Mortality associated with polymyalgia rheumatica in the United States in the 1999-2020 period: a multiple-cause-of-death study.

OBJECTIVE: Multicause-of-death methods were used to analyze mortality and leading causes of death associated with polymyalgia rheumatica (PMR) in the United States from 1999 to 2020. MATERIALS AND METHODS: We analyzed mortality data from the Centers for Disease Control and Prevention (CDC) Data analysis system and selected death certificates that listed PMR as the cause of death based on the International Statistical Classification of Diseases and Related Health Problems (ICD-10) category code. Relevant mortality rates, number of deaths and historical trends were analyzed. The number of PMR-related deaths and age-standardized mortality rate (ASMR) trend charts were made using Excel 2010 version and trend lines were added. RESULTS: Over the last 22 years, the total number of PMR-related deaths in the United States was 15,421 women (89.8%), a ratio of about 1:9 men to women. When PMR is listed as the underlying cause of death, the ASMR for women and men (per 100,000 people) is approximately 1.8-5.1:1, and when it is listed as the non-underlying cause of death, it is 1.8-3.3:1. PMR deaths are more frequent in individuals aged 70 years and above, with patients aged 80 years and above being most affected. Among different ethnicities, the highest number of deaths was found in Caucasians, followed by Black or African American. When it comes to causes of death, heart disease still ranks first, followed by cancer. In addition, we also found that when PMR combined with malignant tumors as a multiple cause of death, the number of female deaths was higher than that of male deaths, the overall number of deaths of both showed an upward trend, and the overall ASMR of both showed a downward trend. CONCLUSIONS: In the past 22 years, we have observed a low mortality rate of PMR in the United States. However, for patients with PMR, especially elderly women, medical workers should be vigilant and pay attention to whether they are combined with other complications, such as malignant neoplasms, and make timely diagnosis and treatment to further reduce the mortality rate of patients with PMR.

Mortality Among Patients With Polymyalgia Rheumatica: A Retrospective Cohort Study.

OBJECTIVE: To determine whether a diagnosis of polymyalgia rheumatica (PMR) is associated with premature mortality. METHODS: We extracted anonymized electronic medical records of patients ages >40 years who were eligible for linkage with the Office for National Statistics Death Registration data set, from the Clinical Practice Research Datalink from 1990 to 2016. Patients with PMR were individually matched, by age, sex, and registered general practice, with up to 5 controls without PMR. The total number and proportion of deaths and mortality rates were calculated. The mortality rate ratio (MRR) with 95% confidence interval (95% CI), adjusted for age, sex, region, smoking status, body mass index, and alcohol consumption, was calculated using Poisson regression. The 20 most common causes of death were tabulated. RESULTS: A total of 18,943 patients with PMR were matched to 87,801 controls. The mean ± SD follow-up after date of diagnosis was 8.0 ± 4.4 years in patients with PMR and 7.9 ± 4.6 years in controls. PMR was not associated with an increase in the risk of death (adjusted MRR 1.00 [95% CI 0.97-1.03]) compared to matched controls. Causes of death were broadly similar between patients with PMR and controls, although patients with PMR were slightly more likely to have a vascular cause of death recorded (24% versus 23%). CONCLUSION: A diagnosis of PMR does not appear to increase the risk of premature death. Minor variations in causes of death were observed, but overall this study is reassuring for patients with PMR and for clinicians.

Familial aggregation of longevity in giant cell arteritis and polymyalgia rheumatica.

