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PubMed2000

The spectrum of conditions mimicking polymyalgia rheumatica in Northwestern Spain.

OBJECTIVE: To examine the spectrum and the main clinical data of patients presenting with polymyalgia symptoms who have conditions other than polymyalgia rheumatica (PMR) or PMR associated with giant cell arteritis (GCA) during a 10 year period in Northwestern Spain. METHODS: Clinical records of patients presenting with polymyalgia symptoms diagnosed at the Hospital Xeral-Calde Lugo from 1987 to 1996 were reviewed by rheumatology staff members. Patients were considered as having a condition suggestive of PMR if they met the following criteria: (1) Age > or =50 years at the onset of symptoms; (2) severe bilateral pain associated with morning stiffness for > 1 mo in at least 2 of 3 areas: neck, shoulder, and/or pelvic girdle; (3) erythrocyte sedimentation rate at the time of diagnosis > or =40 mm/h. Patients with pure PMR or with PMR associated with GCA were excluded from study. RESULTS: Twenty-three of the 208 patients (age 67.8 +/- 9.0 yrs) presenting with PMR symptoms were finally diagnosed as having conditions different from PMR and GCA. Men outnumbered women (61%). Malignancies and rheumatic diseases, especially seronegative symmetrical polyarthritis (SSP), were the most common entities. Elderly patients with solid malignancies had a poor response to low doses of prednisone. In patients with hematologic malignancies atypical symptoms of PMR such as lack of accentuation of symptoms with movement and a more diffuse continuous aching were observed. During followup 5 patients developed episodes of SSP (median duration 13 months, range 5 to 24), particularly in both hands, satisfying the American College of Rheumatology 1987 criteria for rheumatoid arthritis. However, arthritis responded promptly to corticosteroids with no disease progression. No cortical erosions or new episodes of PMR were seen in these patients after a followup of 6.8 +/- 2.6 years. With the exception of these 5 patients, duration of polymyalgia symptoms was not longer than 3 months from the onset of polymyalgia symptoms until a specific diagnosis was made. CONCLUSION: Polymyalgia symptoms are not uncommon as presenting manifestations of a wide spectrum of conditions. Special concern about the presence of diseases different from PMR or GCA must be considered in patients presenting with atypical symptoms of PMR. Also, the possibility of developing a SSP has to be considered during the followup of these patients.

Diagnosis, Differential↗
PubMed1994

Primary systemic amyloidosis presenting as giant cell arteritis and polymyalgia rheumatica.

Primary systemic amyloidosis may present with features suggesting a vasculitis, including giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). In this report, we describe the clinical characteristics, temporal artery biopsy findings, and the response of vascular and musculoskeletal symptoms to corticosteroid therapy in 4 patients with primary systemic amyloidosis who presented with manifestations of GCA or PMR.

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PubMed1980

Polymyalgia rheumatica in a husband and wife.

The case histories of an unrelated husband and wife in whom polymyalgia rheumatica developed within one month are described. In the wife, giant-cell arteritis was present. The clinical courses were similar and there was a typical good response to treatment with prednisone. Both patients were HLA-B8. Only the husband had positive hepatitis-Bs antibody. The aetiology of the disease is discussed in relation to tissue antigens and hepatitis-B infection.

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PubMed1996

The use of methotrexate in polymyalgia rheumatica.

OBJECTIVE: To examine the efficacy and steroid sparing potential of methotrexate (MTX) in the treatment of polymyalgia rheumatica (PMR) without giant cell arteritis. METHODS: 43 patients with PMR were treated with MTX 7.5 mg/wk for 3 months. This dose was raised to 10 and finally 12.5 mg/wk MTX if the desired response was not obtained. RESULTS: No patient benefitted clinically or statistically from treatment with MTX. CONCLUSION: MTX is of no benefit in the treatment of PMR.

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PubMed1989

Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months.

Thirty nine patients with polymyalgia rheumatica (PMR) and 35 with giant cell arteritis (GCA) were treated with high or low dose steroid regimens in a prospective study of the first two months of treatment. Patients with PMR needed 15-20 mg prednisolone initially; 13/20 (65%) relapsed on an initial dose of 10 mg/day. All but two patients with GCA were successfully treated with 40 mg/day prednisolone initially but relapsed on a reduction to 20 mg/day. One patient with GCA receiving 30 mg/day relapsed after four weeks. Six patients with PMR developed GCA during the first two months and required an increased prednisolone dose to control symptoms. The erythrocyte sedimentation rate or C reactive protein did not predict relapse.

