Lung transplantation for idiopathic pulmonary fibrosis

Pavec, Jérôme Le; Dauriat, Gaëlle; Dolidon, Samuel; Hanna, Amir; Feuillet, Séverine; Pradère, Pauline; Crutu, Adrian; Florea, Valentina; Boulate, David; Mitilian, Délphine; Fabre, Dominique; Mussot, Sacha; Mercier, Olaf; Fadel, Élie

doi:10.1016/j.lpm.2020.104026

Cited by 13 publications

(9 citation statements)

References 69 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[86][87][88][89] Although there is no conclusive evidence that transplant outcome is influenced by telomere length, short telomeres may confer vulnerability to bone marrow dysfunction upon administration of immunosuppressive drugs posttransplant. [90][91][92] At this time, telomere length testing and genetic testing in IPF are not routine, but as more tests are performed, we may be able to better tailor immunosuppressants to the patient. 93 Before transplant, antifibrotic treatment is generally maintained and strict adherence to medication regimen is encouraged, even as clinical trials are being conducted to determine if this affects transplant outcome.…”

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

“… 86 , 87 , 88 , 89 Although there is no conclusive evidence that transplant outcome is influenced by telomere length, short telomeres may confer vulnerability to bone marrow dysfunction upon administration of immunosuppressive drugs posttransplant. 90 , 91 , 92 At this time, telomere length testing and genetic testing in IPF are not routine, but as more tests are performed, we may be able to better tailor immunosuppressants to the patient. 93 …”

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

“…Benefits of unilateral transplant are the shorter wait times, easier procedure with lower perioperative complication rate, and the potential to improve the health of two patients from one donor. 90 Data from the United Network for Organ Sharing database and the Organ Procurement and Transplantation network show a better long‐term survival rate for bilateral versus unilateral transplant. 96 , 97 A recent meta‐analysis found no survival advantage with bilateral lung transplant but did document better pulmonary function.…”

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

See 2 more Smart Citations

Idiopathic pulmonary fibrosis: Current and future treatment

Glass

Grossfeld

Renna

et al. 2022

Clinical Respiratory J

145

View full text Add to dashboard Cite

Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea.Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3-5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development.Data Source: The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. Results: Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral

show abstract

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

Section: Nonpharmacologic Therapy: Lung Transplantationmentioning

confidence: 99%

See 1 more Smart Citation

Idiopathic pulmonary fibrosis: Current and future treatment

Glass

Grossfeld

Renna

et al. 2022

Clinical Respiratory J

145

View full text Add to dashboard Cite

show abstract

“…Subclinical bone marrow and liver abnormalities, including significant T-cell immunodeficiency silently existing in these patients, may aggravate post-lung transplantation-immunosuppression-drug toxicities [ 161 ]. Therefore, telomeropathy is now recognized in addition to age, frailty, pulmonary hypertension, cardiovascular risk, and lung cancer as a parameter for which careful consideration must be given to pre-operative optimization, surgical technique, pulmonary rehabilitation, and a tailored immunosuppressive treatment protocol to produce the best post-transplantation outcomes [ 127 , 143 , 162 , 163 , 164 , 165 , 166 ]. Bone-marrow failure presenting as myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) may arise at any timepoint in the disease’s course in almost 10% of patients, especially those younger than 65 years old [ 18 , 19 , 32 , 141 , 167 , 168 , 169 , 170 ].…”

