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PubMed1991

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↗

PubMed1995

Immunofluorescence study of immune complexes in polymyalgia rheumatica.

Two elderly patients with polymyalgia rheumatica (PMR), one with and the other without temporal arteritis (TA), are presented. Immunofluorescence study of muscle biopsy specimens showed IgG, IgA, and fibrinogen deposits in the perifascicular area in the perimysium. This finding suggests that immune complexes play a role in the pathogenesis of this condition and that the pathophysiology of PMR involves an interstitial inflammatory process.

Angiography↗

PubMed1997

[Synchronous variations in the incidence of temporal arteritis and polymyalgia rheumatica in Danish counties. Association with epidemics of Mycoplasma pneumonia infection].

The incidences of temporal arteritis and polymyalgia rheumatica during a twelve year period were studied in different regions of Denmark. Data concerning the incidences of these diagnoses were obtained from two general hospitals from 1982 to 1994 and from the National Patient Register of all diagnoses from all hospitals in 13 of 16 Danish counties from 1982 til 1993. Data from all temporal artery biopsies in two counties were also obtained. Serological epidemiological surveillance data concerning infections causing epidemics in Denmark were obtained from Statens Serum Institut. Data concerning 10,818 patients from 13 counties and 2651 temporal artery biopsies from two counties were analysed. The incidence rate of temporal arteritis in the population aged 50 years and over was 20.4 per 100,000 (95% CI 19-23), and that of polymyalgia rheumatica 41.3 per 100,000 (95% CI 30-67). Significantly higher incidence rates were found in locations with a high population density. The incidence rate of histologically proven temporal arteritis in two counties was 15.1 per 100,000 > 50 years (95% CI 11-20). Pronounced quarterly and annual variations of the incidence were found, with a clustering in five peaks. These cyclic fluctuations were seen simultaneously in several regions. Two periods with an increased incidence of temporal arteritis and polymyalgia rheumatica occurred in close relation to epidemics of Mycoplasma pneumoniae infection. Two peak incidence rates were partly related to epidemics of Parvovirus B19 and one peak to an epidemic of Chlamydia pneumoniae. The synchronous variations in the incidences of temporal arteritis and polymyalgia rheumatica recorded in several regions of Denmark strongly indicate that an environmental infectious factor influences the frequencies. The close concurrence with the above-mentioned epidemics suggests that temporal arteritis and polymyalgia rheumatica may be triggered by certain viral and/or bacterial agents.

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PubMed1990

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↗

PubMed2006

Is the course of steroid-treated polymyalgia rheumatica more severe in women?

Polymyalgia rheumatica (PMR) has a marked preponderance in women. The female sex has been claimed to be a risk factor for longer-course corticosteroid therapy and to be associated with more severe systemic symptoms and lower hemoglobin levels. Eighty consecutive patients affected by PMR, seen at two tertiary referral centers, were followed-up for a mean period of 14.9 months after initiating corticosteroid treatment. At presentation, women had longer disease duration and lower hemoglobin levels (both P = 0.05) than men. In contrast, their systemic signs of PMR were less common (P = 0.01). Women were treated with a slightly higher mean daily dose of prednisone (P = 0.055), and assumed a significantly higher cumulative dosage of the drug (P = 0.01). Accordingly, the mean number of steroid-related side effects was higher among women (P = 0.003). The number of relapses during steroid treatment (P = 0.02), but not that of recurrences, was increased in women. ESR, which was raised at presentation, significantly declined during follow-up to normal values in both subgroups (P < 0.00001 by analysis of variance [ANOVA]). Its decrease was significantly more pronounced in men than in women. Hemoglobin at follow-up was significantly higher in men than in women at any given time point. In conclusion, sex is probably modulating the response to corticosteroids. This finding emphasizes the need to consider differences between males and females in the clinical and therapeutic approach to PMR patients.

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PubMed1987

Polymyalgia rheumatica. Its correct diagnosis and treatment.

