Experience With Nintedanib in Severe Pulmonary Fibrosis Associated With Systemic Sclerosis: A Case Series

Bordas-Martínez, Jaume; Llanos-González, Ana Belén; Jódar-Masanés, R.; Vicens-Zygmunt, Vanesa; Bermudo, Guadalupe; Luburich, Patricio; Dorca, Jordi; Molina-Molina, María; Suárez-Cuartín, Guillermo

doi:10.1016/j.opresp.2020.100080

Cited by 3 publications

(6 citation statements)

References 13 publications

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“…Bordas-Martinez et al (19) hypothesized that patients with severe progressive fibrotic SSc-ILD (FVC <40% predicted or DLCO <30% of predicted) who are candidates for lung transplant may also benefit from nintedanib therapy. In this limited series of 4 cases with progressive fibrosing SSc-ILD, patients were treated with mycophenolate (2x720 mg/day) and nintedanib (2x150 mg/day).…”

Section: Nintedanib In Severe Progressive Fibrotic Ssc-ildmentioning

confidence: 99%

Systemic sclerosis related interstitial lung disease and nintedanib

Atilla

Çetin

Okyar

et al. 2022

Journal of Medicine and Palliative Care

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Although many mechanisms leading to lung fibrosis in systemic sclerosis-associated interstitial lung disease (SSc-ILD) have been suggested, this issue has not been fully understood yet. Recently, there has been increased evidence that the mediators and pathological mechanisms responsible for idiopathic pulmonary fibrosis (IPF) are similar to those in SSc-ILD. Accordingly, studies have been conducted to support that antifibrotic agents used in the treatment of IPF may also be useful in SSc-ILD. There are currently two antifibrotic agents on the market, namely nintedanib and pirfenidon. Although studies on the use of pirfenidone in SSc-ILD are not satisfactory, nintedanib studies have yielded positive results. The SENSCIS (Safety and Efficacy of Nintedanib in Systemic Sclerosis) study is the first and most comprehensive Phase III study on this subject. In 2019, the results of SENSCIS trial showed that, nintedanib significantly reduced the annual decline in lung function in SSc-ILD. After this trial, which did not include SSc-ILD patients with severe lung function loss, nintedanib licenced for the treatment of SSc-ILD worldwide. However, the currently available literature data lacks information about long-term effects and side effects of nintedanibe on SSc-ILD an also about the advanced SSc-ILD. The aim of this study is to review SSc-ILD patients treated with nintedanib, by also mentioning the pathogenesis of this disease according to the current literature.

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Section: Nintedanib In Severe Progressive Fibrotic Ssc-ildmentioning

confidence: 99%

Systemic sclerosis related interstitial lung disease and nintedanib

Atilla

Çetin

Okyar

et al. 2022

Journal of Medicine and Palliative Care

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“…A case series published by Martinez et al studied the effect of nintedanib in four patients with severe, progressive fibrotic SSc-ILD who were undergoing evaluation for lung transplant [ 20 ]. The SENSCIS trial had excluded patients with severe lung fibrosis with FVC less than 40% and DLCO less than 30% of the predicted value, those with severe pulmonary hypertension, and those awaiting lung transplant [ 20 ]. Despite their severe disease, these four patients were started on nintedanib, and the functional decline of their pulmonary function under nintedanib was evaluated [ 20 ].…”

Section: Reviewmentioning

confidence: 99%

“…The SENSCIS trial had excluded patients with severe lung fibrosis with FVC less than 40% and DLCO less than 30% of the predicted value, those with severe pulmonary hypertension, and those awaiting lung transplant [ 20 ]. Despite their severe disease, these four patients were started on nintedanib, and the functional decline of their pulmonary function under nintedanib was evaluated [ 20 ]. The descriptive data obtained from these patients showed that two out of four patients had significant improvement in FVC, and the other two had a decrease in the decline of FVC and DLCO after initiation of nintedanib [ 20 ].…”

Section: Reviewmentioning

confidence: 99%

“…Despite their severe disease, these four patients were started on nintedanib, and the functional decline of their pulmonary function under nintedanib was evaluated [ 20 ]. The descriptive data obtained from these patients showed that two out of four patients had significant improvement in FVC, and the other two had a decrease in the decline of FVC and DLCO after initiation of nintedanib [ 20 ].…”

Section: Reviewmentioning

confidence: 99%

“…The SENSCIS is the only RCT study to date that has assessed nintedanib's efficacy in patients with SSc-ILD [ 17 ]. The effectiveness of nintedanib in the management of SSc-ILD is evident from these studies [ 16 , 17 , 20 ], making it an FDA-approved drug to treat this disease [ 21 ]. It decreases disease progression by various mechanisms and ultimately helps in the improvement of the health condition of patients suffering from SSc-ILD.…”

Section: Reviewmentioning

confidence: 99%

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Systemic Sclerosis Associated Interstitial Lung Disease and Nintedanib: A Rare Disease and a Promising Drug

Kafle¹,

Magar²,

Patel³

et al. 2021

Cureus

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Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a rare disease with a progressive nature, eventually leading to lung fibrosis. Nintedanib, a tyrosine kinase inhibitor, is a widely accepted drug for treating idiopathic pulmonary fibrosis (IPF), a disease that shares some similarities with SSc-ILD regarding pathological disease processes. In this review, we aim to discuss the pathogenesis of SSc-ILD and the overall role of nintedanib in the management of SSc-ILD. SSc-ILD involves multiple pathological mediators contributing to various pathways that ultimately cause lung fibrosis. The pathogenesis of SSc-ILD is a complex phenomenon and still needs further study. Nintedanib has demonstrated its efficacy in the treatment of SSc-ILD by reducing the progression of the pathological process. It has also proven its clinical significance in the management of SSc-ILD. However, the currently available literature does not have any evidence to compare the effectiveness of nintedanib with the already available treatment modalities such as cyclophosphamide (CYC), mycophenolate mofetil (MMF), and azathioprine (AZT). The current literature also lacks information about nintedanib's long-term consequences on patients with SSc-ILD. Therefore, to create better evidence-based treatment guidelines, we recommend that researchers conduct randomized clinical trials comparing nintedanib to MMF, CYC, AZT, etc., and continue surveillance to explore the longterm consequences of nintedanib.

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