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Visual complications of polymyalgia rheumatica (polymyalgia arteritica).

Four case histories are reported in which patients with polymyalgia rheumatica (polymyalgia arteritic) developed evidence of cranial arteritis (in one case two years and in one six months) following withdrawal of steroid therapy after apparent cure. In three cases partial or complete loss of sight has resulted. Steroid therapy should not only be introduced rapidly at appropriate dosage levels as soon as the diagnosis is made but should not be reduced or discontinued prematurely.

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Polymyalgia rheumatica.

Polymyalgia rheumatica is a syndrome that occurs in the elderly and is characterized by pain and stiffness involving the neck, the shoulder girdle, and the hip girdle. The aching should be present for greater than one month. Polymyalgia rheumatica may be more common than reported. The etiology remains unknown. There is generally little found pathologically in this disease. The physical examination is often not impressive. Synovitis may be a main contributing factor to many of the symptoms seen in patients with polymyalgia rheumatica. Symptoms often do not correlate with physical findings. Polymyalgia rheumatica must be differentiated from many conditions since the diagnosis remains entirely clinical. Osteoarthritis, flu syndromes, inflammatory myopathies, fibromyalgia, and depression all have features that may mimic polymyalgia rheumatica. Malignancies and infections may also be difficult to separate from polymyalgia rheumatica. Polymyalgia rheumatica may also be extremely difficult to differentiate from seronegative rheumatoid arthritis in patients older than 50 years. Although some patients with polymyalgia rheumatica have underlying giant cell arteritis, the majority apparently do not. The distinction between polymyalgia rheumatica and giant cell arteritis cannot be made on the basis of laboratory studies and relies solely on clinical symptoms and physical findings. Although nonsteroidal antiinflammatory medications may control symptoms in patients with mild disease, most patients with polymyalgia rheumatica require low-dose corticosteroids. The tapering schedule for the corticosteroids is contingent upon the response of symptoms and laboratory parameters. Polymyalgia rheumatica usually follows a benign course with almost complete response to an adequate treatment program. Recently, there have been several studies suggesting that the course of polymyalgia rheumatica may not be as short and simple as once proposed. Nevertheless, many patients may be completely weaned from corticosteroids. Other agents have been used in this disease, but for the most part their use remains somewhat controversial. Patients must be monitored carefully. Most patients do well, and treatment is effective.

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Relation of giant cell arteritis to polymyalgia rheumatica.

Polymyalgia rheumatica and temporal arteritis appear to be separate syndromes rather than two manifestations of an underlying giant cell arteritis. Polymyalgia rheumatica is a synovitis that may be persistent or recurrent, while temporal arteritis is almost always a single episode; documented recurrences are rare. The two syndromes frequently occur in the same patient although not necessarily at the same time and they may be separated by a long interval. In some patients with polymyalgia rheumatica, giant cell arteritis is found on biopsy of an asymptomatic temporal artery. The frequency of this concurrence is variable in different populations. It is high in Scandinavia, low in Israel and intermediate between these extremes in other populations that have been studied.

Giant Cell Arteritis

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.

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Locked jaw in polymyalgia rheumatica.

Polymyalgia rheumatica is a common disease affecting the elderly population. Symptoms and signs are often nonspecific, including pain and stiffness in the proximal muscles, anorexia, fatigue, depression, weight loss, fever and temporal headaches. We would like to report a case of polymyalgia rheumatica with locked jaw, a feature yet unrecorded.

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Giant cell arteritis-a systemic spectrum including temporal arteritis and polymyalgia rheumatica.

Polymyalgia rheumatica and giant cell arteritis are closely associated disorders that may pose serious threats to the elderly. While the etiological factors remain unknown, these clinical entities may represent different expressions of a common disorder. Recent evidence suggests that autoimmunological involvement of the internal elastic lamina of the large and medium-sized arteries might play an importent role in pathogenesis. Genetic and environmental factors may also contribute. Polymyalgia rheumatica and giant cell arteritis have subtle and protean manifestations and formes frustes occur, contributing to delays in diagnosis unless the clinician holds a high index of suspicion while treating the elderly. The sequelae, including generalized disability, blindness, myocardial and cerebrovascular catastrophies can probably be avoided by timely and prudent use of corticosteroids. These are not uncommon disorders are among the most amenable to treatment of the rheumatological diseases. Family physicians must be diligent in their efforts to diagnose these disorders since early generalized and focal signs and symptoms frequently precede the catastrophic events.

Adrenal Cortex Hormones

Epidural lipomatosis as a cause of spinal cord compression in polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is a common rheumatologic disorder in older individuals that usually requires chronic, low dose corticosteroid therapy for adequate control of symptoms. Epidural lipomatosis is a rare complication of chronic corticosteroid use in which neurologic deficits occur due to extradural fat compression of the spinal cord. The author describes the first reported case of epidural lipomatosis occurring as a complication of corticosteroid therapy in a patient with PMR.

Adrenal Cortex Hormones

Polymyalgia rheumatica.

Polymyalgia rheumatica should be considered when a syndrome of constitutional symptoms, especially weight loss, low-grade fever, weakness, wasting proximal muscles, fatigue, malaise and depression, is seen in the elderly. Giant-cell arteritis plays a part later in the course. Thus the need for biopsy of a long segment of the temporal artery to help in determining diagnosis and therapy. An elevated erythrocyte sedimentation rate (ESR) is an important clue. The usual high value is about 80 mm/hour; if it is over 100 mm/hour, giant-cell arteritis should be suspected. Salicylates, indomethacin, phenylbutazone and hydroxychloroquine produce some clinical improvement but do not lower the high ESR; moreover, the patients are prone to experience relapses. Prednisone, however, not only produces clinical improvement but lowers the high ESR. Potassium p-aminobenzoate may be useful in maintaining the remission.

