title: Mortality and Causes of Death in Patients with Sporadic Inclusion Body Myositis: Survey Study Based on the Clinical Experience of Specialists in Australia, Europe and the USA.
abstract: There is a paucity of data on mortality and causes of death (CoDs) in patients with sporadic inclusion body myositis (sIBM), a rare, progressive, degenerative, inflammatory myopathy that typically affects those aged over 50 years.
  
  
  
  Based on patient records and expertise of clinical specialists, this study used questionnaires to evaluate physicians' views on clinical characteristics of sIBM that may impact on premature mortality and CoDs in these patients.
  
  
  
  Thirteen physicians from seven countries completed two questionnaires online between December 20, 2012 and January 15, 2013. Responses to the first questionnaire were collated and presented in the second questionnaire to seek elaboration and identify consensus.
  
  
  
  All 13 physicians completed both questionnaires, providing responses based on 585 living and 149 deceased patients under their care. Patients were reported to have experienced dysphagia (60.2%) and injurious falls (44.3%) during their disease. Over half of physicians reported that a subset of their patients with sIBM had a shortened lifespan (8/13), and agreed that bulbar dysfunction/dysphagia/oropharyngeal involvement (12/13), early-onset disease (8/13), severe symptoms (8/13), and falls (7/13) impacted lifespan. Factors related to sIBM were reported as CoDs in 40% of deceased patients. Oropharyngeal muscle dysfunction was ranked as the leading feature of sIBM that could contribute to death. The risk of premature mortality was higher than the age-matched comparison population.
  
  
  
  In the absence of data from traditional sources, this study suggests that features of sIBM may contribute to premature mortality and may be used to inform future studies.
authors.affiliation: RTI Health Solutions, Research Triangle Park, NC, USA.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Price
authors.affiliation: VEB HealthCare LLC, Morristown, NJ, USA.
authors.forename: Victoria
authors.initials: V
authors.lastname: Barghout
authors.affiliation: Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Department of Internal Medicine, Pierre and Marie Curie University, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Benveniste
authors.affiliation: Johns Hopkins Myositis Center, Division of Rheumatology, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Lisa
authors.initials: L
authors.lastname: Christopher-Stine
authors.affiliation: Department of Neurology, Concord Hospital, Concord, NSW, Australia.
authors.forename: Alastair
authors.initials: A
authors.lastname: Corbett
authors.affiliation: Department of Neurology, Academic Medical Center, Amsterdam, Netherlands.
authors.forename: Marianne
authors.initials: M
authors.lastname: de Visser
authors.affiliation: Department of Neurology, John Radcliffe Hospital, Oxford, UK.
authors.forename: David
authors.initials: D
authors.lastname: Hilton-Jones
authors.affiliation: Departments of Neurology and Pediatrics, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
authors.forename: John T
authors.initials: JT
authors.lastname: Kissel
authors.affiliation: Departments of Neurology and Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Lloyd
authors.affiliation: Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Ingrid E
authors.initials: IE
authors.lastname: Lundberg
authors.affiliation: Institute for Immunology and Infectious Diseases, Murdoch University, WA, Australia.
authors.forename: Francis
authors.initials: F
authors.lastname: Mastaglia
authors.affiliation: Department of Neurology and Orthopedic Surgery, University of California, Irvine, CA, USA.
authors.forename: Tahseen
authors.initials: T
authors.lastname: Mozaffar
authors.affiliation: Western Australian Neuromuscular Research Institute, University of Western Australia, Murdoch University and Department of Neurology, Fiona Stanley Hospital, Perth, WA, Australia.
authors.forename: Merrilee
authors.initials: M
authors.lastname: Needham
authors.affiliation: Department of Neurology, University Medical Center Göttingen; Department of Neuroimmunology, Institute for Multiple Sclerosis Research and Hertie Foundation, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Schmidt
authors.affiliation: Arizona Neurological Research Center, Scottsdale, AZ, USA.
authors.forename: Kumaraswamy
authors.initials: K
authors.lastname: Sivakumar
authors.affiliation: RTI Health Solutions, Research Triangle Park, NC, USA.
authors.forename: Carla
authors.initials: C
authors.lastname: DeMuro
authors.affiliation: Novartis Pharma Corporation, East Hanover, NJ, USA.
authors.forename: Brian S
authors.initials: BS
authors.lastname: Tseng
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D002423:Cause of Death; D005060:Europe / Q000453:epidemiology; D005260:Female; D006306:Health Surveys; D006801:Humans; D008297:Male; D008875:Middle Aged; D061213:Mortality, Premature; D018979:Myositis, Inclusion Body / Q000401:mortality*; D010820:Physicians / Q000706:statistics & numerical data; D014481:United States / Q000453:epidemiology
keywords: Myositis; cause of death; deglutition disorder; inclusion body; morbidity; mortality; myositis; neuromuscular disease