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. 2016 Mar 3;3(1):67-75.
doi: 10.3233/JND-150138.

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

Mark A Price  1 , Victoria Barghout  2 , Olivier Benveniste  3 , Lisa Christopher-Stine  4 , Alastair Corbett  5 , Marianne de Visser  6 , David Hilton-Jones  7 , John T Kissel  8 , Thomas E Lloyd  9 , Ingrid E Lundberg  10 , Francis Mastaglia  11 , Tahseen Mozaffar  12 , Merrilee Needham  13 , Jens Schmidt  14 , Kumaraswamy Sivakumar  15 , Carla DeMuro  1 , Brian S Tseng  16
Affiliations
Free PMC article

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

Mark A Price et al. J Neuromuscul Dis. .
Free PMC article

Abstract

Background: 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.

Objective: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Myositis; cause of death; deglutition disorder; inclusion body; morbidity; mortality; myositis; neuromuscular disease.

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Figures

Fig.1
Fig.1
Ranked features of sIBM that can contribute to death, based on the experience of 13 physicians from seven countries. Features presented for ranking were based on the physicians’ responses in round 1 of the questionnaire. Each physician was asked to score the features of sIBM shown above on the basis of contribution to death in the patients they were following (1 = most common, 5 = least common). Five physicians did not think features of sIBM contributed to death. Of those physicians who provided scores, mean scores were calculated and used to rank features. Physicians did not expand on which comorbidities associated with sIBM could contribute to death. sIBM, sporadic inclusion body myositis.
Fig.2
Fig.2
Ranked end-of-life settings for patients with sIBM, based on the experience of 13 physicians from seven countries. Each physician was asked to score the end-of-life settings shown above (1 = most common, 5 = least common). Based on all the physicians’ responses, the mean score was calculated and used to rank the settings. sIBM, sporadic inclusion body myositis.
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