is a sensitive marker for GCA, which can involve the larger thoracic, abdominal, and peripheral arteries. Polymyalgia rheumatica symptoms in patients with GCA correlate with (peri)synovitis of the shoulders. Relapses of GCA cannot be predicted by results of former PET scintigraphies.  相似文献   

13.
Objective. To determine the frequency and clinical characteristics of diffuse distal extremity swelling with pitting edema occurring in polymyalgia rheumatica (PMR). Methods. Clinical features and laboratory findings were recorded for all 245 residents of Olmsted County, Minnesota who developed PMR over a 22-year period (1970–1991). Those who exhibited ≥1 episode of diffuse distal extremity edema with pitting were selected for this study, and were evaluated further. Results. Thirteen women and 6 men in this incidence cohort of PMR had ≥1 episode of distal extremity swelling with pitting edema. Giant cell arteritis was also identified in 5 patients. In 11 patients, the swelling and edema developed concurrently with proximal PMR symptoms. In 2 patients, the distal swelling was the initial manifestation, and in 6 patients, the distal symptoms developed during relapses or recurrences of PMR. Both upper and lower extremities were affected, usually in a symmetric manner. Other peripheral manifestations were also common. The distal swelling and pitting edema responded promptly to corticosteroids, and slowly or incompletely to nonsteroidal anti-inflammatory drugs; a similar response was observed in the proximal symptoms. The distal swelling appeared to represent tenosynovitis and synovitis of regional structures. Conclusion. Distal extremity swelling with pitting edema represents a manifestation of PMR that has not been well described in previous studies. Awareness of this finding will help facilitate the proper diagnosis and institution of appropriate therapy for this disease.  相似文献   

14.
We describe a typical case of a patient with remitting seronegative symmetrical synovitis and pitting edema (RS3PE) 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 RS3PE syndrome. IL-6 (and VEGF), probably produced from the synovial tissues, are considered to be essential factors in the development of RS3PE syndrome.  相似文献   

15.
We report a patient with sarcoidosis who showed edema in the distal portion of all extremities, particularly the legs, as seen in remitting seronegative symmetrical synovitis with pitting edema (RS3PE). Magnetic resonance imaging demonstrated diffuse abnormal intensity in subcutaneous tissues of both legs, and skin biopsy led to a diagnosis of sarcoidosis. Vascular endothelial growth factor (VEGF) showed a high serum level, which decreased soon after starting oral prednisolone, in parallel with an improvement in the limb edema. In this patient VEGF as well as infiltration by sarcoid granuloma in the skin might have played an important role in the pathogenesis of RS3PE-like symptoms in the extremities. When painful pitting edema is seen predominantly in the distal portion of all extremities, sarcoidosis as well as RS3PE should be considered as a possible diagnosis.Abbreviations ACE Angiotensin converting enzyme - RS3PE Remitting seronegative symmetrical synovitis with pitting edema - VEGF Vascular endothelial growth factor  相似文献   

16.
OBJECTIVE: To determine the frequency and clinical characteristics of polymyalgia rheumatica (PMR) with low erythrocyte sedimentation rate (ESR) at diagnosis in a community based cohort of 232 patients. METHODS: A retrospective review of medical records of all the patients with a diagnosis of PMR in Olmsted County, Minnesota, seen and followed over a 22 year period, from 1970 through 1991. RESULTS: Seventeen (7.3%) patients had ESR < 40 mm/h at diagnosis. The findings and outcome in these patients were compared with the others in the group. There was no difference in sex or age between the 2 groups. Both groups had the same delay to diagnosis, typical gradual onset of the disease, the same frequency of both proximal and distal stiffness/pain, and the same frequency of synovitis. Systemic features were less frequent in the low ESR group than in the high ESR group (59 vs 81%, p = 0.05). Mean ESR in the low ESR group was 26+/-9 mm/h versus 74+/-24 mm/h in the high ESR group. The mean hemoglobin concentration was significantly lower (p = 0.0015) in the high compared to the low ESR group (12.2+/-1.4 g/dl versus 13.3+/-1.3 g/dl). The frequency of positive temporal artery biopsy and of diagnosed giant cell arteritis was the same in the 2 groups. Initial response to therapy, frequency of relapses, number of patients going into remission, time to remission, and daily dose of steroids were the same in both groups. CONCLUSION: Other than more frequent systemic symptoms, our population of patients with PMR and low ESR at diagnosis had similar clinical characteristics and course of disease as patients with high ESR at diagnosis.  相似文献   

