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. 2022 May 5;61(5):2016-2024.
doi: 10.1093/rheumatology/keab716.

Survival and associated comorbidities in inclusion body myositis

Elie Naddaf  1 , Shahar Shelly  1 , Jay Mandrekar  2 , Alanna M Chamberlain  2 , E Matthew Hoffman  1 , Floranne C Ernste  3 , Teerin Liewluck  1
Affiliations

Survival and associated comorbidities in inclusion body myositis

Elie Naddaf et al. Rheumatology (Oxford). .

Erratum in

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.

Keywords: Sjögren’s syndrome; case-control study; inclusion body myositis; large granular lymphocytic leukemia; peripheral neuropathy.

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Figures

<sc>Fig</sc>. 1
Fig. 1
Survival outcomes (A) Kaplan–Meier survival curves comparing IBM patients (blue line), inflammatory myopathy patients (red line) and population controls (orange line). (B) Clear separation in survival curves for IBM patients treated with corticosteroids (blue line) compared with untreated IBM patients (red line). IBM: inclusion body myositis.
See this image and copyright information in PMC

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References

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