Giant cell (temporal) arteritis was first described by Horton and colleagues in 1932, and polymyalgia rheumatica in 1957 by Barber who suggested this title for an entity resembling, but distinct from, rheumatoid arthritis of unknown aetiology in the elderly. Arteritic features were sufficiently common in polymyalgia rheumatica to suggest an arteriopathy as a cause, further evidence of this being the change from the clinical picture picture of polymyalgia rheumatica to giant cell arteritis and vice versa in many patients such that the alternative title polymyalgia arteritica was suggested. The clinical picture of polymyalgia rheumatica is that of an elderly patient, more often female than male, usually over 60 years of age, with painful stiffness in the girdle joints and muscles of the shoulders and hips, but seldom with findings in peripheral or intermediate joints. The painful stiffness in the shoulders, hips and thighs is worse in the early morning. An erythrocyte sedimentation rate over 50mm in 1 hour is usual, and there is a rapid and dramatic response to small doses of corticosteroids (around 10mg prednisolone daily). Arteritic and axial arthritic features have been noted by different authors in different ratios, the disorder gradually abating naturally over periods varying from several months to 7 to 10 years. Deaths, when they occur in this elderly group of patients, have usually been unrelated to the disease or its treatment, but osteoporotic vertebral crush fractures are not uncommon. Partial or complete blindness may occur in patients with either giant cell arteritis or polymyalgia rheumatica, often appearing rapidly after cessation of corticosteroid therapy or rapid reduction of dosage.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones↗

PubMed1996

[Thrombocytosis in polymyalgia rheumatica].

BASIC PROBLEM AND OBJECTIVE OF STUDY: The diagnosis of polymyalgia rheumatica is made difficult by the absence of any pathognomonic findings. Erythrocyte sedimentation rate (ESR) and concentration of C-reactive protein are used as diagnostic criteria and to monitor the disease's course. It was the purpose of this study to evaluate prevalence of thrombocytosis and whether there is any correlation between thrombocytosis and ischaemic complications. PATIENTS AND METHODS: In a retrospective study the data of 23 patients (14 women, 9 men; mean age 67.3 [48-84] years) with polymyalgia rheumatica were analysed: platelet counts, ESR and C-reactive protein had been measured at the time of diagnosis and 7, 14 and 21 days after start of glucocorticoid treatment. At the time of diagnosis 17 of 23 patients (74%) had a thrombocytosis (> 400,000/microliter), at a mean value of 511,000 (418,000-638,000)/microliter. The platelet count decreased more slowly than the inflammatory parameters. On average the platelet count returned to normal on the 14th day. Ischaemic complications developed in three patients (visual disturbances, blindness, cardiac arrhythmias, neurological deficits): all had a high platelet count (462,000-524,000/microliter). CONCLUSIONS: Thrombocytosis can serve as an additional criterion in the diagnosis and follow-up of polymyalgia rheumatica. An association with ischaemic complications is especially likely in the elderly, in case of immobility and if there is evidence of giant-cell arteritis. Treatment with platelet aggregation inhibitors should be considered.

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PubMed1991

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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PubMed1990

Steroid-responsive electromyographic abnormalities in polymyalgia rheumatica.

Prior to definitive diagnosis, electrodiagnostic studies are often requested in patients with polymyalgia rheumatica to evaluate complaints of muscle aching, tenderness, and weakness. These studies are generally normal, although rare reports of electromyographic abnormalities in polymyalgia rheumatica exist. Two patients with polymyalgia rheumatica and electrodiagnostic findings consistent with diffuse denervation are described. Following steroid treatment, both patients experienced impressive clinical and electromyographic improvement. To explain this improvement, we hypothesize a steroid-responsive microvascular arteritis resulting in ischemic damage to axons of motor nerve terminal branches.

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PubMed1996

Temporal arteritis and polymyalgia rheumatica: nosographic and nosologic considerations.

