Inflammatory diseases in older adults: polymyalgia rheumatica

Polymyalgia rheumatica (PMR) is a common inflammatory disease in adults over age 50, and particularly in women. Teasing apart the diagnosis of PMR from other inflammatory and degenerative diseases is mandatory because there are effective treatments for this reversible condition. Onset of PMR is usually acute, with pain typically beginning in the neck and upper arms, though it can begin in the pelvic girdle. Low grade fever may occur; swelling of the joints is not typical, although swelling of the knees may be present on occasion. There are no specific laboratory or radiographic findings although some consider an elevated sedimentation rate (> 50 mmg/hr) to be essential to diagnosis. Differential diagnoses can be myriad. Treatment, typically with prednisone (occasionally methylprednisolone), requires individualized regimens, particularly during tapering.     

Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate

OBJECTIVE: To investigate the inflammatory involvement of shoulder articular and extraarticular structures in polymyalgia rheumatica (PMR) patients with a normal erythrocyte sedimentation rate (ESR) at diagnosis. METHODS: This was a case-control study. All consecutive, untreated new outpatients diagnosed as having PMR with a normal ESR (<40 mm/hour) during a 6-month period were included in the study (case patients). Controls were 12 consecutive, untreated PMR outpatients with an ESR of >40 mm/hour who were observed after the case patients. Before starting corticosteroid therapy, all case patients and controls underwent bilateral shoulder ultrasonography (US) and magnetic resonance imaging (MRI). US and MRI scans were evaluated independently by two radiologists who were blinded to the reciprocal results. RESULTS: Six case patients (4 men and 2 women) and 12 controls (4 men and 8 women) were studied. Both US and MRI demonstrated bilateral subacromial/subdeltoid bursitis in all 6 case patients and in 11 of the 12 (92%) controls (P not significant [NS]). One control had unilateral bursitis. Glenohumeral joint synovitis was found in 4 of 6 case patients (67%) by MRI and in 3 of 6 case patients (50%) by US (P NS), as well as in 8 of 12 controls (67%) by MRI and in 7 of 12 controls (58%) by US (P NS). Both MRI and US detected biceps tenosynovitis in 5 of 6 case patients (83%) and in 8 of 12 controls (67%) (P NS). The severity of bursitis did not differ significantly between the groups. US was as effective as MRI in detecting inflammatory changes of the shoulder. CONCLUSION: MRI and US studies showed that PMR patients with normal or high ESRs have similar inflammatory shoulder lesions. Moreover, bilateral subacromial/subdeltoid bursitis represents the imaging hallmark in PMR patients with a high or normal ESR. MRI or US of the shoulder may facilitate the proper diagnosis in patients with the typical proximal symptoms of PMR who also have normal ESRs.     

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.     

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.     

Corticosteroid requirements in polymyalgia rheumatica

BACKGROUND: Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of unknown cause that affects older individuals. Clinical symptoms respond promptly to corticosteroids, but treatment is often required for several years, frequently resulting in adverse drug effects. Guidelines for the optimal use of corticosteroids that maximize relief of symptoms but minimize adverse effects of the therapy are needed. OBJECTIVE: To determine whether clinical or laboratory parameters in PMR could be identified that allow for stratifying patients into subsets with differences in corticosteroid requirements. PATIENTS AND METHODS: We studied 27 patients with PMR treated with a standardized schedule of prednisone. Patients were reevaluated at monthly intervals for pain scores and physician and patient assessments. Plasma interleukin 6 level and the erythrocyte sedimentation rate were measured at each visit. The duration of steroid therapy and the cumulative steroid dose were calculated. RESULTS: Based on the initial response to therapy and the duration of disease, the 27 patients could be subdivided into 3 distinct groups. Eight with low erythrocyte sedimentation rates responded rapidly and required corticosteroids for less than 1 year with rare disease flares on tapering of prednisone. Twelve others responded well initially but did not tolerate reduction to lower doses and had remitting disease of more than 1 year. Seven patients had only a partial response to the initial steroid regimen. After 4 weeks of therapy, the erythrocyte sedimentation rates improved, but levels of interleukin 6 remained elevated. Pretreatment pain scores were also higher in these partial responder patients (P = .05). CONCLUSIONS: Polymyalgia rheumatica is a heterogeneous disease with variations in the treatment duration and dose of corticosteroids required for suppression of symptoms. Pretreatment erythrocyte sedimentation rate and nonresponsiveness of interleukin 6 to steroid therapy are helpful in dividing patients into subsets with different treatment requirements.     

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.     

MRI findings of the shoulder and hip joint in patients with polymyalgia rheumatica

Abstract

Objectives. The purpose of this study is to evaluate magnetic resonance imaging (MRI) findings of the shoulder and hip joint in patients with polymyalgia rheumatica (PMR).

Methods. MR images of a total of 25 PMR patients (23 shoulders and 6 hips), 43 rheumatoid arthritis (RA) patients (22 shoulders and 22 hips), and 50 control patients (25 shoulders and 25 hips) were examined. The following MRI findings were evaluated: In the shoulder, thickness and abnormalities of the supraspinatus tendon, effusion around the glenohumeral joint, subacromial-subdeltoid bursa, and the biceps tendon; In the hip, effusion around the acetabulofemoral joint, iliopsoas bursa, and trochanteric bursa. Periarticular soft-tissue edema and bone findings were also analyzed.

Results. The supraspinatus tendon was significantly thicker in PMR patients than in RA patients and control patients (p < 0.05). Severe rotator cuff tendinopathy was frequently observed in PMR patients (p = 0.002). The scores for the amount of effusions (joint, bursa, and tendon sheath in the shoulder and bursa in the hip) were much higher in PMR patients (p < 0.05). Periarticular soft tissue edema was detected more frequently in PMR patients than in RA patients and control patients (p < 0.05).

Conclusions. Thick supraspinatus tendon, severe rotator cuff tendinopathy, effusion around the joints, and periarticular soft tissue edema can be good indicators for the diagnosis of PMR.     

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.