The long-term mortality in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) is unexpectedly decreased or at least not increased regardless of several mortality risk factors that these diseases share with other chronic immune-mediated rheumatic diseases. The genetic and immunological profile of PMR/GCA patients is unique, therefore, the hypothesis that this profile provides some survival advantage to PMR/GCA patients should be considered. The longevity is a phenomenon that was demonstrated to be familial. The familial aggregation of longevity can be studied by analysis of life expectancy in family members. Here we test the hypothesis of the aggregation of an increased longevity in the families of PMR/GCA patients. We compared the age of death of 358 parents of 179 PMR and GCA patients with corresponding data retrieved from 506 parents of 253 randomly collected age and sex-matched controls. The number of nonagenarian (≥ 90-year -old) mothers of PMR/GCA patients was significantly higher (OR = 2.34, 95%CI 1.11-11.95, p < 0.0005) vs controls. Both nonagenarian parents were found in 6 patients (3.35%) and none in the control cohort (OR = 8.77, 95%CI 2.26-405.10, p = 0.003). Our data suggest the familial aggregation of nonagenarians in PMR/GCA patients.

High Relapse Rate in Patients with Polymyalgia Rheumatica despite the Combination of Immunosuppressants and Prednisolone: A Single Center Experience of 89 patients.

Polymyalgia rheumatica (PMR) is an inflammatory disorder in the elderly and is characterized by pain in the shoulders and lower back. Previous studies from western countries have shown that relapse is frequent; however, there are only a few reports on the relapse rate in Japan. Here we examined the relapse rate, and sought to identify factors that predict recurrence in patients with PMR. Of 110 patients who fulfilled the Bird's criteria for PMR between May 2011 and June 2019, 21 patients were excluded, and the remaining 89 patients were followed up until July 2019. Relapse was defined when clinical symptoms were exacerbated and serum C-reactive protein level increased. The relapse-free survival curves were plotted using the Kaplan-Meier method, and log-rank test was used for statistical analysis. The mean age of the 89 patients (50 males and 39 females) was 71.8 years. The mean dose of initial prednisolone (PSL) was 11.8 mg/day. The 1-, 3-, and 5-year relapse-free survival rates were 81.6%, 58.0%, and 52.3% (N = 59, 21, and 7), respectively. In patients who experienced recurrence, the 1- and 3-year second relapse-free survival rates were 58.3% and 27.3% (N = 18 and 3), respectively. Immunosuppressants, such as methotrexate and tacrolimus, were added to PSL in 19 of 30 patients who experienced relapse at the discretion of the attending physicians; however, none of the immunosuppressants worked for preventing second relapses and had steroid-sparing effects. These results indicate that effective immunosuppressants are required to suppress relapse in the treatment of PMR.

Epidemiology of Polymyalgia Rheumatica 2000-2014 and Examination of Incidence and Survival Trends Over 45 Years: A Population-Based Study.

OBJECTIVE: To determine time trends in the incidence and survival of polymyalgia rheumatica (PMR) over a 15-year period in Olmsted County, Minnesota, and to examine trends in incidence of PMR in the population by comparing this time period to a previous incidence cohort from the same population base. METHODS: All cases of incident PMR among Olmsted County, Minnesota residents in 2000-2014 were identified to extend the previous 1970-1999 cohort. Detailed review of all individual medical records was performed. Incidence rates were age- and sex-adjusted to the US white 2010 population. Survival rates were compared with the expected rates in the population of Minnesota. RESULTS: There were 377 incident cases of PMR during the 15-year study period. Of these, 64% were female and the mean age at incidence was 74.1 years. The overall age- and sex-adjusted annual incidence of PMR was 63.9 (95% confidence interval [95% CI] 57.4-70.4) per 100,000 population ages ≥50 years. Incidence rates increased with age in both sexes, but incidence fell after age 80 years. There was a slight increase in incidence of PMR in the recent time period compared to 1970-1999 (P = 0.063). Mortality among individuals with PMR was not significantly worse than that expected in the general population (standardized mortality ratio 0.70 [95% CI 0.57-0.85]). CONCLUSION: The incidence of PMR has increased slightly in the past 15 years compared to previous decades. Survivorship in patients with PMR is not worse than in the general population.

Associations between polymyalgia rheumatica and giant cell arteritis and 12 cardiovascular diseases.

