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PubMed1999

[The dissecting aortic aneurysm associated with polymyalgia rheumatica: a case report].

We report a patient with a dissecting aortic aneurysm associated with polymyalgia rheumatica (PMR). The patient is a 55-year-old Japanese man without a history of hypertension, diabetes mellitus and syphilis. He was admitted to an emergency hospital because of severe back pain, and was diagnosed as having a dissecting aneurysm of the descending aorta. After the admission, he began to notice severe muscle pain in his bilateral shoulder. Although his back pain gradually improved, his muscle pain progressively worsened, and his lower extremities were also involved. Then, he was introduced to our hospital. On neurological examination, he was alert and oriented. His cranial nerves were all intact. There was no muscle weakness nor sensory disturbance. Laboratory studies revealed that his erhythrocyte sedimentation rate was extremely high without elevation of the serum level of creatine phoshpokinase, rheumatoid factors and c-reactive protein. He was diagnosed as having PMR, and oral administration of prednisolone++ was started. Within several days, his muscle pain dramatically disappeared. As is known, there is a close relationship between PMR and temporal arteritis of giant cell arteritis. In general, PMR is a benign disease and responds well to steroid therapy, and prevalence of the giant cell arteritis is low in Japanese people. However, it should be kept in mind that the dissecting aneurysm is a relevant, severe complication of PMR because arteritis can be latently present in PMR.

Aortic Dissection↗
PubMed2004

Interferon-gamma gene microsatellite polymorphisms in patients with biopsy-proven giant cell arteritis and isolated polymyalgia rheumatica.

OBJECTIVES: Inflammatory cytokines are implicated in the pathogenesis of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). In this study we have examined the potential association of a CA repeat polymorphism in the first intron of the interferon gamma (IFN-gamma) gene with disease susceptibility and clinical expression of these conditions. METHODS: Seventy-nine patients with isolated PMR, 59 biopsy-proven GCA patients and 129 ethnically matched controls from Lugo (NW Spain) were studied. Patients and controls were genotyped by molecular methods for the microsatellite dinucleotide (CA) repeat within the first intron of IFN-gamma gene. RESULTS: No significant differences in the distribution of alleles for the IFN-gamma gene polymorphism between GCA and isolated PMR patients and controls were found. However, the frequency of IFN-gamma allele *4 (128 bp) was reduced in GCA patients (33.1%) compared with isolated PMR patients (46.2%). Also, GCA patients with visual ischemic manifestations exhibited a significantly reduced frequency of IFN-gamma allele *4 compared with those without visual manifestations (17.9% versus 42.5%; p = 0.05 [OR: 0.36, 95% CI: 0.13-1.00]). Moreover, allele *3 (126 bp) was over-represented in the GCA patients with visual ischemic manifestations (71.4% versus 44.4% in the remaining GCA patients; p = 0.01 [OR = 3.13, 95% CI: 1.27-7.68]). CONCLUSIONS: In GCA, IFN-gamma functional polymorphisms are associated with clinical manifestations of severity rather than susceptibility to this vasculitis.

Biopsy↗
PubMed2002

Analysis of the B cell repertoire against autoantigens in patients with giant cell arteritis and polymyalgia rheumatica.

The analysis of the antibody repertoire of patients with giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) might identify target antigens of the autoimmune response with potential relevance to our understanding of the pathogenesis of the disease and to the development of serodiagnostic tests. To detect such antigens, we screened a cDNA library derived from normal human testis for antigens reacting with IgG antibodies in the 1 : 250 diluted sera of three patients with untreated GCA using SEREX, the serological identification of antigens by recombinant cDNA expression cloning. Of 100 000 clones screened with each serum, six, 28 and six clones, respectively, were positive, representing a total of 33 different antigens. Most of the antigens reacted only with the serum used for identification and/or at a similar frequency with normal control sera. However, lamin C and the nuclear antigen of 14 kD reacted specifically with 32% of GCA/PMR, but with none of the control sera, while human cytokeratin 15, mitochondrial cytochrome oxidase subunit II, and a new gene product were detected preferentially, but not exclusively by sera from GCA/PMR patients. We conclude that patients with GCA/PMR develop antibodies against a broad spectrum of human autoantigens. Antibodies against human lamin C, the nuclear autoantigen of 14 kD as well as human cytokeratin 15, mitochondrial cytochrome oxidase subunit II and the product of a new gene should be investigated further to determine their value as tools for the diagnosis and/or the definition of clinical subgroups of patients with GCA/PMR.

