Inclusion body myositis (IBM) is a slowly progressing muscle disease of unknown cause that currently has no effective treatment. IBM is the most common inflammatory myopathy in older individuals, with a rising prevalence of 18.2 per 100,000 in adults older than 50.1,2 The disease characteristically affects the quadriceps and finger flexors, and in later stages it can affect diffuse muscle groups, leading to significant physical disability. Dysphagia is a prominent symptom, often resulting from esophageal strictures and muscle weakness, and can lead to recurrent aspirations and malnutrition. The disease progresses slowly, often resulting in a delay in diagnosis, with some studies suggesting a gap of 4–5.6 years between symptom onset and diagnosis.3,4 Consequently, individuals often face significant loss in functional status, psychological distress and may have been exposed to unnecessary or inappropriate interventions by the time they are diagnosed.
Individuals with IBM face significant morbidity, in addition to having complex psychosocial, spiritual and interpersonal needs that would benefit from more robust and comprehensive supportive care. Palliative care focuses on quality of life by addressing physical symptoms, emotional and psychological distress, spiritual/existential distress and financial stressors that accompany serious life-limiting illnesses.5 It incorporates the patient’s values and goals in a multidisciplinary approach that allows for holistic, patient-centered care.
Case Report
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Described as the “life of the party,” Mr. F was a professional pianist, an excellent chef and a compassionate social worker. In his mid-40s, he was experiencing minimal chronic health issues until he gradually began to have difficulty lifting his feet to climb stairs. Over a matter of months, his weakness progressed, and he developed significant fatigue and dysphagia.
During evaluation by his primary care doctor, his lab work demonstrated an elevated creatinine kinase, concerning for ongoing muscle damage, but the etiology of his symptoms was not identified.
Over the next five years, he saw numerous specialists and underwent countless tests while his physical condition deteriorated. He underwent a muscle biopsy that was interpreted as polymyositis and was started on high-dose glucocorticoids for treatment. This failed to make an impact on his muscle strength. He was told not to exercise under the premise of causing further muscle damage, which likely exacerbated his weakness. Due to his progressive symptoms, he presented to the Johns Hopkins Myositis Center for a second opinion and was ultimately diagnosed with IBM based on his clinical history, muscle biopsy and physical exam (see Table 1 and Figures A, B & C).