OBJECTIVES: Evidence of the association of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) with the full range of cardiovascular diseases (CVDs) is limited. We examined their relationship with the first clinical presentation of the 12 most common CVDs in an unselected population-based cohort of men and women. METHODS: We analysed CArdiovascular disease research using LInked Bespoke studies and Electronic health Records (CALIBER) data, which links primary care and hospital and mortality data in England, from 1997 to 2010. We assembled a cohort of men and women initially free from CVD at baseline and included all patients with PMR and/or GCA (PMR/GCA) diagnosis, matched by age, sex and general practice with up to 10 individuals without PMR/GCA. Random effects Poisson regression analysis was used to study the association between PMR/GCA and the initial presentation of 12 types of CVDs. RESULTS: The analysis included 9776 patients with PMR only, 1164 with GCA only, 627 with PMR and GCA and 105 504 without either condition. During a median of 3.14 years of follow-up 2787 (24.1%) individuals with PMR/GCA and 21 559 (20.4%) without PMR/GCA developed CVDs. Patients with PMR/GCA had lower rates of unheralded coronary death (3.18 vs 3.61/1000 person-years; adjusted incidence ratio 0.79, 95% CI 0.66 to 0.95), transient ischaemic attack (5.11 vs 5.61/1000 person-years; 0.67, 95% CI 0.54 to 0.84) and coronary and death composite (24.17 vs 25.80/1000 person-years; 0.90, 95% CI 0.82 to 0.98). No associations were observed for other CVDs or cerebrovascular diseases, and in patients with only PMR or GCA. No evidence of interaction by age or sex was found. Estimates decreased with longer PMR/GCA duration and findings were robust to multiple sensitivity analyses. CONCLUSIONS: In this large contemporary population-based cohort the presence of PMR and/or GCA was not associated with an increased risk of CVDs or cerebrovascular diseases regardless of PMR/GCA duration.

[Glucocorticoid-induced adverse events in patients with giant cell arteritis or polymyalgia rheumatica]. / Complications de la corticothérapie chez les patients souffrant de maladie de Horton ou de pseudopolyarthrite rhizomélique.

Giant cell arteritis and polymyalgia rheumatica are common reasons for prescribing long-term glucocorticoid therapy. Glucocorticoids efficacy in these patients is rapid and undeniable, but it is often overshadowed by adverse events, appearing more or less early during treatment course. These adverse events are usually minor but some of them may be life threatening. Because of the absence of randomized, controlled trials comparing glucocorticoids to placebo in this indication and in this aged population, it is difficult to precise the role of corticosteroids in the occurrence of some complications that can also be precipitated by the disease itself (e.g., vascular complications) or related to aging. In this context, analysis of the crude morbidity and mortality can help in anticipating the potential problems that these patients will encounter. In addition, the long duration of glucocorticoid exposure should prompt physicians to be particularly vigilant regarding some adverse events that are minimally symptomatic on the short term but have a major long-term impact.

Association between polymyalgia rheumatica and vascular disease: a systematic review.

OBJECTIVE: To systematically identify and appraise the existing literature on the association between polymyalgia rheumatica (PMR) and vascular disease. METHODS: The Medline, Embase, CINAHL, and Cochrane databases were searched from inception to September 2010. Search criteria included observational studies containing patients with isolated PMR reporting on a vascular outcome. Data were extracted and articles were assessed for quality. RESULTS: The database search identified 545 articles. Eight articles reporting on 7 unique studies were included in the final review. Four studies reported on vascular mortality and 3 studies reported on other vascular outcomes. Four of the studies reported statistically significant positive associations between PMR and vascular disease and 3 studies reported no statistically significant results. The heterogeneity of the results was investigated; the strongest explanatory variable was that 2 of 3 studies that reported nonsignificant results were prospective, whereas all of those studies reporting significant results were retrospective. CONCLUSION: There is some evidence to suggest that, like other inflammatory rheumatic disorders, PMR may be linked to an increased risk of vascular disease. Further studies are required to quantify the level of risk to determine whether patients with PMR should be actively screened for cardiovascular disease and its risk factors.

Causes of death in polymyalgia rheumatica. A prospective longitudinal study of 315 cases and matched population controls.