Section: Clinical Implications Of Carriership Of Pathogenic Variation...mentioning

confidence: 99%

Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective

et al. 2022

View full text Add to dashboard Cite

Background: Unraveling the genetic background in a significant proportion of patients with both sporadic and familial IPF provided new insights into the pathogenic pathways of pulmonary fibrosis. Aim: The aim of the present study is to overview the clinical significance of genetics in IPF. Perspective: It is fascinating to realize the so-far underestimated but dynamically increasing impact that genetics has on aspects related to the pathophysiology, accurate and early diagnosis, and treatment and prevention of this devastating disease. Genetics in IPF have contributed as no other in unchaining the disease from the dogma of a “a sporadic entity of the elderly, limited to the lungs” and allowed all scientists, but mostly clinicians, all over the world to consider its many aspects and “faces” in all age groups, including its co-existence with several extra pulmonary conditions from cutaneous albinism to bone-marrow and liver failure. Conclusion: By providing additional evidence for unsuspected characteristics such as immunodeficiency, impaired mucus, and surfactant and telomere maintenance that very often co-exist through the interaction of common and rare genetic variants in the same patient, genetics have created a generous and pluralistic yet unifying platform that could lead to the understanding of the injurious and pro-fibrotic effects of many seemingly unrelated extrinsic and intrinsic offending factors. The same platform constantly instructs us about our limitations as well as about the heritability, the knowledge and the wisdom that is still missing.

show abstract

“…PF with unknown etiology is termed idiopathic pulmonary fibrosis (IPF), whose median survival time from diagnosis is 3~4 years and the incidence continues to rise [ 2 ]. Currently, lung transplantation is the only intervention shown to extend the life expectation of patients with IPF [ 3 ]. Notably, one of the major complications with infection of the novel coronavirus severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is PF, which leads to increased chronic dyspnea and impaired quality of life in patients with COVID-19 [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Melatonin ameliorates bleomycin-induced pulmonary fibrosis via activating NRF2 and inhibiting galectin-3 expression

Lan

Cheng

et al. 2022

Acta Pharmacol Sin

View full text Add to dashboard Cite

Pulmonary fibrosis (PF) is a chronic interstitial lung disease with no effective therapies. Galectin-3 (Gal-3), a marker of oxidative stress, plays a key role in the pathogenesis of PF. Fibroblast-myofibroblast differentiation (FMD) is an important source of fibrotic cells in PF. Previous studies showed that melatonin (MT) exerted anti-fibrotic effect in many diseases including PF through its antioxidant activity. In the present study we investigated the relationships among Gal-3, NRF2, ROS in FMD and their regulation by MT. We established an in vitro model of FMD in TGF-β1-treated human fetal lung fibroblast1 (HFL1) cells and a PF mouse model via bleomycin (BLM) intratracheal instillation. We found that Gal-3 expression was significantly increased both in vitro and in vivo. Knockdown of Gal-3 in HFL1 cells markedly attenuated TGF-β1-induced FMD process and ROS accumulation. In TGF-β1-treated HFL1 cells, pretreatment with NRF2-specific inhibitor ML385 (5 μM) significantly increased the levels of Gal-3, α-SMA and ROS, suggesting that the expression of Gal-3 was regulated by NRF2. Treatment with NRF2-activator MT (250 μM) blocked α-SMA and ROS accumulation accompanied by reduced Gal-3 expression. In BLM-induced PF model, administration of MT (5 mg·kg −1 ·d −1 , ip for 14 or 28 days) significantly attenuated the progression of lung fibrosis through up-regulating NRF2 and down-regulating Gal-3 expression in lung tissues. These results suggest that Gal-3 regulates TGF-β1-induced pro-fibrogenic responses and ROS production in FMD, and MT activates NRF2 to block FMD process by down-regulating Gal-3 expression. This study provides a useful clue for a clinical strategy to prevent PF. Graphic abstract of the mechanisms. MT attenuated BLM-induced PF via activating NRF2 and inhibiting Gal-3 expression.

show abstract

Lung transplantation for idiopathic pulmonary fibrosis

Cited by 13 publications

References 69 publications

Idiopathic pulmonary fibrosis: Current and future treatment

Idiopathic pulmonary fibrosis: Current and future treatment

Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective

Melatonin ameliorates bleomycin-induced pulmonary fibrosis via activating NRF2 and inhibiting galectin-3 expression

Contact Info

Product

Resources

About