Academic Journal
Lysyl oxidase—a possible role in systemic sclerosis–associated pulmonary hypertension: a multicentre study
| العنوان: | Lysyl oxidase—a possible role in systemic sclerosis–associated pulmonary hypertension: a multicentre study |
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| المؤلفون: | Vadasz, Zahava, Balbir Gurman, Alexandra, Meroni, Pierluigi, Farge, Dominique, Levi, Yair, Ingegnoli, Francesca, Braun-Moscovici, Yolada, Rosner, Itzhak, Slobodin, Gleb, Rozenbaum, Michael, Jiries, Nizar, Kaly, Lisa, Boulman, Nina, Zilber, Karina, Ginsberg, Shira, Awisat, Abid, Goldberg, Yair, Lurie, Michael, Ghigna, Maria-Rosa, Guignabert, Christophe, Humbert, Marc, Rimar, Doron |
| المساهمون: | Allergy, Clinical Immunology and Rheumatology, Università degli Studi di Milano = University of Milan (UNIMI), Service de Médecine Interne Saint-Louis, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal Paris, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), McGill University = Université McGill Montréal, Canada, Center of reference for rare systemic autoimmune diseases (FAI2R), Bnai Zion Medical Center, Partenaires INRAE, Technion - Israel Institute of Technology Haifa, Bnei Zion Hospital Haifa, Centre Chirurgical Marie Lannelongue (CCML), Hypertension pulmonaire : physiopathologie et innovation thérapeutique (HPPIT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique (HPPIT), Université Paris-Sud - Paris 11 (UP11)-Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Université Paris-Saclay |
| المصدر: | ISSN: 1462-0324. |
| بيانات النشر: | HAL CCSD Oxford University Press (OUP) |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | [SDV]Life Sciences [q-bio] |
| الوصف: | International audience ; Abstract Objective Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. Methods A total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with primary RP (PRP) and 80 healthy controls. LOX serum levels were determined by ELISA. Five lung and 12 skin biopsies from SSc patients were stained for LOX and compared with controls. Results Serum levels of LOX in SSc were significantly higher than in VEDOSS, PRP and healthy controls (P < 0.001). LOX inversely correlated with the diffusing capacity of the lung for carbon monoxide diffusing capacity (DLCO) in diffuse SSc (r = −0.376, P = 0.02). Patients with moderate to severe estimated systolic PAH had higher LOX levels (P < 0.01). Lung biopsies demonstrated intense LOX staining in SSc patients with PAH that was predominantly located in the endothelium of the remodelled pulmonary vessels. Conclusion Serum LOX levels are increased in established SSc and inversely correlate with the DLCO. LOX is elevated in patients with moderate to severe PAH and is located in the proliferating endothelium in lung arterioles, suggesting a possible role for LOX in SSc-associated PAH. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1093/rheumatology/kez035 |
| الاتاحة: | https://hal.science/hal-04526702 https://doi.org/10.1093/rheumatology/kez035 |
| رقم الانضمام: | edsbas.609BBF6D |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/rheumatology/kez035 |
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