Original reports on temporal arteritis and polymyalgia rheumatica were reviewed before and after the introduction of steroid therapy to prevent blindness in temporal arteritis. In some cases, the original data were reworked. There is evidence that both diseases have become more benign. As a result, the perceived risk of blindness is presently overestimated, as is the perceived benefit of steroids in reducing this risk, and neither should be used as support for an essential difference between temporal arteritis and polymyalgia rheumatica. Indeed, no qualitative differences otherwise exist and both are best viewed as facets of a common disease spectrum with variable risk of adverse outcome. Some of the difficulties in dealing with diseases characterized by variable risk within present dichotomous classifications are discussed. It is likely that in many patients benign disease is presently undiagnosed. It is likely that certain classifications of temporal arteritis and polymyalgia rheumatica now in use lead to an overtreatment of some patients with relatively benign disease.

Arteritis↗

PubMed1975

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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PubMed1992

Polymyalgia rheumatica and temporal arteritis. Related conditions with differing therapies.

Polymyalgia rheumatica and temporal arteritis are entities seen chiefly in older adults. Polymyalgia rheumatica is characterized by muscle and joint aches and an elevated erythrocyte sedimentation rate, and it responds rapidly to low-dose corticosteroid therapy. Temporal arteritis is a vasculitic process, the diagnosis of which must be established by invasive procedures. Higher doses of steroids are necessary to treat it, and the potential for steroid-induced side effects is high.

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PubMed1982

Polymyalgia rheumatica syndrome.

Eleven (14%( of 80 patients with polymyalgia rheumatica syndrome (PMR-S) recovered spontaneously within one year; two of them had received repeated injections of depot steroids before remission occurred. The remaining 69 patients, including 14 with giant cell arteritis (GCA), were all treated with systemic glucocorticoids for an average of 30 months (range 3-81 months). Nine of the patients developed side effects from the treatment, which in seven cases were severe osteoporosis and spinal compression. None suffered from loss of vision. The patients' mortality rate and causes of death did not differ from those of the population at large. The diagnostic criteria are discussed, with the omission of those applied for exclusion. Use of the common term PMR-S is preferred, as GCA and polymyalgia rheumatica (PMR) are clinically indistinguishable. Systemic glucocorticoids are urgently needed when arteritis or ocular manifestations are detected or even suspected. In uncomplicated cases of PMR we recommend that trials be made with repeated injections of depot steroids. This procedure could also be used in patients suffering from severe subjective recurrences after cessation of treatment with systemic glucocorticoids, thereby reducing the number of cases where steroid treatment is unquestionably maintained for too long.

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PubMed1992

Polymyalgia rheumatica and mitochondrial myopathy: clinicopathologic and biochemical studies in five cases.

PURPOSE: The coexistence of mitochondrial myopathy and polymyalgia rheumatica without giant cell arteritis is an interesting association. The frequency of this association was assessed in a prospective study. PATIENTS AND METHODS: Muscle biopsy specimens were obtained from 15 patients with polymyalgia rheumatica. When ragged red fibers (RRF) were observed, histochemical, ultrastructural, and biochemical studies were performed. RESULTS: In five cases, we found the typical appearance of mitochondrial myopathy, with the presence of numerous RRF. Histochemical and biochemical results confirmed these mitochondrial myopathies, showing miscellaneous deficiencies of mitochondrial respiratory chain enzymes. CONCLUSION: Persistence of histologic and biochemical abnormalities after steroid treatment in two patients seems to indicate that a subclinical mitochondrial myopathy preceded polymyalgia rheumatica. How a mitochondrial myopathy could induce or facilitate the emergence of a polymyalgia rheumatica remains unknown.

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PubMed2004

Correlation between 18-fluorodeoxyglucose accumulation in large vessels and serological markers of inflammation in polymyalgia rheumatica: a quantitative PET study.

