Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia

Abstract

Background: Idiopathic inflammatory myopathies (IIMs) are rare systemic diseases associated with significant morbidity and mortality. The aim of our study was to estimate for the first time the survival of IIM patients in Slovenia.Methods: We included IIM patients diagnosed between January 2005 and December 2020 and followed at two secondary/tertiary rheumatology centers in the country. To study survival/mortality the censor date of April 14 2021 was set. Kaplan–Meier analysis and standardized mortality ratio (SMR) were plotted using data of age and sex matched Slovenian population as a reference. Cox proportional hazards regression analysis was used to study prognostic factors for IIM mortality.Results: During the 16-year observation period, we identified 217 new IIM patients. During follow up 65 (30.0%) patients died. In the first year following IIM diagnosis the SMR was nearly 7-fold higher compared to the matched general population [SMR 6.88 (95%CI 4.41–10.24)] and remained higher also during the following 4 years. However, when excluding IIM patients with cancer, the survival outcome was, except in the first year after IIM diagnosis [SMR 5.55 (95%CI 3.10–9.15)], comparable to matched general population. In addition to cancer [HR 3.71 (95% CI 2.18–6.04)], cardiac involvement [HR 2.18 (95% CI 1.07–4.45)], fever [HR 2.13 (95% CI 1.13–4.03)], and older age [HR 1.07 (95% CI 1.04–1.09)] were extracted as prognostic factors associated with death.Conclusion: The survival of patients with IIM patients was substantially worse compared to matched general population. Cancer was the leading cause of death in our cohort.

Long-term survival of patients with idiopathic inflammatory myopathies: anatomy of a single-centre cohort

“…Organ involvement also differ considerably between cohorts. In our study, cardiac, lung and GI involvement affected 23.7%, 38.5% and 36.5% of the patients, respectively while Slovenian and Italian cohorts reported much lower cardiac involvement (11.5% and 14.3%), and Spanish REMICAM cohort reported lower ILD prevalence (27%) (8,12,15). A Japanese cohort reported almost a doble ILD prevalence (64%) and lower cardiac and GI involvement (17% and 21%, respectively) (14).…”

“…As expected, most patients were treated with a combination of steroids and immunosuppressive drugs, with azathioprine being the most common (73.4%). There were some differences in drug preferences between studies, with other authors favouring the use of methotrexate (12,15), which may be due to drug policies, but also differences in organ involvement. In our study, we found that mortality rate was lower in patients who were ever given MMF, whereas those who received cyclophosphamide at anytime had higher mortality rates.…”

“…To the best of our knowledge, we describe the IIM cohort with the longest mean follow-up time of almost 15 years, with more than one fifth of our cohort having 20 years or more of follow-up. Previously, most other studies have a span time of 10 years or lower (8,12,15,16). Additionally, we reported higher 5 and 10-year survival rates (89% and 74%, respectively) than older studies (6,7,16,18), but similar to recent cohorts (8,10,12,14), which may reflect better management and treatment strategies.…”

“…However, in the last decades the prognosis has substantially improved, with recent studies reporting 5 and 10-year survival rates of 86% and 77%, respectively (8). Malignancy, infections, lung and cardiovascular complications are reported as the most common cause of death in IIM-patients (8)(9)(10)(11)(12)(13)(14)(15)(16). There is an increas-ing interest in studying IIM mortality, however, most studies have a relatively short follow-up period, usually less than 10 years (6,7,10,14,16).…”

The mosaic of autoimmunity – Finally discussing in person. The 13th international congress on autoimmunity 2022 (AUTO13) Athens

“…Holc demonstrated that Slovenian patients with idiopathic inflammatory myopathy (IIMs) had higher mortality rates compared to the general population in Slovenia during a 16-year observation period. The first year following diagnosis was characterized by 7 times higher mortality rate [ 155 ]. Vojinovic and colleagues found no close link between creatinine kinase (CK) levels, various types of IIM, and myositis-specific autoantibodies.…”