Lower ESM CSA and ESM MA were independently associated with poor prognosis in IPF and iPPFE, respectively. Distinct changes in body composition had prognostic significance among patients with IPF and iPPFE.

Multivariate analyses showed that ESM CSA in IPF and ESM MA in iPPFE were independent risk factors for mortality. Conversely, decreased ESM MA were associated with worse survival in iPPFE patients. Lower ESM CSA in IPF patients were associated with poorer prognoses. Lower ESM CSA with decreased BMI were recorded in iPPFE patients versus IPF patients, whilst IPF patients had decreased ESM CSA without BMI decline. The ESM CSA of ILD patients, but not ESM MA, was significantly smaller than that in controls. Subsequently, implications with prognosis were evaluated. 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.

We hypothesized changes in body composition would be associated with mortality in ILDs. However, its relevance in interstitial lung diseases (ILDs) remains unclear. 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.

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