OBJECTIVE: To quantify 18-fluorodeoxyglucose (FDG) accumulation in large vessels in patients with polymyalgia rheumatica by positron emission tomography (PET), and to compare these data with serological markers of inflammation. METHODS: 13 untreated patients with active polymyalgia rheumatica underwent FDG positron emission tomography; eight were analysed in a second PET when in clinical remission. Six patients with other highly inflammatory conditions served as controls. For quantitative analysis, FDG uptake over nine defined vascular regions, divided by an individual background value, was expressed as a region of interest (ROI) index. These data were compared with the clinical status of the patient and with erythrocyte sedimentation rate (ESR), C reactive protein, haemoglobin, and platelet and leucocyte counts. RESULTS: By visual evaluation, 12 of the 13 patients showed an increased tracer uptake of the aorta or its major branches. By quantitative analysis, FDG uptake was significantly increased in polymyalgia rheumatica. In patients with active disease, the mean ROI index for all vascular regions exceeded that of controls by 70% (mean (SD): 1.58 (0.37) v 0.93 (0.12); p<0.001). In the eight patients who underwent follow up PET, the index declined substantially. In active polymyalgia rheumatica, FDG uptake was significantly correlated with C reactive protein (r = 0.8), ESR (r = 0.79), and platelet counts (r = 0.68). CONCLUSIONS: The observed FDG accumulation in the aorta and its branches and a strong correlation between tracer uptake and markers of inflammation is suggestive of large vessel arteritis. Quantitative ROI analysis appears to be a sensitive tool for detecting such inflammation.

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PubMed1977

HLA antigens in patients with giant cell arteritis and polymyalgia rheumatica.

The frequency of 24 HLA antigens was determined in 43 patients with giant cell arteritis (25 of whom had polymyalgia rheumatica) and 12 others with polymyalgia rheumatica without evident arteritis. No haplotype was present in significantly increased frequency above controls in the total group nor when the patients were separated according to the presence or absence of polymyalgia rheumatica.

Giant Cell Arteritis↗

PubMed1993

Immunopathogenesis, diagnosis, and treatment of giant cell arteritis, temporal arteritis, polymyalgia rheumatica, and Takayasu's arteritis.

Although the causes of giant cell arteritis, polymyalgia rheumatica, and Takayasu's arteritis are not known, the immune system participates in the pathogenesis of these disorders. Many reports indicate that cellular immunity is active in giant cell arteritis. The diagnosis of Takayasu's arteritis has been advanced by magnetic resonance imaging and ultrasonography. Neither technique has replaced angiography as the gold standard for diagnosis, but both may help monitor the response of Takayasu's arteritis to therapy. The diagnosis of giant cell arteritis with a low erythrocyte sedimentation rate is unusual but is more frequent in patients with a history of polymyalgia rheumatica or corticosteroid use. One recent retrospective study suggests that 20 mg/d of prednisolone is as effective as higher doses in treating giant cell arteritis. Other preliminary studies indicate that methotrexate may effectively spare corticosteroid use in giant cell arteritis and Takayasu's arteritis. Angioplasty for renal artery stenosis in Takayasu's arteritis initially cures 30% of patients and improves the condition in 70%; however, the long-term success rate is 50%. New studies demonstrate that patients with giant cell arteritis and polymyalgia rheumatica have an increased risk of hypothyroidism.

Giant Cell Arteritis↗

PubMed1976

Joint imaging in polymyalgia rheumatica.

Technetium pertechnetate joint scintigrams were abnormal in 24 of 25 patients with polymyalgia rheumatica, in all 16 with rheumatoid arthritis, in 4 of 13 with nonarticular rheumatism, but in none of 26 control patients. Abnormal uptake in polymyalgia patients was commonest in shoulders and was less likely to be symmetric than in patients with rheumatoid arthritis, in whom distal joint abnormalities predominated. The pattern of abnormal uptake in polymyalgia rheumatica was not different in those with biopsy-proved giant cell arteritis. Correlation between symptoms and abnormal scintigrams was 72%, and abnormal uptake was present in 81% of joints of patients having physical abnormalities. Biopsy showed lymphocytic synovitis in the knee of one patient. After treatment the number of abnormal joints declined. These findings suggest that synovitis is common in polymyalgia rheumatica, and that it may account for some or most of the symptoms in this condition.

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