Adult↗
PubMed2001

Musculoskeletal manifestations in polymyalgia rheumatica and temporal arteritis.

OBJECTIVE: To evaluate the incidence and characteristics of musculoskeletal manifestations in polymyalgia rheumatica (PMR) and temporal arteritis (TA). METHODS: The records of 163 cases of PMR or TA diagnosed over a 15 year period in one area of Spain were reviewed for the presence and type of musculoskeletal manifestations. RESULTS: Of 163 patients, 90 had isolated PMR and 73 had TA. Eighteen of the 90 patients (20%) with isolated PMR developed distal peripheral arthritis either at diagnosis or during the course of the disease. When it occurred, synovitis was mild, monoarticular or pauci-articular, asymmetrical, transient, and not destructive. Other distal manifestations observed in these patients were carpal tunnel syndrome and distal extremity swelling with pitting oedema. In all cases these manifestations occurred in conjunction with active PMR. As expected, PMR was the most frequent musculoskeletal manifestation in patients with TA, occurring in 56% of cases. On the contrary, only 11% of patients with TA developed peripheral arthritis. An important finding was that peripheral arthritis in these patients appears to be linked only temporally to the presence of simultaneous PMR and is not observed in its absence. Distal extremity swelling or defined polyarthritis were not observed. CONCLUSION: The spectrum of distal musculoskeletal manifestations of PMR in our series is similar to that reported in other populations. By contrast, distal musculoskeletal symptoms are uncommon in TA. The almost complete absence of distal musculoskeletal manifestations in patients with pure TA suggests different mechanisms of disease in PMR and TA, supporting the view of two separate conditions or one common disease in which host susceptibility influences the clinical expression.

Aged↗
PubMed2000

The adrenal steroid status in relation to inflammatory cytokines (interleukin-6 and tumour necrosis factor) in polymyalgia rheumatica.

OBJECTIVES: To determine the correlation between inflammatory cytokines and adrenal hormones in patients with polymyalgia rheumatica (PMR) and to compare the ratio of serum cortisol and androstenedione (ASD) or dehydroepiandrosterone sulphate (DHEAS) in normal subjects with PMR patients. METHODS: In 102 patients with PMR (32 beginning and 70 chronic disease) and 31 age-matched and sex-matched healthy subjects, ASD, cortisol, DHEAS, interleukin-6 (IL-6), and tumour necrosis factor (TNF) were measured by immunometric assays. RESULTS: Serum levels of IL-6 were elevated in patients with PMR as compared with normal subjects (10.0 +/- 1.6 vs 2.1 +/- 0.1 pg/ml, P = 0.01), which was not found for TNF. In PMR patients, serum levels of IL-6 were positively correlated with serum levels of ASD (P < 0.001), cortisol (P < 0.001), and DHEAS (P = 0. 038) irrespective of corticosteroid treatment. Serum levels of cortisol in relation to IL-6 were significantly lower in patients with chronic disease and long-standing corticosteroid administration as compared with patients with recent onset of the disease and without corticosteroid therapy (P < 0.01). CONCLUSIONS: In PMR, as expected, there was an increase in IL-6 serum levels that was associated with elevated serum levels of ASD, DHEAS, and cortisol which was more marked in patients with recent-onset disease and without corticosteroids. However, serum levels of cortisol in patients with and without corticosteroids were lower than expected by considering the inflammatory status (increased IL-6). This may indicate a change in the hypothalamic-pituitary-adrenal (HPA) axis responsiveness to inflammatory stimuli such as IL-6 during chronic disease. Furthermore, there seems to be a shift of biosynthesis to cortisol in relation to DHEAS or ASD in chronic disease.

Adrenal Glands↗
PubMedDate not supplied

Evaluation of the hypothalamic-pituitary-adrenal axis in untreated patients with polymyalgia rheumatica and healthy controls.

