The spectrum of idiopathic inflammatory myopathies in Western Australia: epidemiological characteristics and mortality over time.

To determine long term overall and subgroup specific incidence rates and associated mortality for idiopathic inflammatory myopathies (IIM) in a population wide study. We included patients hospitalised between 1980 and 2015 with incident IIM as defined by relevant diagnostic codes for dermatomyositis (DM) polymyositis (PM), inclusion body myositis (IBM), other IIM and overlap myositis (OM) in the Western Australia Health Hospital Morbidity Data Collection (n = 846). Trends over time for annual incidence rate per million population (AIR) were analysed by least square regression and Kaplan-Meier survival and mortality rates (MR)/100 person years compared with a matched control group (n = 3681). The averaged AIR for all IIM was 19 (CI 10.4-27.5) and stable over time with point prevalence reaching 205.3 (CI 185.6-226.6) per million in 2015. Over time, the AIR for DM 5.0 (CI 0.6-9.4) and IBM 3.3 (CI 0.7-9.6) was stable, while AIR decreased for PM (p < 0.01) and increased for other IIM (p < 0.01) and OM (p < 0.01). IBM patients were eldest at diagnosis (68 years, CI 59-77) with male preponderance in IBM (53.4%) and other IIM (55.8%) groups. Crude mortality (54.5 vs 41.3%), MR ratio (6.65 vs 5.91) and 5 (65.8% vs 71.6%) and 10-year (52.5% vs 58.7%) survival were all worse for IIM patients (all p < 0.05). IBM patients had highest MR (10.1; CI 8.38-12.14) and lowest 10-year survival (39.2%). While cardiovascular disease and cancer were predominant causes of death, they were proportionally lower in IIM patients, where respiratory and rheumatic disease were more frequent causes of death. While the overall incidence of IIM in WA was stable over 35 years, the spectrum of IIM has changed significantly with increases especially in other IIM and OM. The overall prognosis with IIM remains guarded with 10-year survival just over 50%.