17.
Abstract

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology characterized by diffuse pain and morning stiffness involving neck, shoulder, and pelvic girdles. To facilitate an understanding of PMR and its proper diagnosis, we evaluated clinical symptoms, laboratory data, and radiographic findings of 32 Japanese patients with it. Distal musculoskeletal manifestations were more frequently observed than had been thought before (81% of the patients), and peripheral arthritis was most common (75%). The joints most often affected were knees and wrists, and most episodes were presented as bilateral oligo- or polyarthritis. A swelling of hands was observed in 34% of the patients. Using contrast-enhanced fat suppression magnetic resonance imaging (MRI) of the shoulder, we found the evidence of subacromial and subdeltoid bursitis (100%), glenohumeral joint synovitis (93%), and biceps tenosynovitis (57%) in the PMR patients examined. Inflammatory changes in soft tissues around the joint capsule were prominent. By knee MRI, suprapatellar bursitis and joint synovitis were visualized in all cases examined, and extracapsular abnormalities were also prominent in 90% of the patients. Serum matrix metalloproteinase-3, a parameter of synovial inflammation, was significantly increased in PMR patients. Anticyclic citrullinated peptide antibody was useful for differential diagnosis between PMR and elderly onset rheumatoid arthritis. In conclusion, joint and periarticular synovitis seems to be commonly and primarily responsible for the proximal and distal musculoskeletal symptoms of PMR. The presence of the extracapsular change, probably a nonspecific extension of synovitis, can explain the severe discomfort that radiates toward the periphery. To avoid making a wrong diagnosis, we should be aware that peripheral synovitis is one of the hallmarks of PMR.  相似文献   

18.
A follow-up study of 56 patients with the provisional diagnosisof polymyalgia rheumatica showed that 12 developed peripheralsynovitis during the course of the illness. Five of the 12 werenoted to have synovitis on presentation which was characteristicallymild, pauci-articular and cleared quickly after commencing prednisolonetherapy. The remaining seven had persistent synovitis of peripheral smalljoints despite an initial brisk response to oral prednisolone.Five of these patients were found to satisfy the ARA criteriafor rheumatoid arthritis after a mean follow-up period of 3.5years. It is suggested that the persistence of peripheral synovitisin patients treated as polymyalgia rheumatica should alert theclinician to the possible development of rheumatoid arthritis. KEY WORDS: Polymyalgia rheumatica, Rheumatoid arthritis, Prednisolone, Peripheral synovitis  相似文献   

19.
Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology characterized by diffuse pain and morning stiffness involving neck, shoulder, and pelvic girdles. To facilitate an understanding of PMR and its proper diagnosis, we evaluated clinical symptoms, laboratory data, and radiographic findings of 32 Japanese patients with it. Distal musculoskeletal manifestations were more frequently observed than had been thought before (81% of the patients), and peripheral arthritis was most common (75%). The joints most often affected were knees and wrists, and most episodes were presented as bilateral oligo- or polyarthritis. A swelling of hands was observed in 34% of the patients. Using contrast-enhanced fat suppression magnetic resonance imaging (MRI) of the shoulder, we found the evidence of subacromial and subdeltoid bursitis (100%), glenohumeral joint synovitis (93%), and biceps tenosynovitis (57%) in the PMR patients examined. Inflammatory changes in soft tissues around the joint capsule were prominent. By knee MRI, suprapatellar bursitis and joint synovitis were visualized in all cases examined, and extracapsular abnormalities were also prominent in 90% of the patients. Serum matrix metalloproteinase-3, a parameter of synovial inflammation, was significantly increased in PMR patients. Anticyclic citrullinated peptide antibody was useful for differential diagnosis between PMR and elderly onset rheumatoid arthritis. In conclusion, joint and periarticular synovitis seems to be commonly and primarily responsible for the proximal and distal musculoskeletal symptoms of PMR. The presence of the extracapsular change, probably a nonspecific extension of synovitis, can explain the severe discomfort that radiates toward the periphery. To avoid making a wrong diagnosis, we should be aware that peripheral synovitis is one of the hallmarks of PMR.  相似文献   

20.
Giant cell arteritis, a large-sized vessel vasculitis, may be associated with musculoskeletal proximal (polymyalgia rheumatica) or distal manifestations. A 68-year-old woman, who had inflammatory pelvic girdle pain, was diagnosed with giant cell arteritis and was successfully treated with corticosteroids. The magnetic resonance imaging and ultrasonography revealed a bilateral bursitis and pelvic girdle enthesopathy. Bursitis is the main anatomic lesion occurring in polymyalgia rheumatica and can be underlined by ultrasonography.  相似文献   

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