OBJECTIVE: To explore the hypothalamic-pituitary-adrenal (HPA) axis in polymyalgia rheumatica (PMR). SUBJECTS AND METHODS: This study was carried out on 13 female patients with PMR who were diagnosed according to the criteria of Chuang et al (Ann Intern Med 1982;97:672-80) and 10 healthy female subjects in the Department of Physical Medicine and Rehabilitation, Erciyes University Medical School. In the patient and control groups, basal cortisol, adrenocorticotrophic hormone (ACTH), 17alpha-hydroxyprogesterone (17-OHP), 11-deoxycortisol (11-S), dehydroepiandrosterone sulfate (DHEAS), androstenedione (A), prolactin (PRL), and thyroid stimulating hormone (TSH) levels were measured. Cortisol, 17-OHP, 11-S and A responses after the low-dose (1 microg) ACTH stimulation test and cortisol and DHEAS responses after the dexamethasone suppression test were detected. We also measured acute phase reactants including C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). RESULTS: Age and sex characteristics were similar in both patient and control groups. The levels of basal hormones including cortisol, ACTH, 17-OHP, 11-S, DHEAS, A, prolactin and TSH and cortisol and DHEAS levels after the low-dose dexamethasone suppression test were not significantly different between the patient and control groups. However, cortisol/CRP and ACTH/CRP ratios were significantly lower in the patient group. Cortisol and DHEAS responses after the low-dose dexamethasone suppression test were not significantly different between the patient and control groups. Cortisol response after the 1 microg ACTH stimulation test was significantly lower in the patients than in the control group, but there were no significant differences in 17-OHP, 11-S and A responses between the patients and controls. Correlation analysis showed that there was a negative correlation between peak cortisol levels after the ACTH stimulation test and disease duration, and also a positive correlation between cortisol levels after the low-dose dexamethasone suppression test and acute phase reactants including CRP and ESR. CONCLUSION: A significant low cortisol response to ACTH stimulation was detected in the patients with PMR. In addition, a negative correlation after the 1 microg ACTH stimulation test between peak cortisol levels and disease duration was detected. These findings may indicate hypoactivation in the HPA axis.

Adrenal Cortex Hormones↗
PubMed1992

[Biopsy of the temporal artery in polymyalgia rheumatica].

Biopsy of the temporal artery is still the most important component in the diagnosis of polymyalgia rheumatica and giant cell arteritis. Although this is a minor surgical intervention, certain vascular complications must be considered. Therefore, preoperative Doppler sonography should be performed. Finally, to prevent false-negative results, the temporal artery should be excised bilaterally and not shorter than 4 cm.

Aged↗
PubMed1989

[Polymyalgia rheumatica with drug eruption--an important differential diagnosis to dermatomyositis].

With regard to certain clinical features, polymyalgia rheumatica (PR) may closely resemble dermatomyositis. In contrast to dermatomyositis, PR usually does not show any cutaneous manifestations, although there might be seen concomitant giant cell arteritis. Furthermore, we do not find muscle enzymes in the serum with PR, and there is no histologic evidence of myositis. In rare cases, however, PR may be associated with cutaneous drug eruption and/or non-specific increase of muscle enzymes, which might cause considerable difficulties regarding the diagnostic differentiation from dermatomyositis.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2000

Utilisation and costs of investigations, and accuracy of diagnosis of polymyalgia rheumatica by family physicians.

To determine the utilisation and costs of investigations, and the accuracy of polymyalgia rheumatica (PMR) by family physicians, a retrospective chart review of 123 patients referred to a tertiary care rheumatology clinic was undertaken. The accuracy of diagnosis of PMR in the cohort was 24%. A variable number of investigations and costs occurred prior to referral, ranging from $110 in those with an unspecified locomotor diagnosis, through $74 in those with a correct diagnosis of PMR, to $24 where no diagnosis was entertained. In general, these costs were considered higher than necessary to make the diagnosis compared to those advocated by rheumatologists. We conclude that there is a low accuracy of diagnosis of PMR and an overutilisation of investigations, with resultant increased costs, by family physicians referring patients with PMR. Appropriate educational interventions are required to address both diagnostic and investigational needs.

Aged↗
PubMed2000

[Risk factors for the development of vision disorders in polymyalgia rheumatica with giant cell arteritis].