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Polymyalgia rheumatica and temporal arteritis.

Polymyalgia rheumatica and temporal arteritis are a clinical syndrome and clinicopathologic entity, respectively. Polymyalgia rheumatica occurs more commonly than temporal arteritis, with approximately half of all patients with temporal arteritis having the polymyalgia rheumatica syndrome. Both conditions are found in the population over 50 years of age and are associated with an elevated ESR. The etiology of both is unclear, although genetic, and potentially, environmental factors may play significant roles. Both conditions respond to corticosteroid therapy, but patients with temporal arteritis require significantly higher doses to control symptoms and to prevent blindness.

Adrenal Cortex Hormones

Polymyalgia rheumatica and temporal arteritis.

Polymyalgia rheumatica and temporal arteritis are separate but overlapping rheumatic diseases commonly seen among elderly persons. Polymyalgia rheumatica is characterized by upper body and trunk myalgias/arthralgias and an elevation in erythrocyte sedimentation rate. In about half of patients, temporal arteritis begins after polymyalgia rheumatica. Temporal arteritis is a systemic granulomatous disease that predominantly affects branches of the carotid artery. Claudication of the muscles of mastication and a painful burning tongue may develop during temporal arteritis or be the initial presenting symptoms. Recognition that these orofacial manifestations may be part of the disease process is mandatory because one third of patients with untreated temporal arteritis may go blind.

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[Paraneoplastic polymyalgia rheumatica. Case contribution].

Polymyalgia rheumatica (PMR) is an inflammatory disease which mainly affects the elderly and is highly responsive to steroid therapy. PMR can be associated with giant cell arteritis as well as with malignancy. Three cases of malignant neoplasms of the digestive apparatus beginning with a clinical picture similar to that of the "idiopathic" PMR, but with poor response to steroid therapy are presented. In one case the primary neoplasm was found only at the autopsy. These observations suggest that a careful clinical evaluation and a long follow-up are necessary for a correct diagnosis of "idiopathic" PMR.

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Polymyalgia rheumatica. Clinical features and management.

Polymyalgia rheumatica is a clinical syndrome that occurs almost exclusively in older patients. It is characterized by muscle aching and stiffness and an elevated erythrocyte sedimentation rate. It must be distinguished from other common disorders, particularly polymyositis, fibromyalgia, and other chronic inflammatory or neoplastic diseases. A temporal artery biopsy should be done in patients with confirmed polymyalgia rheumatica and suspected coexistent temporal arteritis, which can have serious manifestations. Patients with polymyalgia rheumatica respond dramatically to low doses of corticosteroids, although prolonged daily treatment often may be needed.

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Polymyositis, not polymyalgia rheumatica.

The distinction between polymyalgia rheumatica and polymyositis is important for treatment and prognosis. Four elderly patients were diagnosed and treated for polymyalgia: raised creatine kinase led to muscle biopsy and a diagnosis of polymyositis.

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Polymyalgia rheumatica.

Early diagnosis of polymyalgia rheumatica is often difficult. A missed diagnosis and inadequate treatment can result in severe and prolonged disability and expose the patient to the hazards of temporal arteritis. Treatment is effective and relatively straightforward provided that good doctor-patient communication is maintained.

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Unusual electromyographic findings in a patient with polymyalgia rheumatica.

A case of polymyalgia rheumatica (PMR) with biopsy-proven giant cell arteritis (GCA) is described in which there were marked abnormalities noted on electromyography (EMG). Spike potentials, positive waves, and fibrillation potentials were seen in the proximal musculature of the upper and lower extremities as well as in the erector spinae muscles. Investigation failed to reveal abnormalities in either creatinine phosphokinase (CPK), aldolase, or muscle obtained at biopsy. The presence of these EMG findings in the PMR-GCA syndrome is unusual. Response to therapy with systemic corticosteroids and normalization of the EMG are documented.

Creatine Kinase

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.

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Immunogenetics of polymyalgia rheumatica.

Evidence suggests that polymyalgia rheumatica (PMR) may be an immune-mediated disease. Therefore, the role of HLA class II genes, the switch region of immunoglobin mu and alpha 1 heavy chain as the T-cell receptor (TcR) genes were investigated by Southern blot analysis. The frequency of DR4 specificity was increased in PMR (67.4% versus 30.3%; P = 0.00017). No association was found with the DQA and DQB genes, the switch region of immunoglobulin mu and alpha 1 heavy chain genes, and the TcR alpha, beta and gamma genes. This study suggests that HLA-DR4 is an important susceptibility factor for PMR. Further studies are needed to elucidate the role of Ig and TcR genes.

Alleles

Hepatitis-B antibody in polymyalgia Rheumatica.

Thirteen patients with polymyalgia rheumatica (P.M.R.) were examined for evidence of viral infection. Hepatitis-B surface antibody (HBsAb) was detected in nine out of twelve patients tested prior to therapy. The antibody persisted up to six months in four patients but reverted to negative in the other five. HBsAb was found in only one of twelve age-matched controls. Hepatitis-B surface antigen was not detected in any patient or control. No significant elevation of antibody titre was detected to a panel of twelve other organisms. Immunoglobulin levels were elevated prior to treatment in several patients. With steroid therapy the IgG and IgA levels fell serially but the IgM levels increased in six patients. These results suggest that hepatitis B is an important trigger for P.M.R. In view of the association with giant-cell arteritis, P.M.R. may represent an abnormal immunological response to infection in elderly patients.

Antibodies, Viral