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Immunoglobulin free light chains are increased in hypersensitivity pneumonitis and idiopathic pulmonary fibrosis.

AbstractBACKGROUND:
Idiopathic pulmonary fibrosis (IPF), a devastating lung disorder of unknown aetiology, and chronic hypersensitivity pneumonitis (HP), a disease provoked by an immunopathologic reaction to inhaled antigens, are two common interstitial lung diseases with uncertain pathogenic mechanisms. Previously, we have shown in other upper and lower airway diseases that immunoglobulin free light chains (FLCs) are increased and may be involved in initiating a local inflammation. In this study we explored if such a mechanism may also apply to HP and IPF.
METHODS:
In this study we examined the presence of FLC in serum and BAL fluid from 21 IPF and 22 HP patients and controls. IgG, IgE and tryptase concentrations were measured in BAL fluid only. The presence of FLCs, plasma cells, B cells and mast cells in lung tissue of 3 HP and 3 IPF patients and 1 control was analyzed using immunohistochemistry.
RESULTS:
FLC concentrations in serum and BAL fluid were increased in IPF and HP patients as compared to control subjects. IgG concentrations were only increased in HP patients, whereas IgE concentrations were comparable to controls in both patient groups. FLC-positive cells, B cells, plasma cells, and large numbers of activated mast cells were all detected in the lungs of HP and IPF patients, not in control lung.
CONCLUSION:
These results show that FLC concentrations are increased in serum and BAL fluid of IPF and HP patients and that FLCs are present within affected lung tissue. This suggests that FLCs may be involved in mediating pathology in both diseases.
AuthorsTom Groot Kormelink, Annie Pardo, Karen Knipping, Ivette Buendía-Roldán, Carolina García-de-Alba, Bart R Blokhuis, Moises Selman, Frank A Redegeld
JournalPloS one (PLoS One) Vol. 6 Issue 9 Pg. e25392 ( 2011) ISSN: 1932-6203 [Electronic] United States
PMID21980441 (Publication Type: Journal Article)
Chemical References
  • Immunoglobulin G
  • Immunoglobulin Light Chains
  • Immunoglobulin E
  • Tryptases
Topics
  • Adult
  • Aged
  • Alveolitis, Extrinsic Allergic (blood, immunology, metabolism, physiopathology)
  • Bronchoalveolar Lavage Fluid
  • Case-Control Studies
  • Female
  • Gene Expression Regulation
  • Humans
  • Idiopathic Pulmonary Fibrosis (blood, immunology, metabolism, physiopathology)
  • Immunoglobulin E (metabolism)
  • Immunoglobulin G (metabolism)
  • Immunoglobulin Light Chains (blood, metabolism)
  • Lung (immunology, physiopathology)
  • Male
  • Mast Cells (metabolism)
  • Middle Aged
  • Plasma Cells (metabolism)
  • Respiratory Function Tests
  • Tryptases (metabolism)

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