BACKGROUND AND OBJECTIVE: Visual disorders, even blindness, are serious complications of polymyalgia rheumatica (PMR) associated with temporal arteritis. Their early recognition in patients at high risk is essential to avoid the development of such visual disorders. It was the aim of this study to identify these risk factors. PATIENTS AND METHODS: Clinical and laboratory data and biopsy findings in 131 patients (94 women, 37 men; mean age 74 years) with PMR and concomitant temporal arteritis were analysed retrospectively. RESULTS: Visual disorders occurred in 61 of the 131 patients. Temporal artery biopsy was not sufficient to detect those at high risk. But this was possible by identifying a typical clinical pattern in that most patients with visual disorders had severe cerebral symptoms, while they had only minor forms of PMR and few generalized symptoms. There was no correlation between any of the laboratory tests and high risk. CONCLUSIONS: A typical pattern of clinical manifestations can provide early identification of those patients who have PMR associated with temporal arteritis and are at high risk of developing visual disorders.

Aged↗
PubMed1995

Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991.

OBJECTIVE: To determine the incidence, prevalence, and survival of polymyalgia rheumatica (PMR) over a 22-year period in Olmsted County, Minnesota. METHODS: Using the unified record system at the Mayo Clinic for the Olmsted County population, we reviewed all medical records with a diagnosis of PMR from 1970 through 1991. RESULTS: There were 245 (173 female; 72 male) incidence cases of PMR between 1970 and 1991. The average annual age- and sex-adjusted incidence of PMR per 100,000 population > or = 50 years was 52.5 (95% confidence interval [CI] 45.9-59.2), with a significantly higher incidence in females (61.7; 95% CI 52.3-71.2) than in males (39.9; 95% CI 30.7-49.2). The incidence varied over the period of observation, but no significant trends were found. The prevalence of PMR among persons > or = 50 years on January 1, 1992 was 6/1,000. There was a small but significantly increased survival rate among male PMR patients compared with the general population. CONCLUSION: Our data demonstrate that PMR is a common nonfatal disease in the elderly, the incidence and clinical manifestations of which have varied but remained relatively stable over the last 2 decades.

Age Distribution↗
PubMed2003

[Serum amyloid A as a useful index of disease activity in polymyalgia rheumatica].

PURPOSE: To clarify the usefulness of measuring serum amyloid A (SAA) levels in patients with polymyalgia rheumatica (PMR), we compared this parameter to C-reactive protein (CRP). SUBJECTS AND METHODS: The study included 10 patients with PMR, who could be prospectively followed up from the start of prednisolone (PSL) treatment until the CRP level decreased to 1 mg/dl or less. When the CRP level decreased, the subjects were divided into the group in which the symptom persisted (n = 6) and those in which the symptom disappeared (n = 4). RESULTS: In the group in which the symptom persisted, both CRP and SAA levels were significantly higher. When the CRP level decreased, there was no significant difference in the CRP level between the two groups. However, the mean SAA level in the group in which the symptom persisted (137.8 micrograms/ml) was significantly higher than that in the group in which the symptom disappeared (21.8 micrograms/ml). On the initial consultation, there was a positive correlation between CRP and SAA (R = 0.77). The SAA level was more sensitive than the CRP level (y = 94.899 x -51.22). When the CRP level decreased, SAA was much more sensitive (y = 222.92 x +6.9121), suggesting the usefulness of SAA after the start of PSL treatment. CONCLUSION: SAA may be a useful parameter of PMR activity.

Aged↗
PubMed2000

Vascular endothelial growth factor production in polymyalgia rheumatica.

OBJECTIVE: To evaluate peripheral production and synovial expression of vascular endothelial growth factor (VEGF) in polymyalgia rheumatica (PMR). METHODS: Circulating levels of VEGF in PMR (serum concentration and in vitro release by peripheral blood mononuclear cells [PBMC]) were investigated by enzyme-linked immunosorbent assay. Local expression of VEGF in shoulder synovial tissue was investigated by immunohistochemical analysis. Investigations were performed in patients with active, untreated disease and in patients treated with corticosteroids. RESULTS: VEGF serum concentrations were significantly higher in untreated PMR patients than in normal control subjects. During steroid treatment, VEGF serum concentrations reached their lowest level after the sixth month of treatment. PBMC isolated from untreated PMR patients spontaneously secreted a higher amount of VEGF compared with PBMC from control subjects. Corticosteroid therapy did not affect the ability of PBMC to produce VEGF. Immunohistochemical staining performed on shoulder synovial tissue showed VEGF expression in both the lining layer and the sublining area. In 3 of 4 treated patients, no VEGF staining was found in synovial tissue during corticosteroid therapy. VEGF expression correlated with vessel density, but was not associated with alphavbeta3 and alphavbeta5 integrin expression. CONCLUSION: Peripheral and local VEGF releases have different responses to steroid treatment in PMR. The lack of response to corticosteroids by peripheral VEGF production supports the hypothesis that systemic involvement is dominant in PMR. At the synovial level, VEGF production is linked to vascular proliferation and is thus directly involved in the pathogenesis of synovitis.

