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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

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 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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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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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

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

Sarcoidosis with thyroid involvement, polymyalgia rheumatica and breast carcinoma. A case report.

Polymyalgia rheumatica developed in a 71-year-old patient within 2 years of the onset of acute sarcoidosis with biopsy-verified involvement of the thyroid, and concomitant autoimmune thyroiditis with hyperthyroid symptoms. Three years after the onset of muscle symptoms a non-metastasizing breast carcinoma was discovered and treated surgically. Neither the long interval between the onset of polymyalgia rheumatica and the discovery of the breast tumour, nor the good response of muscle symptoms to a one-year maintenance treatment with corticosteroids, was consistent with a paraneoplastic mechanism of the polymyalgia rheumatica syndrome. It was therefore hypothesized that the various disorders suffered by this patient might be related to a partly age-dependent depression of T-lymphocyte function, leading to an altered immunological reactivity to which the various clinical manifestations could be attributed. Such a hypothesis is supported by recent reports showing that in old people and in ageing experimental animals, a decrease in T-lymphocyte function and in the number of circulating T-cells occurs concomitantly with an increase in the incidence of a variety of neoplasms and autoimmune disorders.

Adrenal Cortex Hormones

Polymyalgia rheumatica and temporal arteritis in blacks--clinical features and HLA typing.

Reports of polymyalgia rheumatica and temporal arteritis in blacks are rare. We analyzed five cases of polymyalgia rheumatica and one case of temporal arteritis appearing in blacks. Polymyalgia rheumatica and temporal arteritis in blacks have the same presentation, course, and response to treatment as in Caucasians. A previously unrecorded case of polymyalgia rheumatica and biopsy-proven temporal arteritis in a black had a similar presentation and course as cases in whites. HLA typing of five cases of polymyalgia rheumatica in blacks revealed an increased incidence of AW30 and BW16 in comparison to whites and polymyalgia.

Black or African American

Simultaneous occurrence of polymyalgia rheumatica in a married couple.

The case of a married couple who simultaneously developed polymyalgia rheumatica is presented. Both patients responded to steroid treatment, and when the steroid dose was gradually reduced both patients relapsed. The aetiology and pathogenesis of polymyalgia rheumatica remain unclear; familial aggregation indicates the involvement of genetic factors, whereas conjugal aggregation indicates infectious/environmental agents. In previous reports of polymyalgia rheumatica in married couples, the onset of symptoms in the two patients has usually been separated by a long chronological interval. It is concluded that the present report of simultaneous development of polymyalgia rheumatica in a married couple lends further support to an environmental or contagious aetiology.

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Prognosis of polymyalgia rheumatica and giant cell arteritis.

Polymyalgia rheumatica and giant cell arteritis are amongst the most satisfying conditions for clinicians to diagnose and treat because the unpleasant effects and serious consequences of these conditions can be almost entirely prevented by corticosteroid treatment; the fact that the side-effects of this treatment sometimes seem to be more serious than the complications of the disease is an indication of its effectiveness. Unfortunately, there is no objective way of determining the prognosis in the individual, and decisions concerning duration of treatment remain empirical.

Giant Cell Arteritis

Interleukin-6 in serum of patients with polymyalgia rheumatica and giant cell arteritis.

Polymyalgia rheumatica and giant cell arteritis are diseases which are characterized by a brisk acute phase response. Cytokines, principally interleukin-1 and interleukin-6, are thought to mediate the release of acute phase proteins from hepatocytes. Employing a sensitive bioassay based on the hybridoma growth factor properties of interleukin-6, we investigated its levels in sequential sera obtained from 15 initially untreated patients with PMR/GCA. We found that interleukin-6 activity was significantly elevated in all untreated patients. Although there was a significant decline in its levels after successful steroid therapy, seven patients continued to have raised serum levels of interleukin-6 for up to 6 months after initiation of treatment. Activated endothelial cells are a potent source of interleukin-6 and its persistence in the circulation may be taken as evidence of continuing disease activity. What is not explicable is the normalization of the acute phase response soon after the commencement of steroid therapy when circulating levels of interleukin-6 are still high. This suggests that steroids may be having additional effects on hepatocyte function.

Adult

Polymyalgia rheumatica and rheumatoid arthritis of the elderly: a clinical, laboratory, and scintigraphic comparison.

Clinical, laboratory, and scintigraphic features of 16 patients with polymyalgia rheumatica and 23 patients matched for age presenting with classical or definite rheumatoid arthritis (American Rheumatism Association 1958 criteria) of the elderly were compared in order to define features that might distinguish between these two syndromes. The sensitivity of proposed diagnostic criteria for polymyalgia rheumatica was always higher in the group with polymyalgia rheumatica, though only significantly so for morning stiffness. A comparison of 27 different laboratory features showed few significant differences between the diseases, though correlation between laboratory variables within each of the disease groups differed, perhaps suggesting a fundamental pathogenetic difference between them. Scintigraphy of the shoulder joint proved of no value in differential diagnosis. It was concluded that polymyalgia rheumatica and rheumatoid arthritis of the elderly are probably discrete clinical entities. Bilateral upper arm tenderness, lack of positive rheumatoid factor, and a normal caeruloplasmin are the most valuable features for distinguishing polymyalgia rheumatica from rheumatoid arthritis of the elderly.

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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.

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