A rearranged form of Epstein-Barr virus DNA is associated with idiopathic pulmonary fibrosis

Am J Respir Crit Care Med. 2002 Aug 15;166(4):510-3. doi: 10.1164/rccm.2103058.


An association between idiopathic pulmonary fibrosis (IPF) and productive Epstein-Barr virus (EBV) infection has been found previously. Productive EBV replication can be associated with a rearrangement in EBV genomes termed WZhet. We hypothesized that WZhet genomes might be present in patients with IPF. Thirty-nine patients with IPF, 26 lung transplant recipients, and 24 normal subjects were studied. When EBV DNA-positive lung tissue biopsies from IPF patients were analyzed, 11 of 18 (61%) were positive for WZhet. Buffy coat DNA analysis showed that 75-85% were EBV DNA-positive in both IPF and control groups. Buffy coat analysis for WZhet was positive in 16 of 27 (59%) IPF patients, compared with none of 32 lung transplant recipients and 1 of 24 (4%) normal blood donors (p < or = 0.001). There was thus a good correlation between the presence of WZhet in lung tissue and peripheral blood. However, there was no significant association between the presence of WZhet and immunosuppressive therapy. These data further confirm the association between active EBV infection and IPF and provide a potential marker in the peripheral blood for the tracking of EBV in this disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blotting, Southern
  • Case-Control Studies
  • Chromosome Mapping
  • DNA, Viral / genetics*
  • DNA-Binding Proteins / genetics
  • Epstein-Barr Virus Infections / blood
  • Epstein-Barr Virus Infections / complications*
  • Epstein-Barr Virus Infections / pathology
  • Female
  • Gene Rearrangement / genetics*
  • Herpesvirus 4, Human / genetics*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Transplantation
  • Male
  • Middle Aged
  • Polymerase Chain Reaction
  • Pulmonary Fibrosis / therapy
  • Pulmonary Fibrosis / virology*
  • Recombination, Genetic / genetics*
  • Trans-Activators / genetics
  • Viral Proteins*
  • Virus Replication / genetics


  • BZLF1 protein, Herpesvirus 4, Human
  • DNA, Viral
  • DNA-Binding Proteins
  • Immunosuppressive Agents
  • Trans-Activators
  • Viral Proteins