DSP rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study
| العنوان: | DSP rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study |
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| المؤلفون: | Vladimira Lostakova, Radka Bittenglova, Simona Littnerová, Pavlína Lisá, Eva Kriegova, Tomas Snizek, Hana Suldova, Michael Doubek, Martina Plačková, Martina Sterclova, Pavlina Musilova, Martina Vasakova, Lenka Šišková, Vladimir Bartos, Martina Doubková, Jana Psikalova, Petra Schneiderova, Monika Zurkova |
| المصدر: | Ther Adv Respir Dis Therapeutic Advances in Respiratory Disease, Vol 15 (2021) |
| بيانات النشر: | SAGE Publications, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Pulmonary and Respiratory Medicine, Oncology, medicine.medical_specialty, Single-nucleotide polymorphism, 03 medical and health sciences, Idiopathic pulmonary fibrosis, chemistry.chemical_compound, Diseases of the respiratory system, 0302 clinical medicine, Internal medicine, medicine, Pharmacology (medical), 030304 developmental biology, Original Research, 0303 health sciences, biology, RC705-779, business.industry, Desmoplakin, Pirfenidone, medicine.disease, 3. Good health, 030228 respiratory system, chemistry, biology.protein, Nintedanib, business, medicine.drug |
| الوصف: | Background: The antifibrotic drugs nintedanib and pirfenidone are used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the association of common profibrotic polymorphisms in MUC5B (mucin 5B, rs35705950) and DSP (desmoplakin, rs2076295) on antifibrotic treatment outcomes in IPF. Methods: MUC5B rs35705950 and DSP rs2076295 were assessed in IPF patients ( n = 210, 139 men/71 women) from the Czech EMPIRE registry and age- or sex-matched healthy individuals ( n = 205, 125 men/80 women). Genetic data were collated with overall survival (OS), acute exacerbation episodes, worsening lung function and antifibrotic treatment. Results: We confirmed overexpression of the MUC5B rs35705950*T allele (55.2% versus 20.9%, p CO (diffuse lung capacity) at the IPF diagnosis were associated with survival. Conclusion: Our real-world study showed that IPF patients with MUC5B T* allele or DSP G* allele profit from antifibrotic treatment by lower mortability. Moreover, carriers of the DSP rs2076295*G allele benefit from treatment with nintedanib, and TT genotype from treatment with pirfenidone. MUC5B rs35705950 did not impact the outcome of treatment with either nintedanib or pirfenidone. Our single-registry pilot study should be confirmed with an independent patient cohort. |
| اللغة: | English |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::085ba847fd6bdfb3daff573e1c43b896Test https://europepmc.org/articles/PMC8442489Test/ |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....085ba847fd6bdfb3daff573e1c43b896 |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |