scholarly journals Patterns of Survival Among Patients With Myeloproliferative Neoplasms Diagnosed in Sweden From 1973 to 2008: A Population-Based Study

2012 ◽  
Vol 30 (24) ◽  
pp. 2995-3001 ◽  
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Yngvi Kristinsson ◽  
Therese M.-L. Andersson ◽  
Ola Landgren ◽  
Sandra Eloranta ◽  
...  

PurposeReported survival in patients with myeloproliferative neoplasms (MPNs) shows great variation. Patients with primary myelofibrosis (PMF) have substantially reduced life expectancy, whereas patients with polycythemia vera (PV) and essential thrombocythemia (ET) have moderately reduced survival in most, but not all, studies. We conducted a large population-based study to establish patterns of survival in more than 9,000 patients with MPNs.Patients and MethodsWe identified 9,384 patients with MPNs (from the Swedish Cancer Register) diagnosed from 1973 to 2008 (divided into four calendar periods) with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios were computed as measures of survival.ResultsPatient survival was considerably lower in all MPN subtypes compared with expected survival in the general population, reflected in 10-year RSRs of 0.64 (95% CI, 0.62 to 0.67) in patients with PV, 0.68 (95% CI, 0.64 to 0.71) in those with ET, and 0.21 (95% CI, 0.18 to 0.25) in those with PMF. Excess mortality was observed in patients with any MPN subtype during all four calendar periods (P < .001). Survival improved significantly over time (P < .001); however, the improvement was less pronounced after the year 2000 and was confined to patients with PV and ET.ConclusionWe found patients with any MPN subtype to have significantly reduced life expectancy compared with the general population. The improvement over time is most likely explained by better overall clinical management of patients with MPN. The decreased life expectancy even in the most recent calendar period emphasizes the need for new treatment options for these patients.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3071-3071
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Y. Kristinsson ◽  
Therese M-L Andersson ◽  
Sandra Eloranta ◽  
Åsa Rangert Derolf ◽  
...  

Abstract Abstract 3071 Background: Available data on survival patterns among patients diagnosed with myeloproliferative neoplasms (MPN) show a great diversity. For instance, in essential thrombocythemia (ET) there are reports stating that survival is not affected by the disease while other investigators consider ET to be a serious disease that significantly reduces life expectancy. Patients with primary myelofibrosis (PMF) are consistently reported to have a shortened life span while polycythemia vera (PV) is associated with a reduced survival in many, but not all, studies. We conducted a comprehensive, population-based study to assess survival and to define causes of death MPN patients, and to compare patterns to the general population. Patients and Methods: The nationwide Swedish Cancer Registry was used to identify all cases of MPN between 1973 and 2008 with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios (EMRRs) were computed as measures of survival. The Cause of Death Registry was used