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Change in body composition with skeletal muscle wasting, a major component of pulmonary cachexia, is associated with mortality in chronic obstructive pulmonary disease and cancer. However, its relevance in interstitial lung diseases (ILDs) remains unclear. We hypothesized changes in body composition would be associated with mortality in ILDs. We measured the cross-sectional-area (ESM CSA ) and muscle attenuation (ESM MA ) of erector-spinae muscles, as determined by CT-imaging, in patients with idiopathic pulmonary fibrosis (IPF; n = 131) and idiopathic pleuroparenchymal fibroelastosis (iPPFE; n = 43) and controls. Subsequently, implications with prognosis were evaluated. The ESM CSA  of ILD patients, but not ESM MA , was significantly smaller than that in controls. Lower ESM CSA  with decreased BMI were recorded in iPPFE patients versus IPF patients, whilst IPF patients had decreased ESM CSA  without BMI decline. Lower ESM CSA  in IPF patients were associated with poorer prognoses. Conversely, decreased ESM MA  were associated with worse survival in iPPFE patients. Multivariate analyses showed that ESM CSA  in IPF and ESM MA  in iPPFE were independent risk factors for mortality. Distinct changes in body composition had prognostic significance among patients with IPF and iPPFE. Lower ESM CSA  and ESM MA  were independently associated with poor prognosis in IPF and iPPFE, respectively. These results suggest values to measure body composition changes in managing patients with IPF and iPPFE.

Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis