Objectives . The aims of this study were to determine the incidence and prevalence of inflammatory myopathies (IMs), their epidemiological tendencies over time and their possible key determinants. Methods . All original articles in English or French regarding the prevalence and/or incidence of IMs were searched. The methods of case ascertainment, epidemiological analysis and diagnostic criteria were systematically analysed. Results . Forty-six articles published between 1966 and 2013 were found in which the incidence of IMs as a whole ranged from 1.16 to 19/million/year and their prevalence ranged from 2.4 to 33.8 per 100 000 inhabitants. Methodological heterogeneities limited comparisons, although certain epidemiological tendencies were highlighted. The relative incidence of DM may follow a latitudinal gradient in the northern hemisphere that may be explained by the immunomodulatory action of ultraviolet radiation. The prevalence of sporadic inclusion body myositis (sIBM) was correlated with the frequency of HLA-DR3. Juvenile myositis onset was non-random over seasons and/or time, consistent with a role of infectious diseases, although other environmental factors may be involved. Disparities according to sex, age and geographical origin were also found. The frequency of IM increased over time, which may reflect progress in diagnostic performance, although there is still a need to increase the level of awareness with regard to these diseases, especially sIBM, as attested by its considerably delayed diagnosis. Conclusion . This first systematic literature review confirms the rarity of IM and may highlight certain genetic and environmental determinants of IM. There is a need for uniformity in diagnostic and classification criteria as well as more exhaustive case ascertainment to refine IM epidemiology.

Incidence and prevalence of inflammatory myopathies: a systematic review

Abstract Background The medical care of patients with myositis is a great challenge in clinical practice. This is due to the rarity of these disease, the complexity of diagnosis and management as well as the lack of systematic analyses. Objectives Therefore, the aim of this project was to obtain an overview of the current care of myositis patients in Germany and to evaluate epidemiological trends in recent years. Methods In collaboration with BARMER Insurance, retrospective analysis of outpatient and inpatient data from an average of approximately 8.7 million insured patients between January 2005 and December 2019 was performed using ICD-10 codes for myositis for identification of relevant data. In addition, a comparative analysis was performed between myositis patients and an age-matched comparison group from other populations insured by BARMER. Results 45,800 BARMER-insured individuals received a diagnosis of myositis during the observation period, with a relatively stable prevalence throughout. With regard to comorbidities, a significantly higher rate of cardiovascular disease as well as neoplasm was observed compared to the control group within the BARMER-insured population. In addition, myositis patients suffer more frequently from psychiatric disorders, such as depression and somatoform disorders. However, the ICD-10 catalogue only includes the specific coding of “dermatomyositis” and “polymyositis” and thus does not allow for a sufficient analysis of all idiopathic inflammatory myopathies subtypes. Conclusion The current data provide a comprehensive epidemiological analysis of myositis in Germany, highlighting the multimorbidity of myositis patients. This underlines the need for multidisciplinary management. However, the ICD-10 codes currently still in use do not allow for specific analysis of the subtypes of myositis. The upcoming ICD-11 coding may improve future analyses in this regard. 

/pdf/myositis-in-germany-epidemiological-insights-over-15-years-10umodqg.pdf

Myositis in Germany: epidemiological insights over 15 years from 2005 to 2019

Background In the UK, large-scale electronic primary care datasets can provide up-to-date, accurate epidemiological information on rarer diseases, where specialist diagnoses from hospital discharges and clinic letters are generally well recorded and electronically searchable. Current estimates of the number of people living with neuromuscular disease (NMD) have largely been based on secondary care data sources and lacked direct denominators. Objective To estimate trends in the recording of neuromuscular disease in UK primary care between 2000–2019. Methods The Clinical Practice Research Datalink (CPRD) database was searched electronically to estimate incidence and prevalence rates (per 100,000) for a range of NMDs in each year. To compare trends over time, rates were age standardised to the most recent CPRD population (2019). Results Approximately 13 million patients were actively registered in each year. By 2019, 28,230 active patients had ever received a NMD diagnosis (223.6), which was higher among males (239.0) than females (208.3). The most common classifications were Guillain-Barre syndrome (40.1), myasthenia gravis (33.7), muscular dystrophy (29.5), Charcot-Marie-Tooth (29.5) and inflammatory myopathies (25.0). Since 2000, overall prevalence grew by 63%, with the largest increases seen at older ages (≥65-years). However, overall incidence remained constant, though myasthenia gravis incidence has risen steadily since 2008, while new cases of muscular dystrophy fell over the same period. Conclusions Lifetime recording of many NMDs on primary care records exceed current estimates of people living with these conditions; these are important data for health service and care planning. Temporal trends suggest this number is steadily increasing, and while this may partially be due to better recording, it cannot be simply explained by new cases, as incidence remained constant. The increase in prevalence among older ages suggests increases in life expectancy among those living with NMDs may have occurred.