to obtain information on causes of death both in the patient and the general population. Results: A total of 9,384 MPN patients were identified (PV n=4,389, ET n=2,559, PMF n=1,048 and MPN not otherwise specified (MPN NOS) n=1,288); 47% were males and the median age at diagnosis was 71. The reporting rate to the Cancer Registry increased over time being well above 95% during the most recent calendar period. There was a significant overall excess mortality in all subtypes of MPN, reflected in 5-year and 10-year RSRs of 0.83 (95% CI 0.81–0.84) and 0.64 (0.62-0.67) for PV, 0.80 (0.78-0.82) and 0.68 (0.64-0.71) for ET and 0.39 (0.35-0.43) and 0.21 (0.18-0.25) for PMF, respectively. Higher age at MPN diagnosis was associated with a poorer survival. For example, the 10-year RSR for patients <50 years was 0.86 (0.83-0.88) as compared to 0.35 (0.29-0.43; p<0.001) in those >80 years. Females had a superior survival, EMRR 0.72 (0.66-0.78), compared to males (reference 1.00). Survival of patients with MPN improved significantly over time with an EMRR of 0.60 (0.53-0.67) in 1983–1992, 0.29 (0.25-0.34) in 1993–2000 and 0.23 (0.19-0.27) 2001–2008 using the calendar period 1973–1982 as a reference (1.00). However, MPN patients of all subtypes including PV and ET had an inferior survival compared to the general population during all calendar periods indicating that these patients continue to experience higher mortality. The 10-year RSRs for patients diagnosed 1993–2008 were 0.72 (0.67-0.76) for PV and 0.83 (0.79-0.88) for ET (Figure). The most common causes of death in MPN patients were, in order, hematological malignancy 27.2%, cardiac disease 27.1%, solid tumors 12.4% and vascular events including thromboembolism and bleeding, 9.2%. Four per cent of patients in this cohort were reported to have died due to acute myeloid leukemia. Over time, the proportion of deaths due to cardiac disease and thromboembolic events has decreased. On the other hand, we observed an increasing relative number of deaths due to hematological malignancies during the more recent calendar periods. The relative risks of dying from these causes in relation to the general population will be presented. Summary/conclusion: In this large population based study including over 9,000 MPN patients, we found all MPN subtypes to have a significantly lower life expectancy compared to the general population. Survival improved over time, however patients of all subtypes including ET had an inferior relative survival even in the most recent calendar period. Especially during earlier years, a certain misclassification and under reporting of ET may have contributed to a reduction in survival rates in the ET group. The relative number of deaths due to cardiac disease and thromboembolic events decreased during more recent calendar periods. This, and the improvement in survival might reflect the introduction of better treatment strategies for both the disease itself and for the prevention and treatment of thromboembolic complications of MPNs. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4645-4645
Author(s):  
Avinash G Dinmohamed ◽  
Mirian Brink ◽  
Otto Visser ◽  
Pieter Sonneveld ◽  
Arjan A van de Loosdrecht ◽  
...  

