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Elderly patients with undiagnosed polymyalgia rheumatica and giant cell arteritis are not uncommon, and they may present to the emergency department with nonspecific physical complaints. We describe the cases of two patients who had the "typical" constellation of symptoms. It is important that emergency physicians consider the diagnosis and provide appropriate testing, therapy, and follow-up.
We report a case of polyarteritis nodosa with a clinical presentation mimicking polymyalgia rheumatica, as well as pathological findings of non-giant-cell arteritis on temporal artery biopsy with symptoms of jaw claudication. Although certain clinical syndromes have been attributed to specific types of systemic vasculitis, considerable overlap occurs. Obtaining tissue biopsy in cases of vasculitis is mandatory for diagnosis and classification.
The minimum incidence of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) was studied during one year in a stable Caucasian population in southern Sweden. The data obtained show an incidence rate higher than that previously found in Sweden and almost as high as the rate recently found in Denmark. In addition, the finding of a low incidence of ocular complications during 3 years of follow-up suggests the positive influence of training primary health care physicians to identify and treat these patients early. Furthermore, the previously described transition from pure PMR into GCA was not seen which might imply a further effect of early treatment.
Sera from patients with giant cell arteritis and/or polymyalgia rheumatica have been found to contain increased levels of circulating immune complexes (IC). Results with the polyethyleneglycol precipitation complement consumption (PEG-CC) assay have been correlated with disease activity. 44% of samples from an active untreated group (21 patients) had increased levels of ICs compared with 23% from an inactive treated group (49 patients). Further analysis of circulating ICs was performed by 125I-Clq binding, the PEG-C4 test, and 125I-conglutinin binding assays. Although we did not find a high correlation between IC levels and disease activity, isolation and analysis of the ICs may lead to further understanding of this disorder.
OBJECTIVES: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common and frequently overlapping diseases. In this manuscript similarities and differences between these conditions have been assessed. METHODS: A retrospective review of the literature was conducted. Reports emphasizing features in common and possible differences are reviewed. RESULTS: GCA and PMR are characterized by late age at disease onset, are more common in women, exhibit evidence of a systemic inflammatory response, and generally respond well to corticosteroids. In biopsy-proven GCA, PMR manifestations are observed in up to 50% of cases. PMR may be the presenting feature in patients who later develop typical cranial manifestations of GCA. However, PMR manifestations may be observed in diverse conditions other than GCA. Patients with isolated PMR are younger than those with PMR associated to biopsy-proven GCA and exhibit milder inflammatory disease as shown by significantly less abnormality in most laboratory findings. Recent observations have shown that the frequency of pathologic features of GCA in temporal artery biopsies of patients with clinically isolated PMR is less than that previously reported. Besides different steroid requirements, GCA is associated with more vascular complications. Genetic differences, in particular different HLA-DRB1 associations, also have been observed. CONCLUSIONS: Polymyalgia manifestations may be observed in patients with biopsy-proven GCA, but isolated PMR may be the only clinical feature or the phenotypic expression of a number of conditions. Clinical features and immunogenetic studies show subtle differences between GCA and PMR.
OBJECTIVE: Evaluation of serum concentrations of chemotactic cytokines (chemokines) in patients with polymyalgia rheumatica at disease diagnosis and during a six-month period of corticosteroid treatment. METHODS: Sera were obtained from patients at the time of PMR diagnosis and subsequently after one week, two weeks, one month, three months and six months. At each time point a clinical and laboratory evaluation was performed. Serum concentrations of IL-8, GRO alpha, RANTES, MCP-1, and MIP1 alpha were evaluated by commercial ELISA kits. RESULTS: Only RANTES serum levels were significantly higher in PMR patients than in controls. No correlation was found between RANTES concentrations and laboratory and clinical features. Chemokine levels reached their lowest level after one month of steroid treatment and remained in the normal range thereafter. CONCLUSIONS: The production of RANTES is enhanced in untreated PMR patients; corticosteroid treatment results in a normalization of chemokine levels.
There has been little basis on which to standardise a diagnosis of polymyalgia rheumatica (PMR), and so 11 rheumatology units in the south and west of Great Britain have collaborated in a study to evaluate possible criteria. Symptoms and laboratory findings claimed to be of diagnostic value in PMR were included in an analysis of the features of 236 patients considered to have unequivocal PMR and 70 patients thought to have possible PMR. The results were compared with similar information from 253 patients with conditions that mimic PMR and from 201 consecutive new presentations to outpatients. The 7 most valuable criteria for differentiation were bilateral shoulder pain or stiffness, onset of illness of less than 2 weeks' duration, initial ESR greater than 40 mm/h, duration of morning stiffness exceeding 1 hour, age 65 years or more, depression and/or weight loss, and bilateral tenderness in the upper arms. We suggest that a patient might be regarded as having probable PMR if any 3 or more of these criteria are fulfilled, or if at least 1 criterion coexists with a clinical or pathological abnormality of the temporal artery. A standardised therapeutic test with prednisolone has value in making the diagnosis of PMR more certain.
