| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Dept of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, CO, USA.
CORRESPONDENCE: M. R. Nicolls, Box C272, 4200 E. 9th Ave, UCHSC, Denver, CO 80262, USA. Fax: 1 3033155632. E-mail: mark.nicolls{at}uchsc.edu
Keywords: Auto-antibodies, autoimmunity, endothelium, immune regulation, immune tolerance, pulmonary hypertension
Received: April 15, 2005
Accepted July 12, 2005
The association between autoimmunity and pulmonary arterial hypertension (PAH) has been appreciated for >40 yrs, but how autoimmune injury might contribute to the pathogenesis of this disease has only been examined in a case-specific manner.
It is becoming increasingly clear that a variety of diverse clinical diseases, ranging from viral infections to connective tissue disorders, can culminate in pulmonary vascular pathology that is indistinguishable. Is there a hitherto unappreciated biology that unites these seemingly unrelated conditions?
The answer to this question may come from the increasing body of evidence concerned with the central importance of regulatory T-cells in preventing inappropriate B-cell activity. Two striking similarities between conditions associated with severe angioproliferative pulmonary hypertension are a defect in the CD4 T-cell compartment and auto-antibody production. Pathogenic auto-antibodies targeting endothelial cells are capable of inducing vascular endothelial apoptosis and may initiate the development of PAH.
The present review will focus on what is known about autoimmune phenomena in pulmonary arterial hypertension patients, in order to better consider whether an early loss of self-tolerance followed by autoimmune injury could influence the early development of severe angioproliferative pulmonary hypertension.
This article has been cited by other articles:
|
|
 |
|
  E. Daley, C. Emson, C. Guignabert, R. de Waal Malefyt, J. Louten, V. P. Kurup, C. Hogaboam, L. Taraseviciene-Stewart, N. F. Voelkel, M. Rabinovitch, et al. Pulmonary arterial remodeling induced by a Th2 immune response J. Exp. Med., February 18, 2008; 205(2): 361 - 372. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. A. Hamidi, S. Prabhakar, and S. I. Said Enhancement of pulmonary vascular remodelling and inflammatory genes with VIP gene deletion Eur. Respir. J., January 1, 2008; 31(1): 135 - 139. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. O. Meyrick, D. B. Friedman, D. D. Billheimer, J. D. Cogan, M. A. Prince, J. A. Phillips III, and J. E. Loyd Proteomics of Transformed Lymphocytes from a Family with Familial Pulmonary Arterial Hypertension Am. J. Respir. Crit. Care Med., January 1, 2008; 177(1): 99 - 107. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Bonnet, G. Rochefort, G. Sutendra, S. L. Archer, A. Haromy, L. Webster, K. Hashimoto, S. N. Bonnet, and E. D. Michelakis The nuclear factor of activated T cells in pulmonary arterial hypertension can be therapeutically targeted PNAS, July 3, 2007; 104(27): 11418 - 11423. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Taraseviciene-Stewart, M. R. Nicolls, D. Kraskauskas, R. Scerbavicius, N. Burns, C. Cool, K. Wood, J. E. Parr, S. A. Boackle, and N. F. Voelkel Absence of T Cells Confers Increased Pulmonary Arterial Hypertension and Vascular Remodeling Am. J. Respir. Crit. Care Med., June 15, 2007; 175(12): 1280 - 1289. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Hagen, K. Fagan, W. Steudel, M. Carr, K. Lane, D. M. Rodman, and J. West Interaction of interleukin-6 and the BMP pathway in pulmonary smooth muscle Am J Physiol Lung Cell Mol Physiol, June 1, 2007; 292(6): L1473 - L1479. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. I. Said, S. A. Hamidi, K. G. Dickman, A. M. Szema, S. Lyubsky, R. Z. Lin, Y.-P. Jiang, J. J. Chen, J. A. Waschek, and S. Kort Moderate Pulmonary Arterial Hypertension in Male Mice Lacking the Vasoactive Intestinal Peptide Gene Circulation, March 13, 2007; 115(10): 1260 - 1268. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. N. Lambrecht and L. M. van den Toorn The pressure mounts on lung dendritic cells Eur. Respir. J., March 1, 2007; 29(3): 435 - 437. [Full Text] [PDF]
|
|
|
|
|
|
F. Perros, P. Dorfmuller, R. Souza, I. Durand-Gasselin, S. Mussot, M. Mazmanian, P. Herve, D. Emilie, G. Simonneau, and M. Humbert Dendritic cell recruitment in lesions of human and experimental pulmonary hypertension Eur. Respir. J., March 1, 2007; 29(3): 462 - 468. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Gomberg-Maitland Learning to pair therapies and the expanding matrix for pulmonary arterial hypertension: is more better? Eur. Respir. J., October 1, 2006; 28(4): 683 - 686. [Full Text] [PDF]
|
|
|
|
|
|
M. C. Tamby, M. Humbert, P. Guilpain, A. Servettaz, N. Dupin, J. J. Christner, G. Simonneau, J. Fermanian, B. Weill, L. Guillevin, et al. Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension Eur. Respir. J., October 1, 2006; 28(4): 799 - 807. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Mouthon, L. Guillevin, and M. Humbert Pulmonary arterial hypertension: an autoimmune disease? Eur. Respir. J., December 1, 2005; 26(6): 986 - 988. [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
