title: Idiopathic pulmonary fibrosis a rare disease with severe bone fragility.
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.
authors.affiliation: UOC Internal Medicine, Department of Medicine, Surgery and Neuroscience, University of Siena, Policlinico Le Scotte, Viale Bracci 2, 53100, Siena, Italy. carlacaffarelli@yahoo.it.
authors.forename: Carla
authors.initials: C
authors.lastname: Caffarelli
authors.affiliation: UOC Internal Medicine, Department of Medicine, Surgery and Neuroscience, University of Siena, Policlinico Le Scotte, Viale Bracci 2, 53100, Siena, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Gonnelli
authors.affiliation: UOC Internal Medicine, Department of Medicine, Surgery and Neuroscience, University of Siena, Policlinico Le Scotte, Viale Bracci 2, 53100, Siena, Italy.
authors.forename: Maria Dea
authors.initials: MD
authors.lastname: Tomai Pitinca
authors.affiliation: UOC Internal Medicine, Department of Medicine, Surgery and Neuroscience, University of Siena, Policlinico Le Scotte, Viale Bracci 2, 53100, Siena, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Francolini
authors.affiliation: UOC Respiratory Diseases and Lung Transplantation, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Fui
authors.affiliation: UOC Respiratory Diseases and Lung Transplantation, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Bargagli
authors.affiliation: UOC Respiratory Diseases and Lung Transplantation, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
authors.forename: Rosa Metella
authors.initials: RM
authors.lastname: Refini
authors.affiliation: UOC Respiratory Diseases and Lung Transplantation, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
authors.forename: David
authors.initials: D
authors.lastname: Bennett
authors.affiliation: UOC Internal Medicine, Department of Medicine, Surgery and Neuroscience, University of Siena, Policlinico Le Scotte, Viale Bracci 2, 53100, Siena, Italy.
authors.forename: Ranuccio
authors.initials: R
authors.lastname: Nuti
authors.affiliation: UOC Respiratory Diseases and Lung Transplantation, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Rottoli
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015519:Bone Density / Q000502:physiology; D003430:Cross-Sectional Studies; D005260:Female; D050723:Fractures, Bone / Q000503:physiopathology*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications*; D007558:Italy; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D013131:Spine / Q000503:physiopathology; D014807:Vitamin D / Q000097:blood
chemical_list: D014807:Vitamin D
keywords: Diffusion capacity for carbon monoxide (DLCO); Forced vital capacity (FVC); Idiopathic pulmonary fibrosis; Osteoporosis; Spinal deformity index (SDI); Vertebral fracture