| Popis: |
Investigation of the frequency and progression of ventilatory muscle dysfunction in patients with inclusion body myositis (IBM), the most common myopathy after age 50. Prior research is limited to case series and cross-section studies.A retrospective review of pulmonary function tests, respiratory symptoms, and muscle strength testing.Of the 54 patients reviewed (mean age: 65 ± 9 years and disease duration: 7 ± 7 years), the majority (n = 32, 59%) had restrictive forced vital capacity (FVC) deficits at initial visit. Patients with reduced FVC showed higher prevalence of respiratory symptoms; but age, body mass index, and limb strength were similar when compared to patients without restrictive FVC. Mean rate of FVC decline of 0.108 L/year in IBM patients. Lower baseline limb strength correlated with longer disease duration and future FVC decline (e.g., weaker patients experienced faster decline).Based on FVC, there is a high frequency of ventilatory pump muscle weakness in IBM, which is associated with a higher burden of respiratory symptoms. Baseline strength may indicate risk of respiratory decline and need for vigilant screening. Importantly, ventilatory and limb muscle decline may not progress in a corresponding manner, highlighting the importance of pulmonary function surveillance. |