Abstract Background Chronic myelomonocytic leukemia (CMML) is a rare hematological malignancy with features of both myelodysplastic syndromes (MDS) and myeloproliferative neoplasms. Most data on CMML arrive from the few available clinical and epidemiological studies where CMML was often combined with MDS. So far, phase 3 clinical trials and large population-based studies specifically addressing CMML are lacking. We conducted a large nationwide population-based study to assess trends in incidence, primary treatment and survival among CMML patients in the Netherlands from 1989-2012. Methods We selected all patients diagnosed with CMML in 1989-2012 (N = 1,359; median age 75 years; age range 22-95 years; 63% males) from the nationwide population-based Netherlands Cancer Registry (NCR). Patients with juvenile myelomonocytic leukemia were excluded. Despite changes in classification, separate morphology codes for CMML were available in all editions of the International Classification of Diseases for Oncology (ICD-O; 9893, 9868 and 9945 in the first, second and third edition, respectively) and could therefore be identified in the NCR throughout the whole study period. The ICD-O does not have separate codes for CMML-1 or 2. Data on primary treatment, that is, no therapy or only supportive care (NT/SC), chemotherapy (CT) and CT followed by a stem cell transplantation (CT + SCT), were retrieved from the NCR. Patients were categorized into three calendar periods (1989-2000, 2001-2006 and 2007-2012) and four age groups (18-59, 60-69, 70-79 and ≥80 years), unless otherwise stated. Incidence rates were age-standardized to the European standard population and calculated per 100,000 person-years. Relative survival rates (RSRs) were computed as a measure of disease-specific survival. Results The overall age-standardized incidence rate (ASR) of CMML increased from 0.23 per 100,000 in 1989-2000, 0.31 in 2001-2006 to 0.38 in 2007-2012. The annual ASR became stable at around 0.4 per 100,000 since 2008 (Fig 1A). The proportion of patients diagnosed in individuals aged ≥70 years was 70%. The incidence of CMML was higher in men than in women, which was ascribed to the higher incidence among the 70-year-old men compared with the equivalent female group (Fig 1B). The primary treatment of CMML patients remained unchanged during the entire study period. In the overall series, 975 (72%), 365 (27%) and 19 (1%) CMML patients received NT/SC, CT and CT + SCT, respectively. The use of CT + SCT was mainly restricted to patients 18-59 (n = 13) and 60-69 (n = 6) years of age. Survival of CMML patients was poor and did not improve over time as the 5-year RSRs (with 95% confidence interval) were 16% (12%-20%), 20% (15%-25%) and 20% (15%-25%) in the three calendar periods, respectively. As shown in Figure 2, the overall 5-year RSRs for patients in the four age groups were 21% (13%-29%), 23% (18%-29%), 20% (16%-24%) and 12% (7%-18%), respectively. With the limitation of small numbers (n = 19), the overall 5- and 10-year RSRs were 29% (10%-52%) and 30% (10%-53%) for patients undergoing CT + SCT as primary treatment. In other words, the RSR reached a plateau after 5 years since diagnosis. In the most recent period, the 5-year RSR was 73% (25%-95%) for patients undergoing CT + SCT (n = 7). Conclusions In this first large population-based study including almost 1400 CMML patients, we found that the incidence of CMML increased over time until the year 2007. This rise is probably explained by improved case ascertainment and augmented disease awareness, rather than by changes in etiologic factors. Primary treatment remained conservative throughout the study period as treatment options for CMML, which primarily affects the elderly, are very limited. As a consequence, relative survival remained poor and essentially unchanged in both younger and older patients over the past two decades. Therefore, CMML-specific prognostic models should be applied in the diagnostic work-up to evaluate prognosis and plan risk-adapted treatment, and assist in designing clinical trials that specifically assess therapeutic options in CMML patients in order to improve their survival. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 632-632 ◽  
Author(s):  
Malin Hultcrantz ◽  
Therese M-L Andersson ◽  
Ola Landgren ◽  
Asa Rangert Derolf ◽  
Paul W Dickman ◽  
...  

