Background and aim: Mucine-1 (MUC1) increases in primary lung disease; however, no data are available on pulmonary arterial hypertension (PAH). Our aim was to analyze MUC1 in PAH and a possible link with pulmonary artery pressure (PAPs), PaO2, PaCO2 and cell-mediated immunity. Methods: We studied nine PAH patients (four males and five females, aged 52 ± 21 years). The control groups were nine patients with pulmonary hypertensions due to lung disease (PPH; five males and four females, aged 63 ± 18 years) and 14 patients with left heart disease (HPH; four males and ten females, aged 73 ± 13 years). All underwent arterial gas analysis and echocardiography. A serum sample was collected to determine MUC1 and CD40L values on ELISA. Results: No differences were found for PAPs and CD40L. MUC1 resulted in comparable values between PAH and HPH but decreased when compared to PPH (16.46 ± 4.12 vs 116.6 ± 47.08 U/ml, p = 0.049). pO2 was higher in PAH (PAH 83.18 ± 1.77 vs PPH 62.75 ± 3.23 mmHg, p = 0.003; vs HPH 65.83 ± 6.94 mmHg, p = 0.036). pCO2 was lower compared to PPH (36.15 ± 2.19 vs 45.83 ± 3.00 mmHg, p = 0.026) but not compared to HPH. In PAH patients the MUC1 correlated with pO2 (r = −0.91), pCO2 (r = 0.80), PAPs (r = 0.82) and CD40L (r = 0.72) while it did not in PPH and HPH. Conclusions: These preliminary data show a possible mechanism of immune stimulation in PAH patients. This may imply an association between lung parenchyma, immunity and increase in vascular resistance. Additional studies are required to confirm these findings.
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Galiè N et al (2016) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 37:67–119CrossRefGoogle Scholar
Price LC et al (2012) Inflammation in pulmonary arterial hypertension. Chest 141:210–221CrossRefGoogle Scholar
Santilli F, Basili S, Ferroni P, Davì G (2007) CD40/CD40L system and vascular disease. Intern Emerg Med 2:256–268CrossRefGoogle Scholar
Allanore Y et al (2005) Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers. Ann Rheum Dis 64:481–483CrossRefGoogle Scholar
Ishikawa N, Hattori N, Yokoyama A, Kohno N (2012) Utility of KL-6/MUC1 in the clinical management of interstitial lung diseases. Respir Investig 50:3–13CrossRefGoogle Scholar
Dobrzanski MJ et al (2012) Immunotherapy with IL-10- and IFN-γ-producing CD4 effector cells modulate ‘natural’ and ‘inducible’ CD4 TReg cell subpopulation levels: observations in four cases of patients with ovarian cancer. Cancer Immunol Immunother 61:839–854CrossRefGoogle Scholar
Xu L et al (2013) KL-6 regulated the expression of HGF, collagen and myofibroblast differentiation. Eur Rev Med Pharmacol Sci 17:3073–3077PubMedGoogle Scholar
May AE, Seizer P, Gawaz M (2008) Platelets: inflammatory firebugs of vascular walls. Arterioscler Thromb Vasc Biol 28:s5–s10CrossRefGoogle Scholar
Ueland T et al (2005) Soluble CD40 ligand in acute and chronic heart failure. Eur Heart J 26:1101–1107CrossRefGoogle Scholar
Damås JK et al (2004) Soluble CD40 ligand in pulmonary arterial hypertension: possible pathogenic role of the interaction between platelets and endothelial cells. Circulation 110:999–1005CrossRefGoogle Scholar
Kawabe T, Matsushima M, Hashimoto N, Imaizumi K, Hasegawa Y (2011) CD40/CD40 ligand interactions in immune responses and pulmonary immunity. Nagoya J Med Sci 73:69–78PubMedPubMedCentralGoogle Scholar
Lombardi V, Singh AK, Akbari O (2010) The role of costimulatory molecules in allergic disease and asthma. Int Arch Allergy Immunol 151:179–189CrossRefGoogle Scholar
Cascio S, Finn OJ (2016) Intra-and extra-cellular events related to altered glycosylation of MUC1 promote chronic inflammation, tumor progression, invasion, and metastasis. Biomol Ther 6:1–16Google Scholar