Clinical features of Japanese patients with inclusion body myositis

Hori, Hiroko; Yamashita, Satoshi; Tawara, Nozomu; Hirahara, Tomoo; Kawakami, K.; Nishikami, T.; Maeda, Yasushi; Ando, Yumiko

doi:10.1016/j.jns.2014.08.009

Cited by 9 publications

(14 citation statements)

References 21 publications

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“…Both myopathic and neuropathic motor unit action potentials may be seen. About one third of patients will also exhibit a mild distal sensory axonal peripheral neuropathy [12,24]. Muscle MRI may show inflammation and fatty infiltration in the quadriceps, medial gastrocnemius, and tibialis anterior, although the clinical role of muscle imaging in IBM remains to be fully defined [25,26].…”

Section: Inclusion Body Myositismentioning

confidence: 99%

Myositis Mimics

Michelle

Mammen

2015

Curr Rheumatol Rep

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Patients with autoimmune myositis typically present with muscle weakness, elevated serum levels of muscle enzymes, and abnormal muscle biopsies. However, patients with other acquired myopathies or genetic muscle diseases may have remarkably similar presentations. Making the correct diagnosis of another muscle disease can prevent these patients from being exposed to the risks of immunosuppressive medications, which benefit those with myositis, but not those with other types of muscle disease. Here, we review some of the most common acquired and inherited muscle diseases that can mimic autoimmune myositis, including inclusion body myositis, limb girdle muscular dystrophies, metabolic myopathies, mitochondrial myopathies, and endocrine myopathies. We emphasize aspects of the medical history, physical exam, laboratory evaluation, and muscle biopsy analysis that can help clinicians distinguish myositis mimics from true autoimmune myositis.

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Section: Inclusion Body Myositismentioning

confidence: 99%

Myositis Mimics

Michelle

Mammen

2015

Curr Rheumatol Rep

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“…Other studies have reported more rapid progression in patients with an older age of onset, though this was not observed in our cohort. 9–11,14…”

Section: Discussionmentioning

confidence: 99%

“…Other studies have reported more rapid progression in patients with an older age of onset, though this was not observed in our cohort. [9][10][11]14 These findings suggest that Black and female patients may represent clinically distinct subgroups within IBM with unique disease trajectories and, potentially, different responses to therapeutic interventions. Although a distinct pattern was not confirmed in patients with a younger age at onset, our analysis was limited by constraining clinical assessments to visits at the JH Myositis Center.…”

Section: Discussionmentioning

confidence: 99%

“…6,7 Of the relatively few studies that have closely examined the clinical symptoms and course of IBM, most have investigated small cohorts (mean 47 patients) with limited follow-up (mean 2•3 years). [8][9][10][11][12][13][14][15][16] Although these studies have described an overall consistent IBM phenotype, there is evidence of substantial variability both within and between studies as regards presentation, clinical progression, and accumulation of debility. While the majority of patients in these studies had an age of onset in their late fifties, a considerable number (5-20%) developed symptoms at less than 50 years of age.…”

Section: Introductionmentioning

confidence: 99%

“…Several studies have shown a more rapid functional decline in patients with older age of onset. [9][10][11]14 However, others found mixed results 13,19 or no correlation between age of onset and progression of weakness, 12 and one study reported a more rapid decline in pinch strength among individuals with a younger age of onset. 15 Oldroyd et al reported a more rapid decline in pinch and knee extensor strength in men, however, sex was not found to have an association with strength decline in other studies.…”

Section: Introductionmentioning

confidence: 99%

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Clinical heterogeneity based on race and sex within a large cohort of inclusion body myositis patients

Michelle

Pinal‐Fernandez

Casal-Domínguez

et al. 2022

Preprint

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Background and Objectives: Sporadic inclusion body myositis (IBM) is the most common acquired myopathy in individuals over age 50. The disorder is slowly progressive and while many therapies have been investigated, response has generally been poor. Clinical heterogeneity may influence treatment responsiveness; however, data regarding heterogeneity in IBM is limited and often conflicting. We aim to identify clinically distinct subgroups within a large IBM cohort, as well as prognostic factors for disease progression. Methods: Clinical, histologic, radiologic, and electrophysiologic data were analyzed for all patients with IBM and other forms of myositis enrolled in a longitudinal cohort from The Johns Hopkins Myositis Center from 2003-2018. Univariate, multivariate, and graphical analyses were used to identify prognostic factors in IBM patients. Results: Among the 335 IBM patients meeting inclusion criteria, 64% were male with an average age of disease onset of 58.7 years and a delay to diagnosis of 5.2 years. Initial misdiagnosis (52%) and immunosuppressant treatment (42%) were common. Less than half (43%) of muscle biopsies demonstrated all three pathologic hallmarks: endomysial inflammation, mononuclear cell invasion, and rimmed vacuoles. Black patients had significantly weaker arm abductors, hip flexors, and knee flexors compared to non-Black patients but were less likely to develop dysphagia. Female patients had stronger finger flexors and knee extensors compared to their male counterparts but were more likely to develop dysphagia. A significant number (20%) of patients had an age of onset less than 50 years. This group of younger patients was weaker at their first visit; however, this may be accounted for by a longer disease duration at first visit. Discussion: Although IBM has long been considered a disorder predominately of older, White men, female, and non-White patients comprise a significant proportion of the IBM population. Our study demonstrates that female and Black patients have distinct clinical phenotypes within the overarching IBM clinical phenotype.

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