OBJECTIVE: To determine causes of death in patients with pure polymyalgia rheumatica (PMR) compared to matched population controls. METHODS: In a population based study from 1987-1997, 315 patients were diagnosed with PMR. The patients were each randomly assigned four population controls, totally 1,260 controls. The date and causes of death were identified from the data files at Statistics Norway up to the end of 1997. RESULTS: A total of 65 cases (20.6%) with PMR died compared to 338 (26.8%) among the controls (mortality rate ratio (MRR) = 0.73, 95% CI 0.56-0.97, p = 0.03). No statistically significant difference was found between patients and controls with regard to mortality from coronary heart disease or stroke (MRR=0.78, 95% CI 0.52-1.18), cancer (MRR = 0.59, 95% CI 0.30-1.17), and other causes (MRR=0.75, 95% CI 0.48-1.17). CONCLUSION: The increased survival found in patients with PMR could not be explained by reduction in any particular cause of death.

Trends in the incidence of polymyalgia rheumatica over a 30 year period in Olmsted County, Minnesota, USA.

OBJECTIVE: To determine time trends in the incidence and survival of polymyalgia rheumatica (PMR) over a 30 year period in Olmsted County, Minnesota, USA. METHODS: Using the unified medical record linkage system of the Rochester Epidemiology Project, we identified all incident cases of PMR among residents of Olmsted County, MN, between January 1, 1970, and December 31, 1999. Incidence rates were estimated and age- and sex-adjusted to the 1990 US white population. The annual incidence rates were graphically illustrated using a 3 year centered moving average. A Poisson regression model was used to evaluate predictors of PMR incidence. Survival rates were computed and compared with the expected rates in the population. RESULTS: There were 378 incident cases of PMR during the 30 year study period. Of these 66.6% were female and the mean age at incidence was 72.8 years. The overall age and sex adjusted annual incidence of PMR per 100,000 population aged > or = 50 years was 58.7 (95% CI 52.8,64.7). Incidence rates increased with age in both sexes, but in women, unlike in men, incidence fell after age 80. The incidence rates varied over the period of observation, but no significant trends over time were found. In the multivariable analysis, sex (p = 0.023), age (p < 0.001), and age2 (p < 0.001), but not calendar year (p = 0.24) were significant predictors of incidence. Survival among individuals with PMR was not significantly different from that expected in the population (p = 0.06). CONCLUSION: The incidence of PMR has remained relatively stable over the past 30 years.

Increased mortality due to cardiovascular disease in patients with giant cell arteritis in northern Sweden.

OBJECTIVE: To study the cause of death pattern in patients with giant cell arteritis (GCA) or polymyalgia rheumatica (PMR), and to analyze the effect of the disease, or its therapy, on the risk of a cardiovascular event (CVE). METHODS: Patients with biopsy proven GCA or with PMR, whose condition was diagnosed between 1973 and 1979, were followed until December 31, 1995. The standardized mortality ratio (SMR) was estimated using data for the population of Västerbotten, Northern Sweden, as reference value. Information for sex, age at diagnosis, erythrocyte sedimentation rate (ESR) at diagnosis, corticosteroid therapy, comorbidity from diagnosis, and date and cause of death was collected. RESULTS: A total of 136 patients with GCA and 35 with PMR were identified. At the time of followup 114 patients with GCA and 25 with PMR were deceased. The overall mortality was significantly increased in the female patients, SMR = 133 (95% CI 110-162). Death due to cardiovascular disease (CVD) was significantly increased in both women and men, SMR = 149 (95% CI 118-189) and 158 (95% CI 112-224), respectively, and mainly due to ischemic heart disease. An excess mortality was found in women with the highest ESR, the highest prescribed dose of prednisolone at diagnosis, or a daily prednisolone dose of 10 mg or more one year after diagnosis. In multiple Cox regression analysis, male sex and hypertension significantly increased the risk of a CVE. CONCLUSION: Death due to CVD was increased in patients with GCA. Increased mortality was related to either the corticosteroid therapy itself or insufficient control of inflammation.