Abstract Background Patients with myeloproliferative neoplasms (MPNs) are reported to have a higher risk of thrombosis compared to the general population. However, the magnitude of the increase in risk in MPNs patients is not known since there is a lack of studies including control subjects. Therefore, we conducted a large population-based study to assess the risk of arterial and venous thrombosis in patients with MPNs in relation to matched controls. Patients and Methods All patients with MPNs reported to the Swedish Cancer Register and/or registered in the Inpatient Register from 1980 to 2009 were included. For each patient, four controls matched for age, sex, and county of residence, were randomly identified from the Register of Total Population. End of follow-up was December 31st 2010. Events of arterial and venous thrombosis, including deaths, were identified from the Inpatient and Outpatient Registers and the Cause of Death Register. Arterial thrombosis was defined as myocardial infarction, ischemic stroke, or peripheral arterial thrombus/embolus. Venous thrombosis was defined as pulmonary embolism, deep venous thrombosis (DVT), liver or splanchnic vein thrombosis, cerebral venous sinus thrombosis, or other venous thrombosis/embolus. Odd ratios (ORs) of arterial and venous thrombosis at diagnosis +/-30 days were calculated using logistic regression. Cox regression and flexible parametric models were used to estimate proportional and non-proportional hazard ratios (HRs) with 95% confidence intervals (CIs). Follow-up in all regression models started 30 days after diagnosis to avoid detection bias at time of diagnosis. Results A total of 11,155 patients and 44,620 matched controls were identified. Forty-six percent (n=5,161) were men and median age at MPN diagnosis was 69 years. The OR for arterial and venous thrombosis at time of MPN diagnosis +/- 30 days was 55.0 (95% CI 51.1-59.2 p<0.001) and 64.3 (42.2-98.1 p<0.001) respectively in MPN patients compared to controls. In MPN patients, the risk of arterial thrombosis was significantly 4.9-fold (4.8-5.0 p<0.001) increased compared to matched controls. The HR of myocardial infarction was 3.9 (3.7-4.1 p<0.001) and stroke 4.9 (4.8-5.0 p<0.001) in MPN patients. The HR of arterial thrombosis in MPN patients decreased shortly after diagnosis but thereafter increased with follow-up time in relation to controls (Figure 1a). There was a similar risk of arterial thrombosis in patients of different MPN subtypes compared to controls, the HR in patients with polycythemia vera (PV) was 5.0 (4.8-5.2), essential thrombocythemia (ET) 4.7 (4.6-5.0), primary myelofibrosis (PMF) 5.0 (4.7-5.3), and MPN-unclassifiable (MPN-U) 5.1 (4.8-5.5), respectively. The HR of venous thrombosis in MPN patients was 6.7 (6.2-7.2 p<0.001), where the HRs of pulmonary embolism was 7.5 (6.6-8.5 p<0.001) and DVT was 5.3 (4.8-5.9 p<0.001) compared to matched controls. MPN patients had a substantially increased risk of liver and splanchnic vein thrombosis, HR=41.4 (26.4-64.9 p<0.001). The risk of venous thrombosis in MPN patients decreased shortly after diagnosis and thereafter remained stable in relation to controls during follow-up time (Figure 1b). Compared to controls, patients with PV had a larger increase in risk of venous thrombosis than the other subtypes (HR=9.0; 8.0-10.1), while the HR of venous thrombosis in patients with ET was 5.4 (4.7-6.2), PMF 6.0 (4.9-7.5), and MPN-U 4.6 (3.7-5.7), respectively. Conclusions Patients with MPNs have an overall five- to sevenfold elevated risk of thrombosis compared to the general population. The highest HRs were seen for venous thrombosis, especially abdominal thrombosis. The odds of having a thrombosis at time of MPN diagnosis were high, indicating that thrombosis is an important first symptom of MPN. The elevated risk of arterial thrombosis increased while the elevated risk of venous thrombosis remained stable during follow-up time. This large population-based study is, to our knowledge, the first to quantify the excess risk of thrombosis in MPN patients compared to the general population. Our results indicate that we need to consider time after diagnosis in risk score models and rethink the strategies for thromboprophylaxis in patients with MPN in order to decrease the risk of thrombotic events. Figure 1A Risk of arterial (a) and venous (b) thrombosis in MPN patients compared to matched controls during follow-up time. Figure 1A. Risk of arterial (a) and venous (b) thrombosis in MPN patients compared to matched controls during follow-up time. Figure 1B Figure 1B. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2012 ◽  
Vol 119 (4) ◽  
pp. 990-996 ◽  
Author(s):  
Jan Sjöberg ◽  
Cat Halthur ◽  
Sigurdur Y. Kristinsson ◽  
Ola Landgren ◽  
Ulla Axdorph Nygell ◽  
...  

