风湿性多肌痛和巨细胞动脉炎

风湿性多肌痛(polymyalgiarheumatica,PMR)是一种以全身疼痛和僵硬伴血沉明显增快为特征的特殊的风湿状态,首发在颈部、肩部、臀部和骨盆带的近端肌群。巨细胞动脉炎(gaintcellarteritis,GCA)或颞动脉炎(temporalarteritis,TA)是一种大中血管的血管炎,常累及起源于主动脉弓的颅支。随着年龄的增长,老年人的非特异性骨骼肌肉疼痛和疲劳常与PMR/GCA相关。一、流行病学在高纬度地区发病率高,不同种族发病有差异。PMR发病年龄在50岁以上,发病率约为600/10万,70～80岁是发病高峰,男女比率为1:1.8。GCA/TA的发病率较PMR低,50岁以上发病…     

Update on polymyalgia rheumatica

Polymyalgia rheumatica is an inflammatory disease of unknown etiology affecting individuals aged fifty years and older, mainly of Caucasian ethnicity. Polymyalgia rheumatica is associated with giant cell arteritis more frequently than expected by chance alone. In both conditions, females are affected two to three times more often than males. The clinical hallmark manifestations of polymyalgia rheumatica are aching and morning stiffness in the shoulder girdle and often in the pelvic girdle and neck. Serum inflammatory markers are typically elevated, while the most consistent abnormal finding on imaging studies is bursitis in the symptomatic areas. A dramatic response to glucocorticoids is characteristic of polymyalgia rheumatica. Many patients are able to discontinue glucocorticoids six months to two years after the onset of clinical symptoms, but some patients may require longstanding glucocorticoid treatment. Glucocorticoid-sparing agents may be helpful in patients with chronic relapsing courses and those at high risk of glucocorticoid-related adverse events.     

Acute poststreptococcal polymyalgia.

Three patients developed severe incapacitating diffuse pain and tenderness of the skeletal muscles after acute streptococcal infection. There was no evidence of concomitant arthritis, glomerulonephritis, or inflammatory muscle disease in any of the cases. All patients responded promptly to anti-inflammatory therapy. Severe myalgia should be considered an additional complication of immunologically mediated poststreptococcal response.     

Current therapy of polymyalgia rheumatica

Polymyalgia rheumatica is characterized by muscular pain and stiffness developing almost exclusively in individuals older than 50 years. Most likely, survival is similar to that of the general population, and perceivably neither malignant diseases nor cardiovascular disorders occurs more frequently than expected. Thus, the main aim of treatment is symptomatic relief and the benefit of such interventions should always be weighed against the possible risks of drug induced side effects. The review addresses the therapeutic options in polymyalgia rheumatica, and focuses on oral corticosteroids, intramuscular and intravenous methylprednisolone, deflazacort, and methotrexate.     

Atypical presentations of polymyalgia rheumatica

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.     

Recent advances in polymyalgia rheumatica

Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder of unknown cause characterised by the subacute onset of shoulder and pelvic girdle pain, and early morning stiffness in men and women over the age of 50 years. Due to the lack of a gold standard investigation, diagnosis is based on a clinical construct and laboratory evidence of inflammation. Heterogeneity in the clinical presentation and disease course of PMR has long been recognised. Aside from the evolution of alternative diagnoses, such as late‐onset rheumatoid arthritis, concomitant giant cell arteritis is also recognised in 16–21% of cases. In 2012, revised classification criteria were released by the European League Against Rheumatism and American College of Rheumatology in order to identify a more homogeneous population upon which future studies could be based. In this article, we aim to provide an updated perspective on the pathogenesis and diagnosis of PMR, with particular focus on imaging modalities, such as ultrasound and whole body positron emission tomography/computed tomography, which have advanced our current understanding of this disease. Future treatment directions, based on recognition of the key cytokines involved in PMR, will also be explored.     

Breast arteritis in polymyalgia rheumatica

A 79-year-old woman with the clinical presentation of polymyalgia rheumatica (PMR) also had breast cancer. Histopathologic evidence of arteritis was found in mastectomy tissue and in a temporal artery, neither of which had produced symptoms before surgery. Previously reported cases are reviewed in the context that breast vasculitis may be more common than in generally recognized in patients with PMR.     

Synovitis in polymyalgia rheumatica.

Synovitis was observed in 13 out of 88 consecutive patients with polymyalgia rheumatica. It is described in detail in five patients, on the basis of clinical radiological observations, joint aspiration, arthroscopy and biopsy. The synovitis of polymyalgia rheumatica cannot be distinguished histologically or at arthroscopy from the appearance seen in mild rheumatoid arthritis. Clinically, however, the synovitis of polymyalgia rheumatica is mild, transient and confined to one or two joints or tendon sheaths. It is not followed by joint deformity or by radiological erosive changes in the bone ends. It may occur at any stage of the disease but particularly at its onset, or when the dose of corticosteroid treatment is reduced.     

The spectrum of polymyalgia rheumatica

Polymyalgia rheumatica and temporal arteritis are common syndromes of unknown cause that afflict older patients, the great majority of whom are white. Polymyalgia, which is seen more frequently, is a benign synovitis and can be differentiated from rheumatoid arthritis by the distribution of inflamed joints and by its rapid and complete response to small doses of prednisone. Temporal or giant cell arteritis occurs in approximately 20 per cent of patients with polymyalgia rheumatica and may present with either localized or systemic symptoms. Once the diagnosis is confirmed by temporal artery biopsy, patients should be treated with a large dose of prednisone for at least 1 month. The erythrocyte sedimentation rate is a useful test in suspecting the diagnosis initially, but is a poor gauge to tapering the steroid dose.