Idiopathic Pulmonary Fibrosis Related to Endothelial Injury and Antiendothelial Cell Antibodies

Magro, Cynthia M.; Waldman, W. James; Knight, Deborah; Allen, James N.; Nádasdy, Tibor; Frambach, Gwyn E.; Ross, Patrick; Marsh, Clay B.

doi:10.1016/j.humimm.2006.02.026

Cited by 77 publications

(76 citation statements)

References 51 publications

(36 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recently, Margo et al indicated the presence of AECA in IPF patients [31]. In contrast to our study, twelve sera from 18 patients reacted with HUVEC.…”

contrasting

confidence: 99%

Interstitial pneumonia

Kurasawa

Fukuda²

2017

Reactions Weekly

View full text Add to dashboard Cite

show abstract

“…Recently, Margo et al indicated the presence of AECA in IPF patients [31]. In contrast to our study, twelve sera from 18 patients reacted with HUVEC.…”

contrasting

confidence: 99%

Interstitial pneumonia

Kurasawa

Fukuda²

2017

Reactions Weekly

View full text Add to dashboard Cite

show abstract

“…Thus, there are numerous biological precedents that could plausibly explain the development of autoimmunity in IPF as a secondary consequence of another, distinctly different lung injury(ies) that actually triggers the disease. Nonetheless, at least some of the many autoimmune responses that have been discovered in IPF patients, in addition to the antivimentin in this study, seem to play important roles in disease severity and progression (2,(4)(5)(6)(7)(8)(9)(10)(13)(14)(15)(16). In addition, pathogenic secondary immune responses could cause or contribute to the well-known resistance of IPF to conventional therapies, because many other autoantibody-mediated lung diseases are similarly refractory to nonspecific agents, including glucocorticoids (8).…”

Section: Discussionmentioning

confidence: 93%

“…The prognosis of IPF is poor, with median survival ∼ 3 y, and the only currently approved medications have modest efficacy. Autoimmunity has been implicated in the development and/or progression of IPF, although many details of these immune responses remain enigmatic (2)(3)(4)(5)(6)(7)(8).…”

mentioning

confidence: 99%

Autoimmunity to Vimentin Is Associated with Outcomes of Patients with Idiopathic Pulmonary Fibrosis

Surolia

et al. 2017

The Journal of Immunology

View full text Add to dashboard Cite

Autoimmunity has been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF); however, the repertoire of autoantigens involved in this disease and the clinical relevance of these autoimmune responses are still being explored. Our initial discovery assays demonstrated that circulating and intrapulmonary vimentin levels are increased in IPF patients. Subsequent studies showed native vimentin induced HLA-DR–dependent in vitro proliferation of CD4 T cells from IPF patients and enhanced the production of IL-4, IL-17, and TGF-β1 by these lymphocytes in contrast to normal control specimens. Vimentin supplementation of IPF PBMC cultures also resulted in HLA-DR–dependent production of IgG with anti-vimentin specificities. Circulating anti-vimentin IgG autoantibody levels were much greater in IPF subjects from the University of Alabama at Birmingham (n = 102) and the University of Pittsburgh (U. Pitt., n = 70) than in normal controls. Anti-vimentin autoantibody levels in IPF patients were HLA biased and inversely correlated with physiological measurements of lung function (i.e., forced expiratory volumes and diffusing capacities). Despite considerable intergroup differences in transplant-free survival between these two independent IPF cohorts, serious adverse outcomes were most frequent among the patients within each population that had the highest anti-vimentin autoantibody levels (University of Alabama at Birmingham: hazard ratio 2.5, 95% confidence interval 1.2–5.3, p = 0.012; University of Pittsburgh: hazard ratio 2.7, 95% confidence interval 1.3–5.5, p = 0.006). These data show that anti-vimentin autoreactivity is prevalent in IPF patients and is strongly associated with disease manifestations. These findings have implications with regard to the pathogenesis of this enigmatic disease and raise the possibility that therapies specifically directed at these autoimmune processes could have therapeutic efficacy.

show abstract

“…Endothelial injury and apoptosis are believed to contribute to the pathogenesis of fibrotic skin and lung disease (2,21). Much of what is known about the role of endothelial cells in fibrotic disease comes from in vitro studies or from serum markers in human fibrotic diseases.…”

Section: Endothelial Cells Are Damaged In Response To the Subcutaneoumentioning

confidence: 99%

Endothelial Cells Recruit Macrophages and Contribute to a Fibrotic Milieu in Bleomycin Lung Injury

Leach¹,

Chrobak

Han

et al. 2013

Am J Respir Cell Mol Biol

View full text Add to dashboard Cite

Idiopathic Pulmonary Fibrosis Related to Endothelial Injury and Antiendothelial Cell Antibodies

Cited by 77 publications

References 51 publications

Interstitial pneumonia

Interstitial pneumonia

Autoimmunity to Vimentin Is Associated with Outcomes of Patients with Idiopathic Pulmonary Fibrosis

Endothelial Cells Recruit Macrophages and Contribute to a Fibrotic Milieu in Bleomycin Lung Injury

Contact Info

Product

Resources

About