Blood Sedimentation↗
PubMed1983

Polymyalgia rheumatica and corticosteroids: how much for how long?

In a prospective study of 176 patients in whom polymyalgia rheumatica (PMR) or giant cell arteritis (GCA) had been diagnosed between 1968 and 1980 the effect of corticosteroid treatment was studied. In those with PMR alone an initial regimen of 10 mg prednisolone daily and for the majority of those with GCA 20 mg daily were adequate to control symptoms. No patient suffered a serious disease complication after starting treatment. Regular follow-up enabled the minimum effective corticosteroid dose to be used. Complications of treatment were infrequent. Corticosteroid treatment has been withdrawn from 72 patients after a mean of 31 months treatment (range 3-103 months). Thirty subsequently relapsed, all within 21 months of withdrawal. No clinical feature predicted those who were more likely to relapse. No rigid treatment schedule should be used in these diseases.

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PubMed1988

Pulmonary function of nonsmoking patients with giant cell arteritis and/or polymyalgia rheumatica; a controlled study.

The pulmonary function of 26 nonsmoking patients with giant cell arteritis (GCA) and/or polymyalgia rheumatica (PMR) was evaluated and the results were compared with those of 28 age and sex matched nonsmoking controls. Normal function was observed in 30.8% of the patients and 50% of the controls (p = non significant). Isolated small airways disease (SAD) was detected in a high percentage of the patients (46.2%), but not significantly different from that of the controls (50%). Its clinical significance remains to be answered. Obstructive, restrictive and diffuse interstitial lung disease (DILD) was seen in one, three and two patients respectively, but was absent in the control population. All the patients had a normal chest radiograph and were asymptomatic, except for three. Two of them had normal respiratory function and one DILD. It is speculated that DILD and restrictive disease, seen only in patients with biopsy proven GCA, may be related to the underlying process.

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PubMed1995

Clinical value of measuring soluble interleukin-2 receptor in polymyalgia rheumatica.

Serum levels of soluble interleukin-2 receptor (sIL-2R) were measured in 15 patients with polymyalgia rheumatica (PMR) and followed prospectively for 11.5 months (range 2-22) and also in 22 patients with PMR treated with corticosteroids for a mean period of 47 months. The controls consisted of 21 patients in the same age range as the PMR patients, admitted to the hospital for various diseases, and of 40 healthy younger subjects. The initial sIL-2R values in the patients with newly diagnosed, untreated PMR were significantly higher than the values seen in the same patients after corticosteroid treatment for a median of 6 months and in PMR patients on corticosteroid treatment for more than a year (p < 0.005 and p < 0.05, respectively). However, while being significantly higher than the values in the normal controls, the sIL-2R values in PMR did not differ from the values seen in the hospital controls. Thus, this assay seems to be of little clinical value in the management of patients with PMR.

Adrenal Cortex Hormones↗
PubMed2005

A comparison of the sensitivity of diagnostic criteria for polymyalgia rheumatica.

OBJECTIVE: To compare the performance of the several different diagnostic criteria sets currently in use for polymyalgia rheumatica (PMR). METHODS: 213 patients attending eight rheumatological centres in eight different European countries were studied. All had recently been referred and were considered by the senior investigator at each centre, selected because of their experience in treatment of PMR, to have this condition. By use of a standard international proforma, the requisite diagnostic points in each criteria set were sought. Sensitivity for each criterion from each set was then calculated, as well as the sensitivity of each criteria set as a whole. RESULTS: Of four criteria sets compared, the Bird (1979) criteria performed best with a sensitivity of 99.5%, and the Hunder (1982) criteria second best, with sensitivity of 93.3%. These both performed significantly better than the two other criteria sets, though each of these was admittedly developed for rather specialised reasons. CONCLUSIONS: Although this study compares homogeneity, we suggest the Bird 1979 or Hunder 1982 criteria should be used whenever possible. Studies that have used alternative criteria may have less sensitivity in diagnosis.

Adult↗
PubMed2001

DRB1 alleles in polymyalgia rheumatica and rheumatoid arthritis in southern France.