Endothelial Growth Factors↗
PubMed2005

Acute-phase reactants and the risk of relapse/recurrence in polymyalgia rheumatica: a prospective followup study.

OBJECTIVE: To determine laboratory parameters that may be useful in identifying polymyalgia rheumatica (PMR) patients who require long-term corticosteroid therapy. METHODS: A prospective followup study of 94 consecutive untreated patients with PMR were assessed for relapse/recurrence for a mean of 39 months. This cohort represented all the patients diagnosed over a 4-year period in 2 Italian secondary referral centers. Patients were monitored for clinical signs and symptoms, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum interleukin-6 (IL-6). IL-6 levels were also measured in 43 controls matched to the patients for age and sex. RESULTS: The ESR was elevated in 91.5% of the patients prior to therapy initiation, as were CRP in 98.9% and serum IL-6 in 92.6%. Forty-seven (50.0%) patients had at least 1 relapse/recurrence during the followup period and 24 (25.5%) had at least 2. After 4 weeks of prednisone therapy, ESR was elevated in 13.2% patients, CRP in 41.9%, and serum IL-6 in 37.2%. IL-6 levels remained persistently elevated in 9.9% and CRP in 8.7% of patients during the first year of followup, whereas no patient had persistently elevated ESR. Persistently elevated CRP and IL-6 levels were significantly associated with an increased risk of relapse/recurrence. In particular, patients with persistently elevated levels of IL-6 during the first year of therapy had the highest relative risk. CONCLUSION: Despite the control of clinical symptoms, corticosteroids do not adequately control the inflammatory process in a subset of patients with PMR who have persistently elevated levels of CRP and IL-6 and who have a higher risk of relapsing.

Acute-Phase Proteins↗
PubMed2006

Increased 67gallium uptake among polymyalgia rheumatica patients. Is it additional evidence of its vasculitic nature?

Giant cell arteritis/temporal arteritis (GCA/TA) and polymyalgia rheumatica (PMR) are closely related conditions that affect middle age and older patients and frequently occur together. Many authorities consider them to be different phases of the same disease. A study was undertaken of the possible contribution of gallium-67 ((67)Ga) single photon emission tomography (SPECT) scintigraphy to the diagnosis of GCA/TA and PMR, and to support the possible hypothesis of vasculitic nature of PMR. Nine consecutive patients with PMR, and nine consecutive patients with GCA/TA were included in the study. All patients fulfilled the American College of Rheumatology criteria for GCA/TA and Healey's criteria for PMR. The control patients group consisted of those who underwent (67)Ga scintigraphy for febrile illness. All patients and controls underwent (67)Ga SPECT scan of the skull on a dual-head Helix gamma camera 2 days after I.V. injection of 8-10 mCi of (67)Ga citrate. (67)Ga uptake ratio of temporal bone region to bone out of temporal area (TR/Bone) was estimated on transaxial and coronary slices after SPECT scan reconstruction. For each patient and control the calculations of both temporal areas were done. All GCA/TA and PMR patients showed increased uptake in both temporal areas of the skull, while (67)Ga uptake among GCA/TA was the highest, 1.31+/-0.14 in tranaxial view and 1.47+/-0.16 in coronary view. (67)Ga uptake among PMR patients was lower, 1.19+/-0.11 and 1.28+/-0.13, respectively. In comparison, the control patients showed the lowest (67)Ga uptake, 1.04+/-0.09 in transaxial view and 1.11+/-0.06 in coronary view. The results differ significantly between the three groups. It seems that (67)Ga SPECT scan may become a useful tool in the investigation of patients with suspicion of GCA/TA and PMR. Our findings of the increased (67)Ga uptake in the temporal areas among PMR patients add new arguments for vasculitic nature of this disorder.

Aged↗