Abstract In recent decades, attention has focused on reducing long-term, treatment-related morbidity and mortality in Hodgkin lymphoma (HL). In the present study, we looked for trends in relative survival for all patients diagnosed with HL in Sweden from 1973-2009 (N = 6949; 3985 men and 2964 women; median age, 45 years) and followed up for death until the end of 2010. Patients were categorized into 6 age groups and 5 calendar periods (1973-1979, 1980-1986, 1987-1994, 1994-2000, and 2001-2009). Relative survival improved in all age groups, with the greatest improvement in patients 51-65 years of age (P < .0005). A plateau in relative survival was observed in patients below 65 years of age during the last calendar period, suggesting a reduced long-term, treatment-related mortality. The 10-year relative survival for patients diagnosed in 2000-2009 was 0.95, 0.96, 0.93, 0.80, and 0.44 for the age groups 0-18, 19-35, 36-50, 51-65, and 66-80, respectively. Therefore, despite progress, age at diagnosis remains an important prognostic factor (P < .0005). Advances in therapy for patients with limited and advanced-stage HL have contributed to an increasing cure rate. In addition, our findings support that long-term mortality of HL therapy has decreased. Elderly HL patients still do poorly, and targeted treatment options associated with fewer side effects will advance the clinical HL field.


2002 ◽  
Vol 12 (2) ◽  
pp. 127-144
Author(s):  
GJF Saldanha ◽  
CG Clough ◽  
N Ward

Little is known about the frequency of headache in the elderly population as few epidemiological studies have been carried out. In one year in the USA, 70% of the general population had a headache, 5% of whom sought medical attention. In a large population-based study carried out in East Boston, US, some 17% of patients over 65 yrs of age reported frequent headache, with 53% of women and 36% of men reporting headache in the previous year.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2408-2408
Author(s):  
Sigurdur Y. Kristinsson ◽  
Ola Landgren ◽  
Paul Dickman ◽  
Asa Derolf ◽  
Magnus Bjorkholm

Abstract Background: Over the last decades there have been advances in the treatment of patients with multiple myeloma (MM) and prognosis has improved with the introduction of new treatment strategies. However, few studies have addressed the issue which patients benefit most from these therapeutic changes over the years. Aims: To evaluate relative survival in all diagnosed MM patients in Sweden 1973–2001 and relate the changes to age, sex and type of hospital where diagnosis was made. Methods: All patients with MM notified to the Swedish Cancer Register in 1973–2001 were followed up by record linkage to the nationwide Cause of Death Register. Survival analyses were performed by obtaining relative survival (RS) defined as the ratio of observed versus expected survival. The study period was divided arbitrarily to four calendar periods: 1973–1979, 1980–1986, 1987–1993, and 1994–2002. Patients were grouped according to age at diagnosis (0–40, 41–50, 51–60, 61–70, 71–80, and 80+), sex, and hospital category. RS was estimated using SAS (Cary, NC, USA) and excess mortality modelled using Poisson regression. Results: A total of 13,376 patients (7,114 males and 6,262 females, mean age 69.8 years, and 32% diagnosed at a university hospital) were diagnosed with MM in Sweden between January 1st 1973 and December 31st 2001. The overall one-year RS estimates were 73%, 78%, 80%, and 81%, respectively, for the four calendar periods. The overall five-year RS was 31%, 32%, 34%, and 36% and the ten-year RS remained stable at 12%, 11% 13% in the first three periods; ten-year RS could not be calculated for the last calendar period. The increase in one-year RS was observed in all age categories over the four calendar periods, while the increase in five-year RS was restricted to patients <70 years. Younger age at onset was associated with a superior survival in all calendar periods. Differences in survival by age at diagnosis and calendar period were highly statistically significant (p<0.0001). Females had a superior 1- (p=0.002), 5- (p=0.024), and 10-year RS (p=0.019) compared to males, after adjusting for age and period. Patients diagnosed at university hospitals had superior 5- and 10-year RS (p=0.007) but not 1-year RS. Summary/conclusions: The present study shows an improved prognosis over time in a population-based study including > 13,000 MM patients diagnosed during a 29-year period. Of interest is that even one-year RS has improved in all age groups over the whole study period. Increase in five-year RS was only observed in patients aged <70 years. The ten-year RS did not improve over the first 20 years and could not be estimated for patients diagnosed in the last period. Younger age at diagnosis was associated with superior one-, five- and ten-year RS in all calendar periods. Females had a significantly better survival than males. A significant difference in survival was seen according to type of hospital, with patients diagnosed at a university hospital surviving longer. In conclusion, the results show that survival of MM patients has improved during the study period. However, long-term survival has not improved significantly. Males, elderly patients and patients diagnosed during early calendar periods experienced higher excess mortality.


PLoS ONE ◽  
2019 ◽  
Vol 14 (1) ◽  
pp. e0210887
Author(s):  
Marissa C. van Maaren ◽  
Robert F. Kneepkens ◽  
Joke Verbaan ◽  
Peter C. Huijgens ◽  
Valery E. P. P. Lemmens ◽  
...  

2011 ◽  
Vol 29 (18) ◽  
pp. 2514-2520 ◽  
Author(s):  
Magnus Björkholm ◽  
Lotta Ohm ◽  
Sandra Eloranta ◽  
Åsa Derolf ◽  
Malin Hultcrantz ◽  
...  