To investigate the association of HLA-DRB1 alleles with polymyalgia rheumatica (PMR) and rheumatoid arthritis (RA), 55 patients with PMR without giant cell arteritis, 203 patients with RA and 230 controls, all from the European population of Marseille, were HLA-DRB1 genotyped by PCR-SSO. HLA-DRB1*01 was significantly increased in both the PMR and RA groups compared to controls (35% versus 17%, P(c) < 0.05, and 41% versus 17%, P(c) < 0.001, respectively). HLA-DRB1*04 was significantly increased in the RA group compared to controls (48% versus 23%, P(c) < 0.001) but not in the PMR group. HLA-DRB1*04 subtype frequencies were significantly different between PMR patients and RA patients. Shared epitope-positive HLA-DRB1*04 alleles (DRB1*0401, 0404, 0405, 0408) were significantly overrepresented in RA patients compared to PMR patients and shared epitope-negative HLA-DRB1*04 alleles were overrepresented in PMR patients compared to RA patients. In conclusion, in the Mediterranean population studied, HLA-DRB1*01 is associated with RA and PMR whereas HLA-DRB1*04 is associated with RA only.

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PubMed1981

Polymyalgia rheumatica and giant cell arteritis--rational diagnosis and treatment predicated and disordered prostaglandin metabolism.

We suggest that polymyalgia rheumatica with giant cell arteritis (PR-GCA) is an arachidonic acid metabolites mediated disease which can be diagnosed more accurately and monitored more precisely for therapeutic benefits by the serial determinations of the major urinary prostaglandin F, serum urinary lysozymes, serum acid phosphatase, and serum angiotensin converting enzyme rather than by the erythrocyte sedimentation rate, and, when necessary by temporal artery biopsy. The pathogenetic role proposed for prostaglandins (PG) and, even more precisely perhaps, the leukotrienes in this disease is consistent with the several published clinical observations that non-steroidal anti-inflammatory drug treatment produces in some cases a therapeutic paradox of symptomatic relief with concurrent, if clinically silent, progression of the arteritis, even to blindness. Furthermore, the impressive response of PR-GCA to low maintenance dose steroid therapy, a clinical conundrum for decades, is rationally explained on the basis of depressed or obstructed PG metabolism early on in the metabolic cascade. These views warrant clinical evaluation, confirmation or correction in whole or in part, and may increase our understanding of PR-GCA.

Acid Phosphatase↗
PubMed2004

Are polymyalgia rheumatica and giant cell arteritis the same disease?

OBJECTIVE: To summarize the evidence about the relationship between polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). METHODS: Review of relevant articles from the English-language literature. RESULTS: Epidemiologic studies suggest that PMR and GCA are closely related conditions affecting people over 50 years and frequently occurring in the same patient. PMR symptoms have been observed in 40 to 60 percent of GCA clinical series. Also, temporal artery biopsy may yield positive results for GCA in patients with isolated PMR. Conflicting HLA-DRB1 genotype results have been reported, and recent studies have shown that PMR and GCA have different expression of RANTES, TNFalpha microsatellite, and IL-6 promoter genetic polymorphisms. Search for a possible common infectious agent have yielded disappointing results. Although parvovirus B19 DNA is present in the artery wall of patients with GCA, this virus may be only an innocent bystander. Cytokine studies on a limited number of temporal artery biopsy specimens have shown that interferon-gamma is produced in GCA and not in PMR, suggesting that this cytokine may be crucial to the development of overt vasculitis. CONCLUSIONS: PMR and GCA frequently occur together but no definitive conclusions can be drawn about the nature of this association.

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PubMed2002

[A patient with polymyalgia rheumatica who exhibited fever as the main symptom for a long period].

