Abstract
Patients with Inclusion Body Myositis (IBM) may present with symptoms such as dysarthria and dysphagia, often before a formal diagnosis. Increased awareness and understanding of IBM among clinicians can help identify early signs, leading to timely referrals to specialist teams. Raising awareness of IBMs oral manifestations will empower clinicians to play a key role in the multidisciplinary management of this progressive disease.
1
Introduction
Inflammatory myopathies, such as dermatomyositis (DM) and polymyositis (PM), are rare immune-mediated diseases characterised by muscle weakness. Inclusion Body Myositis (IBM) is the most common inflammatory myopathy affecting individuals over the age of 50 [ ]. The pathogenesis of IBM remains unclear, with debate over whether it is immune-mediated or degenerative. Non-necrotic muscle fibres are invaded by cytotoxic CD8 + T cells and surrounded by CD4 + T cells, suggesting that T-cell activation is part of an antigen-driven response. The degenerative component is supported by the pathological presence of rimmed vacuoles with abnormal protein aggregation.
Characteristic patterns include weakness of knee extension more than hip flexion and finger flexion more than finger extension. Head and neck manifestations can include facial weakness and atypically macroglossia which may restrict movement, leading to difficulty eating and drinking. Oedema, chronic inflammation, and atrophy of oesophageal muscles can lead to dysphagia and an increased risk of aspiration [ ]. Diagnosis is supported by muscle biopsy which shows evidence of inflammation and degenerative changes.
Currently, there is no effective treatment for IBM. While some patients may initially respond to corticosteroids, resistance to therapy generally follows. Given the slow progression of IBM, treatment focuses on symptom management. Physiotherapy (PT) aims to strengthen musculature and compensate for weakness. Occupational therapy (OT) assists with tasks that become difficult due to muscle weakness, such as providing equipment like stairlifts. Speech and Language Therapy (SALT) evaluates swallowing techniques and looks for signs of aspiration [ ].
Although IBM does not seem to directly affect life expectancy, the progressive symptoms, such as loss of ambulation can lead to frequent falls and eventual reliance on a wheelchair [ ]. Early detection by clinicians can lead to quicker referrals and a reduction in the average diagnostic delay currently of 5–6 years [ ].
2
Case report
A 59-year-old female presented with a 2-year history of gradual tongue enlargement, causing slurred speech and difficulty swallowing, following a 1–2-year history of progressive muscle weakness that led to decreased muscle and grip strength. Her medical history included hypertension, arthritis and bronchiectasis with no known drug allergies. She was a non-smoker and consumed one bottle of wine per weekend. Examination revealed a soft, enlarged tongue without cervical lymphadenopathy ( Fig. 1 ). She expressed significant distress over the lack of diagnosis, as her symptoms were severely impacting her quality of life.
