Noninvasive assessment of peripheral skeletal muscle weakness in idiopathic pulmonary fibrosis: a pilot study with multiparametric MRI of the rectus femoris muscle

BACKGROUND: To investigate differences in magnetic resonance imaging (MRI) features of rectus femoris muscle between idiopathic pulmonary fibrosis (IPF) patients and healthy volunteers. METHODS: Thirteen IPF patients with GAP Index stage II disease were subjected to pulmonary function tests, 6-minute walk test (6MWT), quadriceps femoris muscle strength measurement and MRI of the thigh at rest. At MRI, muscle cross-sectional areas, T2 and T2* relaxometry, and 3-point Dixon fat fraction were measured. The results were compared to those of eight healthy sedentary volunteers. RESULTS: IPF patients had significantly lower %predicted FVC, FEV(1) and DL(CO) (p<0.001 for the three variables) and walked significantly less in the 6MWT (p=0.008). Mean quadriceps femoris muscle strength also was significantly lower in IPF patients (p=0.041). Rectus femoris muscle T2* measurements were significantly shorter in IPF patients (p=0.027). No significant intergroup difference was found regarding average muscle cross-sectional areas (p=0.790 for quadriceps and p=0.816 for rectus femoris) or rectus femoris fat fraction (p=0.901). Rectus femoris T2 values showed a non-significant trend to be shorter in IPF patients (p=0.055). CONCLUSIONS: Our preliminary findings suggest that, besides disuse atrophy, other factors such as hypoxia (but not inflammation) may play a role in the peripheral skeletal muscle dysfunction observed in IPF patients. This might impact the rehabilitation strategies for IPF patients and warrants further investigation.