We encountered a patient with polymyalgia rheumatica (PMR) who exhibited fever as the main symptom for a long period without muscular pain. As an etiological factor, the condition may have been associated with nonsteroidal anti-inflammatory drugs (NSAIDs). A 71-year-old man consulted our Department of Orthopedics for fever and lumbar pain, which initially developed in early September 2000. Administration of NSAIDs resulted in the disappearance of lumbar pain. However, fever persisted. The C-reactive protein (CRP) level was persistently high. Therefore, on October 5, 2000, the patient was referred to our department. At the outpatient clinic, a detailed examination was performed. However, the etiology could not be determined. Repeated administration of NSAIDs resulted in pyretolysis, and the dose of NSAIDs was decreased from January 31, 2001. Severe fever appeared again, and inflammatory reaction also exacerbated. On March 11, 2001, muscular pain involving the bilateral shoulders and forearms suddenly developed. For diagnostic treatment, administration of prednisolone (PSL) at 10 mg/day was started. Muscular pain rapidly disappeared. According to Bird's criteria, PMR was diagnosed. After the dose of PSL was decreased to 7.5 mg/day, the course is good. PMR should be considered as the etiology of idiopathic fever in elderly patients.

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PubMed1995

Polymyalgia rheumatica and temporal arteritis: a retrospective study of 111 patients.

There is no unanimity as to whether polymyalgia rheumatica (PMR) and temporal arteritis (TA) are two distinct diseases or different features of one disease. The objective of this study was to assess the value of histological findings of temporal artery biopsy and the efficacy and complications of drug therapy as well as the frequency of malignancies. It was carried out as a retrospective follow-up study. One hundred eleven patients (89 PMR, 14 TA and 8 PMR+TA) were studied. In 56 patients with PMR a temporal artery biopsy was performed; in none of these biopsies was active arteritis found. Of the 19 patients with TA or PMR+TA, where a temporal artery biopsy was performed, arteritis was found in 15 patients. Reactivation occurred in 27 patients: 4 patients using NSAIDs and 23 patients using corticosteroids. Side effect of the medication included vertebral compression in 10 patients, most of whom were using corticosteroids. Malignancies were diagnosed in 12 of the 111 patients. Most malignancies were diagnosed long before or after the diagnosis of PMR. In case of a PMR diagnosed by the clinician a biopsy of the temporal artery has no value, while the yield of this diagnostic procedure is high in TA. Reactivation was seen quite often and warrants a prolonged period of medical treatment.

Adrenal Cortex Hormones↗
PubMed1994

Serum soluble CD4 and CD8 levels in polymyalgia rheumatica.

OBJECTIVE: Soluble CD4 (sCD4) and sCD8 were measured in the sera of 19 patients with active polymyalgia rheumatica (PMR). METHODS: We correlated the results obtained with lymphocyte subpopulations, soluble interleukin 2 receptors (sIL-2R), and clinical and laboratory variables at diagnosis. In addition 15 patients were prospectively studied during a 6 month period of prednisone therapy. Assays of the sCD4 and sCD8 molecules and of the sIL-2R were performed using an enzyme-linked immunosorbent kit. RESULTS: Serum sCD8 and sIL-2R levels were significantly elevated in patients with active disease compared to normal controls, while serum sCD4 and the relative percentage of CD8+ T cell levels decreased. In the 15 patients prospectively studied sCD8 levels fell significantly after 1 week of therapy along with the remission of clinical disease and normalization of erythrocyte sedimentation rate. At the end of the study period, sCD8 values did not differ from normal controls and they were significantly reduced compared to baseline values. CD8+ lymphopenia persisted at the end of the study. sCD4 levels remained significantly lower during the study period. sIL-2R levels fell significantly at the end of the study period. However, the 6-month levels of sIL-2R remained significantly higher compared to controls. CONCLUSION: The rise of serum sCD8 levels observed in patients with PMR with active disease suggests an early activation of CD8 T cells. The therapeutic effect of steroid in PMR may be partially mediated by its effect on CD8 activated cells.

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PubMed2006

The role of anticyclic citrullinated peptide antibodies in the differential diagnosis of elderly-onset rheumatoid arthritis and polymyalgia rheumatica.

