Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. Academic Article Article uri icon

Overview

MeSH

  • Adult
  • Aged
  • Autoantigens
  • Cell Proliferation
  • Cytokines
  • Female
  • Humans
  • Lung
  • Lymph Nodes
  • Male
  • Middle Aged

MeSH Major

  • Antibody Formation
  • Autoantibodies
  • Autoimmunity
  • CD4-Positive T-Lymphocytes
  • Immunoglobulin G
  • Pulmonary Fibrosis

abstract

  • Idiopathic pulmonary fibrosis (IPF) is a morbid, refractory lung disorder with an unknown pathogenesis. To investigate potential adaptive immune mechanisms in IPF, we compared phenotypes and effector functions of peripheral CD4 T cells, autoantibody production, and proliferative responses of pulmonary hilar lymph node CD4 T cells to autologous lung extracts from afflicted patients and normals. Our results show that greater proportions of peripheral CD4 T lymphocytes in IPF subjects expressed MHC class II and CD154 (CD40L), and they more frequently elaborated TGF-beta1, IL-10, and TNF-alpha. Abnormal CD4 T cell clonal expansions were found in all IPF patients, and 82% of these subjects also had IgG autoantibodies against cellular Ags. IPF lung extracts stimulated proliferations of autologous CD4 T cells, unlike preparations from normals or those with other lung diseases, and the IPF proliferative responses were enhanced by repeated cycles of stimulation. Thus, CD4 T cells from IPF patients have characteristics typical of cell-mediated pathologic responses, including augmented effector functions, provision of facultative help for autoantibody production, oligoclonal expansions, and proliferations driven by an Ag present in diseased tissues. Recognition that an autoreactive immune process is present in IPF can productively focus efforts toward identifying the responsible Ag, and implementing more effective therapies.

publication date

  • August 15, 2007

has subject area

  • Adult
  • Aged
  • Antibody Formation
  • Autoantibodies
  • Autoantigens
  • Autoimmunity
  • CD4-Positive T-Lymphocytes
  • Cell Proliferation
  • Cytokines
  • Female
  • Humans
  • Immunoglobulin G
  • Lung
  • Lymph Nodes
  • Male
  • Middle Aged
  • Pulmonary Fibrosis

Research

keywords

  • Clinical Trial
  • Comparative Study
  • Journal Article

Identity

Language

  • eng

PubMed ID

  • 17675522

Additional Document Info

start page

  • 2592

end page

  • 2599

volume

  • 179

number

  • 4