OBJECTIVE: To evaluate redox status and muscular mitochondrial abnormalities in patients with polymyalgia rheumatica (PMR). METHODS: Prospective evaluation of deltoid muscle biopsy in 15 patients with PMR. Fifteen subjects matched for age and sex, with histologically normal muscle and without clinical evidence of myopathy, were used as controls. Cryostat sections of muscle were processed for conventional dyes, cytochrome c oxidase (COX), usual histochemical reactions, and Sudan black. A total of 300-800 fibres was examined in each case. Blood lactate, pyruvate, and lactate/pyruvate ratio were determined in all patients. RESULTS: Ragged red fibres were found in eight patients with PMR and accounted for 0-0.5% of fibres. Focal COX deficiency was found in 14 (93%) of 15 patients and in nine (60%) of 15 controls. COX deficient fibres were more common in patients with PMR (range 0-2.5%; mean 0.9%) than in controls (range 0-1.2%; mean 0.3%) (paired t test, p=0.001). Seven (47%) of 15 patients had high blood lactate levels (1.50-2.60 mmol/l) or high blood lactate/pyruvate ratios (22-25). CONCLUSIONS: PMR is associated with mitochondrial abnormalities not solely related to the aging process.
Temporal arterial biopsies from 27 patients with a clinical diagnosis of pure polymyalgia rheumatica (PMR) were examined using light microscopy on paraffin and plastic sections. The primary routine examination of the paraffin-embedded parts of the biopsies (biopsy length: 12.7 +/- 4.5 mm, sub-segments: 4.9 +/- 1.2 mm) revealed 4 positive cases, whereas the primary examination of the smaller plastic-embedded parts showed inflammation in 6 cases (biopsy length: 2.7 +/- 1.2 mm, sub-segments: 0.7 +/- 0.3 mm). Serial sectioning with a 50 microns interval of arteries which were negative primarily revealed three new positive cases in the paraffin-embedded material (total length: 174.0 mm), whereas sectioning the plastic-embedded material (total length: 52.8 mm) produced one more positive artery. All the new cases displayed a focal inflammatory process in atrophic, calcified arterial segments. The high yield of positive biopsies in the present material (11 of 27; 40.7%) demonstrates the diagnostic value of temporal arterial biopsy in PMR and the importance of a careful histologic examination. The results also indicate the influence of biopsy length on the yield of positive biopsies. The division of fixed temporal arterial biopsies into approximately 1-mm-long sub-segments before the embedding and the further serial sectioning of those negative biopsies which are atrophic and/or calcified is recommended in cases of pure PMR.
OBJECTIVE: Sex-specific differences in treatment outcomes have been observed in polymyalgia rheumatica (PMR) and temporal arteritis (TA), with a significantly longer course of treatment in women than in men. We analyzed whether these sex differences are related to differences in disease presentation and severity of the inflammatory response. METHODS: The records of 163 cases of PMR and/or TA diagnosed over a 15 year period were reviewed. A comparative study of clinical and laboratory features between men and women was performed. RESULTS: Of 163 patients, 90 had isolated PMR and 73 had TA. Among patients with TA, 49 women and 24 men were identified, with a ratio of 2. While there were no differences in the frequency of classic disease manifestations, the presence of constitutional syndrome (malaise, anorexia, and weight loss) and fever were significantly more frequent in women than in men. Of note, evaluation of laboratory measures at time of diagnosis also revealed more marked laboratory abnormalities reflecting inflammation in the female group. Among patients with isolated PMR, 58 women and 32 men were identified, a ratio of 1.8. Comparing the clinical features at presentation, significant sex differences were also found, with a higher frequency of constitutional syndrome and lower values of hemoglobin in women. Moreover, women also had higher erythrocyte sedimentation rate values, and higher prevalence of fever and hepatic involvement, although the difference did not reach statistical significance. CONCLUSION: Modest differences were found in disease expression between women and men with TA and/or PMR. In both conditions, the inflammatory response seemed to be more severe in women. The strong inflammatory response in women could explain the longer duration of treatment reported in this subgroup of patients.
OBJECTIVE: To develop a composite score for measurement of disease activity in polymyalgia rheumatica (PMR) and assess its internal and external validity. METHODS: A PMR activity score (AS) was designed and assessed for internal and external validity in two patient cohorts: 57 international patients evaluated primarily for development of the PMR-AS at baseline, weeks 4 and 24; and for validation, 24 Austrian patients assessed at baseline, week 4, and at a mean (SD) point of week 33.6 (24.5). The PMR-AS was calculated as: CRP (mg/dl)+VAS p (0-10)+VAS ph (0-10)+(MST (min)x0.1)+EUL (3-0); Cronbach's alpha was calculated. Factor analysis by linear regression was applied, and responses calculated on the basis of the PMR response criteria and the PMR-AS applied. PMR-AS values at different times were compared by paired t tests. RESULTS: Cronbach's alpha for the composite score was 0.91 and 0.88 in the two cohorts. Factor analysis showed that each single item contributed significantly to the total score and the relative weight of each item in both cohorts was equally distributed. Mean PMR-AS at baseline was 27.54 and 28.72, respectively, at week 4, 5.99 and 8.99, and at the final visit 5.35 and 5.92 (NS). PMR-AS values at baseline and at later visits were significantly different (p<0.0001). PMR-AS values <7 indicated low disease activity, 7-17 medium disease activity, and >17 high PMR activity. In a third control cohort the PMR-AS correlated highly with patient's global assessment, patient satisfaction, and ESR (p<0.001). CONCLUSION: The PMR-AS provides an easily applicable and valid tool for monitoring disease activity, and in combination with the PMR response criteria provides a better description of response.
OBJECTIVE: To describe the patterns of care and direct medical costs of polymyalgia rheumatica (PMR) to test the hypothesis that the direct medical costs incurred by patients with PMR are higher than costs incurred by age- and sex-matched population-based controls from the same community. METHODS: The study population comprised 193 Olmsted County, Minnesota residents who were first diagnosed with PMR between January 1, 1987 and December 31, 1999. Inclusion criteria were as follows: age > or = 50 years; bilateral aching and morning stiffness (lasting > or = 30 minutes) persisting for at least 1 month and involving the neck, shoulders, or hip girdle regions; and an erythrocyte sedimentation rate (ESR) > or = 40 mm/hour. In patients who fulfilled the first 2 criteria, but had a normal ESR, a rapid response to low-dose corticosteroids served as the third criterion. A total of 695 age- and sex-matched subjects without PMR served as control subjects. Billing data from the Olmsted County Healthcare Expenditure and Utilization Database (OCHEUD) were used to provide estimates of nationally representative unit costs in the year 2002 inflation-adjusted dollars. All subjects were followed using the OCHEUD records until December 31, 2002 to assess the total direct medical costs. Generalized quantile regression modeling was used to estimate the effect of PMR on direct medical costs, after adjusting for age, sex, Charlson comorbidity score, number of hospital days, and number of radiographs. RESULTS: During the first year following diagnosis, subjects with PMR used a substantially higher number of outpatient services and laboratory tests compared with controls, but during the subsequent 4 years, there were no differences between the 2 groups. In age- and sex-adjusted analysis, PMR was associated with a significant incremental cost of 2,233 dollars at the 10th percentile of costs and 27,712 dollars at the 90th percentile of costs. However, further adjustments for comorbidities, number of hospital days, radiographs, and imaging eliminated the incremental cost difference between the subjects with PMR and control subjects. PMR subjects were significantly more likely to have a history of myocardial infarction (odds ratio [OR] 1.78, 95% confidence interval [95% CI] 1.13, 2.82), peripheral vascular diseases (OR 2.21, 95% CI 1.37, 3.60), and cerebrovascular diseases (OR 1.60, 95% CI 1.08, 2.39) compared with the controls. CONCLUSION: Incremental direct medical costs associated with the management of PMR can be substantial, especially early in the disease course. These incremental costs appear to originate mainly from comorbid cardiovascular conditions that were shown to be more prevalent among subjects with PMR.
A 64 year old woman complained of aches and stiffness of the neck and the shoulders with fever and E.S.R. increase. A nonsteroid anti-inflammatory treatment was unsuccessful. A clinical examination revealed absence of both radial pulses and presence of murmurs at level of the carotids. The angiographic findings confirmed an aortic arch syndrome with severe stenosis of the subclavian and axillary arteries. The diagnostic approach, in spite of a negativity of the temporal artery biopsy, was for systemic giant cell arteries with general manifestations of polymyalgia rheumatica. The biopsies of both subclavian arteries, performed during a surgery revascularization, showed a typical giant cell arteries in acute stage. The histopathological pattern of extratemporal giant cell arteries obtained by means of a surgical biopsy is really uncommon, being the previous reports performed on necroscopic findings only. In addition this case confirms that polymyalgia rheumatica implies a systemic arteries even if the clinical and histopathological signs of temporal arteritis are lacking. Therefore the temporal artery should be only considered as a particular and inconstant localization of this vasculitis.
