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Autoantibodies against endothelin-1 type A receptor (ET_AR) 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-ET_AR antibodies could be prevalent in endstage CF patients prior to lung transplantation (LTx). Also, ET_AR autoantibodies are frequently associated with autoantibodies against the angiotensin II type 1 receptor (AT₁R). We analyzed the presence of anti-ET_AR 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-ET_AR 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 …