Purpose Chronic myeloid leukemia (CML) management changed dramatically with the development of imatinib mesylate (IM), the first tyrosine kinase inhibitor targeting the BCR-ABL1 oncoprotein. In Sweden, the drug was approved in November 2001. We report relative survival (RS) of patients with CML diagnosed during a 36-year period. Patients and Methods Using data from the population-based Swedish Cancer Registry and population life tables, we estimated RS for all patients diagnosed with CML from 1973 to 2008 (n = 3,173; 1,796 males and 1,377 females; median age, 62 years). Patients were categorized into five age groups and five calendar periods, the last being 2001 to 2008. Information on use of upfront IM was collected from the Swedish CML registry. Results Relative survival improved with each calendar period, with the greatest improvement between 1994-2000 and 2001-2008. Five-year cumulative relative survival ratios (95% Cls) were 0.21 (0.17 to 0.24) for patients diagnosed 1973-1979, 0.54 (0.50 to 0.58) for 1994-2000, and 0.80 (0.75 to 0.83) for 2001-2008. This improvement was confined to patients younger than 79 years of age. Five-year RSRs for patients diagnosed from 2001 to 2008 were 0.91 (95% CI, 0.85 to 0.94) and 0.25 (95% CI, 0.10 to 0.47) for patients younger than 50 and older than 79 years, respectively. Men had inferior outcome. Upfront overall use of IM increased from 40% (2002) to 84% (2006). Only 18% of patients older than 80 years of age received IM as first-line therapy. Conclusion This large population-based study shows a major improvement in outcome of patients with CML up to 79 years of age diagnosed from 2001 to 2008, mainly caused by an increasing use of IM. The elderly still have poorer outcome, partly because of a limited use of IM.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2852-2852
Author(s):  
Marek Trneny ◽  
Petra Obrtlikova ◽  
Jiri Schwarz ◽  
Tomas Pavlik ◽  
Jan Muzik ◽  
...  

Abstract Abstract 2852 Background: The chronic lymphocytic leukemia is the most frequent leukemia in the western world predominantly diagnosed in older population. It is still considered to be incurable disease, but the significant proportion of patients do not require any treatment during the course of disease. The outcome is influenced by many factors including leukemia biology, patient's status, complications based on immune dysfunction, treatment choice, supportive care and other factors. Clinical trials are focused on highly selected population in need of treatment. Population based data providing the full picture is extremely rare, especially with the treatment data. The presented population-based study provides the full picture of CLL population including the untreated as well treated cohorts in different age and gender subgroups. Methods: Using data from the nationwide, population-based Czech Cancer Registry (CCR) and Czech population life-tables provided by the Czech Statistical Office to the Human Mortality Database stratified by age, sex, and calendar time we characterized trends in incidence, mortality and prevalence for all pts diagnosed with CLL in Czech Republic 1979–2008 and relative survival for patients diagnosed in five calendar periods (1980–1984, 1985–1989, 1990–1994, 1995–1999, 2000–2003). All computations were performed using Stata 10.1™ software. Results: Altogether 13, 162 pts with CLL diagnosis have been reported to the CCR from 1979 till 2008. Median age 70 years at diagnosis remains unchanged during the whole period, the male/female ratio was 1.5: 1. The CLL incidence increased from 3.3 in 1979 to 5.6 per 100 thousands inhabitants in 2008. The therapy was administered in 54% of all patients, with the significant trend to decrease with calendar period from 60.4% for pts dg in 1980–84 period to 42.3% in 200019403 period. The treatment was administered in 70.2% of pts younger than 60 y compared to 50.1% of older than 60y with the trend to decreased number of treated pts during the time in both cohorts (from 78.5% to 55.3% for younger and from 55.4% to 38.7% for older cohort). The improvement in five years relative survival ratios (RSRs; 95% confidence interval) with calendar period was observed for all patients starting at 0.46 (0.39–0.53), 0.48 (0.42–0.55), 0.52 (0.46–0.59), 0.60 (0.54–0.66) to 0.69 (0.62–0.74). The 5 y RSRs was better for untreated patients compared to treated pts resp. with improvement in both cohorts from 0.53 (0.44–0–62) and 0.34 (0.25–0.45) resp. in 1980–4 period to 0.77 (0.70–0.84) and 0.51 (0.42–0.60) resp. in 2000–3 period. The same trend was observed for young and old, untreated and treated pts. The females have significant better outcome compared to males in all cohorts, young as well old, untreated as well treated. Conclusions: In this large population-based study with more than 13, 000 patients we confirmed the increased CLL incidence together with decreased need of treatment which can be explained by the higher number of patients diagnosed in early stages in the recent time. The patients without need of treatment have siginificantly better 5y RSRs compared to pts with need of treatment, the females have significantly better 5y RSRs compared to males consistently in all subgroups. The patient's survival was improved with calendar period for the whole CLL population as well for the younger and older, females and males, untreated as well treated cohort. Disclosures: No relevant conflicts of interest to declare.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1069.1-1069
Author(s):  
L. Barra ◽  
J. Pope ◽  
P. Pequeno ◽  
J. Gatley ◽  
J. Widdifield

