Anti-ETAR and anti-AT1R autoantibodies are elevated in patients with endstage cystic fibrosis

Autor: E.A. van de Graaf, D. Dragun, Henderikus G. Otten, J.M. Kwakkel-van Erp, T. Hoefnagel, D.A. van Kessel, Kevin Budding, Cornelis E. Hack
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Male
medicine.medical_treatment
Pilot Projects
Severity of Illness Index
AT1R
Cystic fibrosis
Pediatrics
Cohort Studies
Pulmonary Disease
Chronic Obstructive
ETAR
Medicine(all)
COPD
Interstitial lung disease
Middle Aged
Prognosis
Receptor
Endothelin A
Perinatology
and Child Health
Lung transplantation
Female
Adult
Pulmonary and Respiratory Medicine
Adolescent
Bronchiolitis obliterans
Enzyme-Linked Immunosorbent Assay
Risk Assessment
Receptor
Angiotensin
Type 1
Young Adult
medicine
Journal Article
Humans
Comparative Study
Pediatrics
Perinatology
and Child Health
Retrospective Studies
Autoantibodies
business.industry
Patient Selection
Autoantibody
medicine.disease
Angiotensin II
Pulmonary hypertension
ATR
Pediatrics
Perinatology and Child Health
Immunology
Lung Diseases
Interstitial
business
Biomarkers
ETR
Zdroj: Journal of Cystic Fibrosis, 14(1), 42. Elsevier
ISSN: 1569-1993
Popis: Autoantibodies against endothelin-1 type A receptor (ETAR) are present in systemic sclerosis complicated by lung fibrosis and pulmonary hypertension. As increased serum levels and local overproduction of endothelin-1 in the airways are reported in cystic fibrosis (CF) patients, we reasoned that anti-ETAR antibodies could be prevalent in endstage CF patients prior to lung transplantation (LTx). Also, ETAR autoantibodies are frequently associated with autoantibodies against the angiotensin II type 1 receptor (AT1R). We analyzed the presence of anti-ETAR and anti-AT1R autoantibodies in 43 LTx patients (chronic obstructive pulmonary disease (COPD), n=20; CF, n=13; interstitial lung disease (ILD), n=1). We observed overall higher anti-ETAR and anti-AT1R autoantibody titers in sera taken prior to LTx in the CF patient group as compared to COPD. No difference was found in autoantibody levels between patients with CF versus ILD. In sera taken post-LTx we found the same difference in anti-ETAR and anti-AT1R autoantibody titers between patients with CF versus COPD. No difference was found in antibody titers between sera taken prior to or 6months after LTx. There was no association between autoantibody levels and other relevant demographic parameters, and we found no association between autoantibody titers and the development of the bronchiolitis obliterans syndrome. Both autoantibody titers were strongly correlated. We hypothesize that due to prolonged exposure to bacterial infection, increased levels of AT1R and ETAR result in a deregulated immune response causing autoantibody formation. Further research is expedient to elucidate the occurrence of autoantibodies against ETAR and AT1R and their role in disease progression.
Databáze: OpenAIRE
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