Serum specimens from 35 patients with polymyalgia rheumatica and giant cell arteritis (PMR/GCA) were obtained sequentially at variable time intervals up to a year from onset of disease. These were tested for antibodies to intermediate filaments by indirect immunofluorescence using HEp2 cells as substrate. Twenty four of 35 (68%) patients' sera at onset of disease were positive at an anti-intermediate filament antibody (AIFA) titre of greater than 1/40 compared with three outs of 19 (15%) control sera. AIFA were predominantly of IgM class, and there was no significant change in AIFA titres on follow up despite clinical remission of disease.
Thirteen elderly patients with polymyalgia rheumatica (PMR) are presented. The clinical and laboratory findings suggest that many progressive symptoms are due to the non-specific inflammatory changes in various organs of the body, especially in muscles and joints. An immunofluorescence study of muscle biopsy specimens revealed IgG, IgA, and fibrinogen deposits in the perifascicular area of the perimysium. This finding suggests that immune complexes play a role in the pathogenesis of this condition and that the pathophysiology of PMR is an interstitial inflammatory process. We think that the inflammatory findings affecting the interstitial tissue of muscles in the immunofluorescence study are relatively specific to PMR, and will be affected by steroid treatment.
Immunohistochemical studies were performed on muscle biopsy specimens of polymyalgia rheumatica (PMR) to evaluate the extent of muscle and peripheral nerve involvement. The routine histochemistry revealed a mild variation of fiber size, type 2 fiber atrophy and type 2A or 2B fiber deficiency. In 63% of the PMR cases small angular fibers, pyknotic nuclear clumps or target-targetoid fibers were observed, suggesting neurogenic changes, although abnormalities were mild in degree. In immunocytochemical studies, neither major histocompatibility complex (MHC) class 1 nor class 2 products were expressed on the muscle surface membrane of PMR. But regarding intramuscular vessels, MHC class 2 products were distinctly visualized. On serial sections, combined deposits of IgG and Clq in perimysial arteries were seen in 38% of PMR. These results suggest that arteries of small caliber might be involved in immunopathological processes, causing muscle and peripheral nerve damage.
Retrospectively the data were examined of 69 patients with polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA), of whom 62 were treated with corticosteroids. The clinical and laboratory data, and the outcome of temporal artery biopsy were compared. In addition the relation between the course of the disease and survival was investigated. PMR and GCA are closely related syndromes with a multiform clinical presentation. There even is a group of patients with merely systemic symptoms, without specific signs of PMR or GCA. Concerning the course of the disease patients can be divided into groups with a shorter and longer period of disease activity; patients with a smooth stable remission and a chronic disease. The former group shows a significantly better five-year survival. At the time of diagnosis no reliable prediction of the course of the disease is possible.
BACKGROUND: A 73-year-old, previously well woman was admitted to an emergency department because of a 3-month history of severe proximal girdle pain and stiffness with loss of appetite and weight. She was referred to a rheumatologist 10 days after her initial presentation. Within 4 weeks she presented to an outpatient clinic with nausea, vomiting, shortness of breath, painful mouth ulcers, rash on her legs and a further decline in appetite; she was readmitted to hospital. Within 4 months of initial presentation she became jaundiced. INVESTIGATIONS: At initial presentation, physical examination, biochemical, hematological and autoimmune screening, radiography of the pelvis, an abdominal ultrasound, and electromyography were conducted. At referral to a rheumatologist similar tests were repeated. At presentation to the outpatient clinic, hematological and biochemical screening, and a urine dipstick test were conducted. At readmittance to hospital, infectious and autoimmune screening, radiography of the chest, electrocardiogram, ultrasound of the abdomen, and renal biopsy were conducted. At the time of development of jaundice, biochemical and hematological screening, CT of the abdomen and ultrasound-guided biopsy of a pancreatic mass were conducted. DIAGNOSIS: Polymyalgia rheumatica, antineutrophil-cytoplasmic-antibody-positive microscopic polyangiitis with renal involvement and B-cell lymphoma of the head of the pancreas. MANAGEMENT: The patient received oral prednisolone 15 mg daily for polymyalgia rheumatica along with alendronate 70 mg weekly. The patient received intravenous cyclophosphamide 500 mg and methylprednisolone 500 mg every 2 weeks for her microscopic polyangiitis with renal involvement. For B-cell lymphoma of the head of the pancreas, the patient received cyclophosphamide, doxorubicin, vincristine and prednisolone once monthly.