Idiopathic pulmonary fibrosis a rare disease with severe bone fragility

Intern Emerg Med. 2016 Dec;11(8):1087-1094. doi: 10.1007/s11739-016-1501-z. Epub 2016 Jul 8.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a rare form of chronic, progressive fibrosing interstitial pneumonia of unknown cause. The aim of this cross-sectional study was to assess the prevalence of osteoporosis and fragility fracture in a population of adults with IPF and to identify whether any possible clinical and pulmonary function parameters may be associated with increased bone fragility. In 58 IPF patients (mean age 65.1 ± 9.1 years), we measured bone mineral density (BMD) of the lumbar spine, the femoral neck, and the entire hip. Moreover, the presence of vertebral fractures on a lateral chest X-ray study was evaluated, and a vertebral fracture burden was quantified using the spinal deformity index (SDI). As expected, osteoporosis was significantly more frequent in females with respect to males (57.9 vs 20.5 %, respectively), whereas the fractures prevailed in males with respect to females (38.5 vs 26.3 %, respectively). There were positive correlations between BMD at all skeletal sites and respiratory parameters; in particular for FVC % and DLCO % with BMD at femoral sub-regions. Moreover, we compared the average of DLCO (%) measure by values of SDI score that was higher in those patients with lower values of DLCO (%). The study shows a high prevalence of fragility with vertebral fractures in IPF patients, especially in males. Moreover, the vertebral fracture burden is associated with a worsening of FVC (%) and DLCO (%). Therefore, an evaluation of bone status is recommended, especially in those patients who are candidates for lung transplantation.

Keywords: Diffusion capacity for carbon monoxide (DLCO); Forced vital capacity (FVC); Idiopathic pulmonary fibrosis; Osteoporosis; Spinal deformity index (SDI); Vertebral fracture.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Bone Density / physiology
  • Cross-Sectional Studies
  • Female
  • Fractures, Bone / physiopathology*
  • Humans
  • Idiopathic Pulmonary Fibrosis / complications*
  • Italy
  • Lung / physiopathology*
  • Male
  • Middle Aged
  • Prevalence*
  • Spine / physiopathology
  • Vitamin D / blood

Substances

  • Vitamin D