Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia

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...

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Main Authors: Alojzija Hočevar, Andrej Viršček, Monika Krošel, Suzana Gradišnik, Žiga Rotar, Matija Tomšič, Iztok Holc
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-12-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2021.801078/full
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author Alojzija Hočevar
Alojzija Hočevar
Andrej Viršček
Monika Krošel
Monika Krošel
Suzana Gradišnik
Žiga Rotar
Žiga Rotar
Matija Tomšič
Matija Tomšič
Iztok Holc
Iztok Holc
author_facet Alojzija Hočevar
Alojzija Hočevar
Andrej Viršček
Monika Krošel
Monika Krošel
Suzana Gradišnik
Žiga Rotar
Žiga Rotar
Matija Tomšič
Matija Tomšič
Iztok Holc
Iztok Holc
author_sort Alojzija Hočevar
collection DOAJ
description 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.
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spelling doaj.art-d3143e51c6bf4ea7b25b7a600738e56d2022-12-21T23:31:18ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-12-01810.3389/fmed.2021.801078801078Survival of Patients With Idiopathic Inflammatory Myopathies in SloveniaAlojzija Hočevar0Alojzija Hočevar1Andrej Viršček2Monika Krošel3Monika Krošel4Suzana Gradišnik5Žiga Rotar6Žiga Rotar7Matija Tomšič8Matija Tomšič9Iztok Holc10Iztok Holc11Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana, SloveniaStatistical Office of the Republic of Slovenia, Ljubljana, SloveniaDepartment of Rheumatology, University Medical Centre Ljubljana, Ljubljana, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana, SloveniaDepartment of Rheumatology, University Medical Centre Maribor, Maribor, SloveniaDepartment of Rheumatology, University Medical Centre Ljubljana, Ljubljana, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana, SloveniaDepartment of Rheumatology, University Medical Centre Ljubljana, Ljubljana, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana, SloveniaDepartment of Rheumatology, University Medical Centre Maribor, Maribor, SloveniaFaculty of Medicine, University of Maribor, Maribor, SloveniaBackground: 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.https://www.frontiersin.org/articles/10.3389/fmed.2021.801078/fullinflammatory myopathyprognostic factorssurvivalmortalitycancer
spellingShingle Alojzija Hočevar
Alojzija Hočevar
Andrej Viršček
Monika Krošel
Monika Krošel
Suzana Gradišnik
Žiga Rotar
Žiga Rotar
Matija Tomšič
Matija Tomšič
Iztok Holc
Iztok Holc
Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
Frontiers in Medicine
inflammatory myopathy
prognostic factors
survival
mortality
cancer
title Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
title_full Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
title_fullStr Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
title_full_unstemmed Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
title_short Survival of Patients With Idiopathic Inflammatory Myopathies in Slovenia
title_sort survival of patients with idiopathic inflammatory myopathies in slovenia
topic inflammatory myopathy
prognostic factors
survival
mortality
cancer
url https://www.frontiersin.org/articles/10.3389/fmed.2021.801078/full
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