Silva, MA;
Cogollo, E;
Isenberg, DA;
(2016)
Why do patients with myositis die? A retrospective analysis of a single-centre cohort.
Clinical and Experimental Rheumatology
, 34
(5)
pp. 820-826.
1593-098X.
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Abstract
OBJECTIVES: Causes of death in inflammatory myopathies have rarely been studied. We have assessed a cohort of myositis patients followed in a single centre over a 37-year period, reviewing the mortality rate, causes of death and predictors of poor prognosis. METHODS: We performed a single-centre, retrospective study on patients aged ≥16 years fulfilling 3 or 4 of the Bohan and Peter criteria, noting their demographic data, clinical features, serology, treatment and outcome. RESULTS: Of 97 patients identified, 74.2% were female. The mean age at diagnosis was 40.5 years (SD 13.2). 38.1% had adult-onset dermatomyositis, 36.1% adult-onset polymyositis and 25.8% overlap myositis. 96.9% had upper and lower limb involvement (UL+/LL+) and 62.9% had a highest CK≥10 times the upper limit of normal. 33% had significant infection(s). The disease course was chronic persistent in 29.9%, relapsing and remitting in 34% and monophasic in 36.1%. All received steroids and 92.8% other immunosuppressant(s). The median follow-up was 9 years (IQR 11.5). The estimated cumulative proportion survival at 5, 10, 15 and 20 years were 94.6%, 82.2%, 72,1% and 66.1%, respectively. 24.7% of patients died, mostly due to infection (29.2%). In univariate analysis, lung involvement (HR 1.78, p=0.013), infection (HR 4.18, p=0.003) and UL+/LL+ (HR 0.13, p=0.010) were statistically significantly associated with the risk of death. In the multivariate analysis infection (HR 3.68, p=0.009) and UL+/LL+ (HR 0.16, p=0.027) were statistically significantly associated with survival. CONCLUSIONS: A good long-term survival is reported. Nevertheless, careful follow-up of myositis patients is important.
Type: | Article |
---|---|
Title: | Why do patients with myositis die? A retrospective analysis of a single-centre cohort |
Open access status: | An open access version is available from UCL Discovery |
DOI: | 1593-098X |
Publisher version: | http://www.clinexprheumatol.org/abstract.asp?a=996... |
Language: | English |
Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
Keywords: | Inflammatory Myopathies, Extramuscular Complications, Causes of Death, Long-term Survival, Predictors of Poor Prognosis, Idiopathic Inflammatory Myopathies, Onset Polymyositis Dermatomyositis, Interstitial Lung-disease, Laboratory Features, Term, Prognosis, Mortality, Survival, Death, Complications |
UCL classification: | UCL UCL > Provost and Vice Provost Offices UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
URI: | https://discovery.ucl.ac.uk/id/eprint/1536413 |




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