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Lookup NU author(s): Dr James Miller
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OBJECTIVES: Sporadic inclusion body myositis (IBM) is the most common acquired myopathy in those aged above 50. It is classically heralded by weakness in the long finger flexors and quadriceps. The aim of this article is to describe five atypical cases of IBM, outlining two potential emerging clinical subsets of the disease. METHODS: We reviewed relevant clinical documentation and pertinent investigations for five patients with IBM. RESULTS: The first phenotype we describe is young-onset IBM in two patients who had symptoms since their early thirties. The literature supports that IBM can rarely present in this age range or younger. We describe a second phenotype in three middle-aged women who developed early bilateral facial weakness at presentation in tandem with dysphagia and bulbar impairment followed by respiratory failure requiring non-invasive ventilation (NIV). Within this group, two patients were noted to have macroglossia, another possible rare feature of IBM. CONCLUSIONS: Despite the classical phenotype described within the literature IBM can present in a heterogenous fashion. It is important to recognise IBM in younger patients and investigate for specific associations. The described pattern of facial diplegia, severe dysphagia, bulbar dysfunction and respiratory failure in female IBM patients requires further characterisation. Patients with this clinical pattern may require more complex and supportive management. Macroglossia is a potentially under recognised feature of IBM. The presence of macroglossia in IBM warrants further study, as its presence may lead to unnecessary investigations and delay diagnosis.
Author(s): Salam S, Morrow JM, Howard R, Miller JAL, Quinlivan RM, Machado PM
Publication type: Review
Publication status: Published
Journal: Clinical and Experimental Rheumatology
Year: 2023
Volume: 41
Issue: 2
Pages: 340-347
Print publication date: 01/02/2023
Online publication date: 01/03/2023
Acceptance date: 13/02/2023
ISSN (print): 0392-856X
ISSN (electronic): 1593-098X
Publisher: NLM (Medline)
URL: https://doi.org/10.55563/clinexprheumatol/jq7zxd
DOI: 10.55563/clinexprheumatol/jq7zxd
PubMed id: 36861744
