Perrotta Fabio, Sanduzzi Zamparelli Stefano, D'Agnano Vito, Montella Antonia, Fomez Ramona, Pagliaro Raffaella, Schiattarella Angela, Cazzola Mario, Bianco Andrea, Mariniello Domenica Francesca
Department of Translational Medical Sciences, University of Campania "L. Vanvitelli", 80131 Naples, Italy.
Unit of Respiratory Medicine "L. Vanvitelli", A.O. dei Colli, Monaldi Hospital, 80131 Naples, Italy.
Biomedicines. 2024 Jun 21;12(7):1384. doi: 10.3390/biomedicines12071384.
Idiopathic pulmonary fibrosis (IPF) has traditionally been considered the archetype of progressive fibrotic interstitial lung diseases (f-ILDs), but several other f-ILDs can also manifest a progressive phenotype. Integrating genomic signatures into clinical practice for f-ILD patients may help to identify patients predisposed to a progressive phenotype. In addition to the risk of progressive pulmonary fibrosis, there is a growing body of literature examining how pharmacogenomics influences treatment response, particularly regarding the efficacy and safety profiles of antifibrotic and immunomodulatory agents. In this narrative review, we discuss current studies in IPF and other forms of pulmonary fibrosis, including systemic autoimmune disorders associated ILDs, sarcoidosis and hypersensitivity pneumonitis. We also provide insights into the future direction of research in this complex field.
特发性肺纤维化(IPF)传统上被认为是进行性纤维化间质性肺疾病(f-ILDs)的典型代表,但其他几种f-ILDs也可表现出进行性表型。将基因组特征整合到f-ILD患者的临床实践中,可能有助于识别易出现进行性表型的患者。除了进行性肺纤维化的风险外,越来越多的文献探讨了药物基因组学如何影响治疗反应,特别是抗纤维化和免疫调节药物的疗效和安全性。在这篇叙述性综述中,我们讨论了IPF和其他形式肺纤维化的当前研究,包括与系统性自身免疫性疾病相关的ILDs、结节病和过敏性肺炎。我们还对这一复杂领域未来的研究方向提供了见解。