Prevalence and Incidence of Neuromuscular Conditions in the UK Between 2000 and 2019: A Retrospective Study Using Primary Care Data

Objectives To estimate the incidence rate and prevalence of idiopathic inflammatory myopathies (IIMs) in Sweden across clinical subgroups, age, sex, educational level and place of residence and to assess the robustness of register-based case definitions. Methods IIM was identified from the Swedish National Patient Register and the Swedish Rheumatology Quality Register. The base case definition required ⩾2 visits indicating IIM (first ever with a consecutive visit within 1-12 months for incident cases) and the robustness was tested by applying a more liberal and a stricter definition. Results Using the base case definition, 558 incident IIM patients were identified between 2007 and 2011. The incidence was estimated to 11 (13 for women and 9.7 for men) per 1 000 000 person years and was stable across case definitions. Incidence increased with age and peaked at the 50-79 years age groups. No differences were observed between different levels of education and place of residence. We identified 1267 IIM patients on 1 January 2012 corresponding to a prevalence of 14 per 100 000. Conclusion We present nationwide register-based incidence and prevalence estimates for IIM, robust across three different case definitions. In contrast to many other reports, we did not find incidence by age to be bimodal and we found no explanation of incidence variation across education and residency. These register-based case definitions can be included in future population-based studies to better understand disease aetiology, risk factors and comorbidities.

Incidence and prevalence of idiopathic inflammatory myopathies in Sweden: a nationwide population-based study.

Background The idiopathic inflammatory myopathies (IIM) are a rare, heterogeneous group of autoimmune muscle disorders, including dermatomyositis (DM), polymyositis (PM), and inclusion body myositis. Data on the epidemiology of IIM are limited and quite variable with the reported incidence and prevalence ranging from 0.4 to 5.8 per 100,000 person-years (PY) and 3.5 to 25.0 per 100,000 population, respectively. Differences in methods of case identification, small study sizes, geographical differences, and changes in coding and reporting practices may contribute to the variation. Objectives To describe the epidemiology of IIM patients in England using medical record databases. Methods By linking the General Practice Research Database (GPRD) and the Hospital Episode Statistics (HES) database, we identified IIM patients with a diagnosis code of myositis (DM, PM, juvenile DM, and unspecified dermatopolymyositis) in either database and retrospectively measured epidemiologic indicators of IIM. Results From 2000-2009, 326 incident IIM cases were identified of which 62% were female and 81% were in those 45 years of age or older. The overall incidence of IIM was 1.9/100,000 PY (95% CI: 1.7-2.1/100,000 PY) ranging from 1.6 in 2000 to 2.0 in 2009. Among the incident cases, the 5- and 10-year survival was 77% and 74%, respectively. There were 465 prevalent IIM cases identified in 2009 corresponding to the overall prevalence of 27.2/100,000 people (95% CI: 24.8-29.8/100,000 people). The prevalence was positively associated with female gender and older age. Among prevalent IIM cases, 14% had a diagnosis of other autoimmune conditions including rheumatoid arthritis (9%), systemic lupus erythematosus (3%), systemic sclerosis (4%), or Sjogren’s disease (2%). In 2009, the hospitalization rate for IIM was 5.6 per 100,000 in the general populations. Conclusions We found a higher IIM prevalence in England than previously reported in other countries. Studies that confirm the electronic diagnoses are needed to validate the findings. Disclosure of Interest T. Tran Employee of: MedImmune LLC, A. Steffey Employee of: MedImmune LLC, H. Caspard Employee of: MedImmune LLC

https://ard.bmj.com/content/annrheumdis/71/Suppl_3/465.3.full.pdf

FRI0447 Epidemiology of idiopathic inflammatory myopathies in england – a database analysis

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FRI0447 Epidemiology of idiopathic inflammatory myopathies in england – a database analysis