Survival in polymyalgia rheumatica and temporal arteritis: a study of 398 cases and matched population controls.

OBJECTIVE: To estimate survival in polymyalgia rheumatica (PMR) and temporal arteritis (TA). METHODS: The present study encompassed 338 incident cases who were diagnosed at the Department of Rheumatology during the period 1987-1997 and 60 cases diagnosed in the same period but admitted to hospital for reasons other than PMR or TA. The 398 patients were each assigned four age- and sex-matched controls from the same population and mortality ascertained. RESULTS: Among the 338 incident cases, there were 69 deaths compared with 360 deaths among their 1352 controls. The mortality was thus 28% lower in cases than in controls [relative risk (RR)=0.72, 95% confidence interval (CI) 0.55-0.95]. The 274 incident cases with pure PMR had increased survival compared with controls (RR=0.70, 95% CI 0.52-0.95), whilst among the 64 incident TA patients and their controls, no difference in mortality was found (RR=1.2, 95% CI 0.55-2.74). Patients diagnosed at other departments and their controls had the same mortality. In the incident cases, the mean initial dose of prednisolone, the mean maintenance dose of prednisolone, the mean initial erythrocyte sedimentation rate and C-reactive protein and frequency of peripheral arthritis did not differ between survivors and those dying during the observation period. CONCLUSION: The study showed increased survival in patients with PMR compared with controls, whilst mortality in TA equalled that of controls. There was no association between use of corticosteroids and level of disease activity and death. The increased survival in PMR might be explained by improved medical surveillance.

Epidemiology and mortality in 220 patients with polymyalgia rheumatica.

Between 1985 and 1987 a total of 521 people underwent temporal artery biopsy with no histological evidence of arteritis in Göteborg, Sweden. Two-hundred-and-twenty people were diagnosed as having polymyalgia rheumatica (PMR). Among the patients without PMR 30% had rheumatic, 17% malignant and 14% infectious disorders. The annual incidence of PMR with negative biopsy was 17/100,000 and for the population over 50 yr it was 50/100,000. We found an increased mortality in vascular diseases among men with PMR in the first 2 yr after diagnosis with 13 observed deaths compared to the expected six (P < 0.01). There was also a tendency toward an increased mortality among the women with 16 observed deaths compared to the expected 11 (not statistically significant). The mortality in malignant diseases was not increased.

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.

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.

Excess mortality in giant cell arteritis.

A 13-year departmental sample of 34 patients with definite (biopsy-verified) giant cell arteritis (GCA) was reviewed. The mortality of this material was compared to sex-, age- and time-specific death rates in the Danish population. The standardized mortality ratio (SMR) was 1.8 (95% confidence limits, 1.1-2.8). During the same period 146 patients with probable (not biopsied, but clinically diagnosed in the department) GCA and 85 cases of possible (diagnosed and treated before admission) GCA had been admitted to the department. Those two groups did not differ from the biopsy-verified group with respect to SMR, sex distribution or age. In the group of patients with department-diagnosed GCA (definite + probable = 180 patients), the 95% confidence interval for the SMR of the women included 1.0. In all other subgroups there was a significant excess mortality. Excess mortality has been found in two of seven previous studies on survival in GCA. The prevailing opinion that steroid-treated GCA does not affect the life expectancy of patients is probably not correct.

High incidence of polymyalgia rheumatica and giant cell arteritis in a Swedish community.

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.

Long-term survival in giant cell arteritis including temporal arteritis and polymyalgia rheumatica. A follow-up study of 90 patients treated with corticosteroids.

Ninety patients with giant cell arteritis (GCA) were followed for 9-16 years after diagnosis. Corticosteroids were given to all but one patient. Forty-two patients died during the observation period (27 women and 15 men). The survival was compared with that in the general population and found not to be reduced among the GCA patients. In fact, we found a lower mortality than expected after five years from diagnosis (p less than 0.05). We did not see any increase in deaths due to vascular or malignant diseases in our patients.