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Why do patients with myositis die? A retrospective analysis of a single-centre cohort

1, 2, 3


  1. Department of Internal Medicine, Hospital Distrital da Figueira da Foz, Coimbra, Portugal.
  2. Department of Internal Medicine, Hospital Principe de Asturias, Alcala de Henares, Madrid, Spain.
  3. Centre for Rheumatology, Department of Medicine, University College London, UK.

2016 Vol.34, N°5
PI 0820, PF 0826
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PMID: 27494511 [PubMed]

Received: 15/09/2015
Accepted : 23/11/2015
In Press: 02/08/2016
Published: 16/09/2016


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.
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.
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.
A good long-term survival is reported. Nevertheless, careful follow-up of myositis patients is important.

Rheumatology Article