Dataset Information

Survival and associated comorbidities in inclusion body myositis.

ABSTRACT:

Objective

To evaluate survival and associated comorbidities in inclusion body myositis (IBM) in a population-based, case-control study.

Methods

We utilized the expanded Rochester Epidemiology Project medical records-linkage system, including 27 counties in Minnesota and Wisconsin, to identify patients with IBM, other inflammatory myopathies (IIM), and age/sex-matched population-controls. We compared the frequency of various comorbidities and survival among groups.

Results

We identified 50 IBM patients, 65 IIM controls and 294 population controls. Dysphagia was most common in IBM (64%) patients. The frequency of neurodegenerative disorders (dementia/parkinsonism) and solid cancers was not different between groups. Rheumatoid arthritis was the most common rheumatic disease in all groups. A total of 36% of IBM patients had a peripheral neuropathy, 6% had Sjögren's syndrome and 10% had a haematologic malignancy. T-cell large granular lymphocytic leukaemia was only observed in the IBM group. None of the IBM patients had hepatitis B or C, or HIV. IBM patients were 2.7 times more likely to have peripheral neuropathy, 6.2 times more likely to have Sjögren's syndrome and 3.9 times more likely to have a haematologic malignancy than population controls. IBM was associated with increased mortality, with a 10-year survival of 36% from index, compared with 67% in IIM and 59% in population controls. Respiratory failure or pneumonia (44%) was the most common cause of death.

Conclusions

IBM is associated with lower survival, and higher frequency of peripheral neuropathy, Sjögren's syndrome and haematologic malignancies than the general population. Close monitoring of IBM-related complications is warranted.

SUBMITTER: Naddaf E

PROVIDER: S-EPMC9071572 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Publications

Survival and associated comorbidities in inclusion body myositis.

Naddaf Elie E Shelly Shahar S Mandrekar Jay J Chamberlain Alanna M AM Hoffman E Matthew EM Ernste Floranne C FC Liewluck Teerin T

Rheumatology (Oxford, England) 20220501 5

<h4>Objective</h4>To evaluate survival and associated comorbidities in inclusion body myositis (IBM) in a population-based, case-control study.<h4>Methods</h4>We utilized the expanded Rochester Epidemiology Project medical records-linkage system, including 27 counties in Minnesota and Wisconsin, to identify patients with IBM, other inflammatory myopathies (IIM), and age/sex-matched population-controls. We compared the frequency of various comorbidities and survival among groups.<h4>Results</h4>W ...[more]

PMID: 34534271

Similar Datasets

Project description:BACKGROUND:Associations between disease characteristics and payer-relevant outcomes can be difficult to establish for rare and progressive chronic diseases with sparse available data. We developed an exploratory bridging model to predict premature mortality from disease characteristics, and using inclusion body myositis (IBM) as a representative case study. METHODS:Candidate variables that may be potentially associated with premature mortality were identified by disease experts and from the IBM literature. Interdependency between candidate variables in IBM patients were assessed using existing patient-level data. A Bayesian survival model for the IBM population was developed with identified variables as predictors for premature mortality in the model. For model selection and external validation, model predictions were compared to published mortality data in IBM patient cohorts. After validation, the final model was used to simulate the increased risk of premature death in IBM patients. Baseline survival was based on age- and gender-specific survival curves for the general population in Western countries as reported by the World Health Organisation. RESULTS:Presence of dysphagia, aspiration pneumonia, falls, being wheelchair-bound and 6-min walking distance (6MWD in meters) were identified as candidate variables to be used as predictors for premature mortality based on inputs received from disease experts and literature. There was limited correlation between these functional performance measures, which were therefore treated as independent variables in the model. Based on the Bayesian survival model, among all candidate variables, presence of dysphagia and decrease in 6MWD [m] were associated with poorer survival with contributing hazard ratios (HR) 1.61 (95% credible interval [CrI]: 0.84-3.50) and 2.48 (95% CrI: 1.27-5.00) respectively. Excess mortality simulated in an IBM cohort vs. an age- and gender matched general-population cohort was 4.03 (95% prediction interval 1.37-10.61). CONCLUSIONS:For IBM patients, results suggest an increased risk of premature death compared with the general population of the same age and gender. In the absence of hard data, bridging modelling generated survival predictions by combining relevant information. The methodological principle would be applicable to the analysis of associations between disease characteristics and payer-relevant outcomes in progressive chronic and rare diseases. Studies with lifetime follow-up would be needed to confirm the modelling results.

Dataset Information

Survival and associated comorbidities in inclusion body myositis.

Objective

Methods

Results

Conclusions

Publications

Survival and associated comorbidities in inclusion body myositis.

Similar Datasets

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