Background:Individuals with giant cell arteritis (GCA) are at increased risk of serious morbidity including cardiovascular disease and stroke. Yet the risk of mortality among individuals with GCA have produced conflicting reports1.Objectives:Our aim was to evaluate excess all-cause mortality among individuals with GCA relative to the general population over time.Methods:We performed a population-based study in Ontario, Canada, using health administrative data among all individuals 50 years and older. Individuals with GCA were identified using a validated case definition (81% PPV, 100% specificity). All Ontario residents aged 50 and above who do not have GCA served as the General Population comparators. Deaths occurring in each cohort each year were ascertained from vital statistics. Annual crude and age/sex standardized all-cause mortality rates were determined for individuals with and without GCA between 2000 and 2018. Standardized mortality ratios (SMRs) were calculated to measure relative excess mortality over time. Differences in mortality between sexes and ages were also evaluated.Results:Population denominators among individuals 50 years and older with GCA and the General Population increased over time with 12,792 GCA patients and 5,456,966 comparators by 2018. Annual standardized mortality rates among the comparators steadily declined over time and were significantly lower than GCA morality rates (Figure). Annual GCA mortality rates fluctuated between 42-61 deaths per 1000 population (with overlapping confidence intervals) during the same time period. SMRs for GCA ranged from 1.28 (95% CI 1.08,1.47) at the lowest in 2002 to 1.96 (95% CI 1.84, 2.07) at the highest in 2018. GCA mortality rates and SMRs were highest among males and younger age groups.Conclusion:Over a 19-year period, mortality has remained increased among GCA patients relative to the general population. GCA mortality rates were higher among males and more premature deaths were occurring at younger age groups. In our study, improvements to the relative excess mortality for GCA patients over time (mortality gap) did not occur. Understanding cause-specific mortality and other factors are necessary to inform contributors to premature mortality among GCA patients.References:[1]Hill CL, et al. Risk of mortality in patients with giant cell arteritis: a systematic review and meta-analysis. Semin Arthritis Rheum. 2017;46(4):513-9.Figure.Acknowledgments: :This study was supported by a CIORA grantDisclosure of Interests:Lillian Barra: None declared, Janet Pope Grant/research support from: AbbVie, Bristol-Myers Squibb, Eli Lilly & Company, Merck, Roche, Seattle Genetics, UCB, Consultant of: AbbVie, Actelion, Amgen, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Eicos Sciences, Eli Lilly & Company, Emerald, Gilead Sciences, Inc., Janssen, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB, Speakers bureau: UCB, Priscila Pequeno: None declared, Jodi Gatley: None declared, Jessica Widdifield: None declared


Sign in / Sign up

Export Citation Format

Share Document