There are clinical difficulties to differentiate elderly-onset rheumatoid arthritis (EORA) patients from those with polymyalgia rheumatica (PMR), especially when dealing with EORA-like PMR-onset, seronegative EORA, and PMR with peripheral synovitis, which constitute the subgroups presenting the greatest difficulties. Serum samples were obtained from two groups of patients, one with EORA diagnosis and another with a PMR diagnosis. Anticyclic citrullinated peptide (anti-CCP) antibodies (enzyme-linked immunosorbent assay method) and rheumatoid factor (RF; latex technique) were determined. Of the 16 EORA patients, 9 presented anti-CCP antibodies, 4 of whom tested positive for RF. Of the 12 EORA patients who remained negative to RF, 5 were positive for anti-CCP antibodies. Eight of the EORA patients started with polymyalgic symptoms. Three of these patients showed positive titles of anti-CCP antibodies with negative RF. All PMR patients presented negative anti-CCP antibodies, except one with weak positive titles, and all were negative for RF. Of 15 patients with PMR, 7 presented oligoarticular synovitis at the onset. After a mean follow-up of 3 months, two patients developed RA. When evaluating them for RF and anti-CCP antibodies, one tested negative, while the other was positive for both antibodies. We observed a tendency to higher values of anti-CCP antibodies in patients with extraarticular manifestations, radiological damage, and disease-modifying antirheumatic drugs. When compared to the PMR group, EORA patients presented positive anticitrulline antibodies at the beginning of the disease in a statistically significant amount. One third of the seronegative EORA patients presented positive anti-CCP antibodies at the onset.

Age of Onset↗
PubMed1999

Polymyalgia rheumatica with low erythrocyte sedimentation rate at diagnosis.

OBJECTIVE: To determine the frequency and clinical characteristics of polymyalgia rheumatica (PMR) with low erythrocyte sedimentation rate (ESR) at diagnosis in a community based cohort of 232 patients. METHODS: A retrospective review of medical records of all the patients with a diagnosis of PMR in Olmsted County, Minnesota, seen and followed over a 22 year period, from 1970 through 1991. RESULTS: Seventeen (7.3%) patients had ESR < 40 mm/h at diagnosis. The findings and outcome in these patients were compared with the others in the group. There was no difference in sex or age between the 2 groups. Both groups had the same delay to diagnosis, typical gradual onset of the disease, the same frequency of both proximal and distal stiffness/pain, and the same frequency of synovitis. Systemic features were less frequent in the low ESR group than in the high ESR group (59 vs 81%, p = 0.05). Mean ESR in the low ESR group was 26+/-9 mm/h versus 74+/-24 mm/h in the high ESR group. The mean hemoglobin concentration was significantly lower (p = 0.0015) in the high compared to the low ESR group (12.2+/-1.4 g/dl versus 13.3+/-1.3 g/dl). The frequency of positive temporal artery biopsy and of diagnosed giant cell arteritis was the same in the 2 groups. Initial response to therapy, frequency of relapses, number of patients going into remission, time to remission, and daily dose of steroids were the same in both groups. CONCLUSION: Other than more frequent systemic symptoms, our population of patients with PMR and low ESR at diagnosis had similar clinical characteristics and course of disease as patients with high ESR at diagnosis.

Adrenal Cortex Hormones↗
PubMed2000

Association of giant cell arteritis and polymyalgia rheumatica with different tumor necrosis factor microsatellite polymorphisms.

OBJECTIVE: To determine whether giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are associated with different tumor necrosis factor (TNF) microsatellite polymorphisms. METHODS: Typing of TNF microsatellite polymorphisms was carried out by molecular-based techniques on DNA obtained from a population sample of residents from Lugo, northwestern Spain. A case-control approach was used to compare 136 patients with GCA and/or PMR with 147 ethnically matched controls. The association of disease with TNF microsatellite polymorphisms was investigated using chi-square tests and multivariate logistic regression analyses. RESULTS: Different TNF microsatellite associations were found with GCA and PMR. In patients with isolated GCA, the primary association was with TNFa2, which was independent of the GCA associations with HLA-DRB1*0401 and *0101. A negative association was found with TNFa10. In patients with isolated PMR, there was a positive association with TNFb3. This was found to be independent of the HLA-DRB1*13/*14 association in isolated PMR. TNFd4 was negatively associated with isolated PMR. Forward stepwise logistic regression analyses indicated that the strongest association with GCA was provided by the TNFa2 allele, although DRB1*0401 and *0101 were still associated. PMR was primarily associated with TNFb3. A direct comparison of TNF allele frequencies between isolated GCA and isolated PMR indicated that the main difference between these conditions occurred in the frequency of TNFa10. CONCLUSION: GCA and PMR in individuals from northwestern Spain are associated with different TNF microsatellite polymorphisms. The primary TNF associations (TNFa2 and TNFb3) appear to influence susceptibility to these conditions independent of any HLA-DRB1 association.

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