Effectiveness of Lung Transplantation in Patients With Interstitial Lung Diseases

Stącel, Tomasz; Nęcki, Mirosław; Antończyk, Remigiusz; Latos, Magdalena; Urlik, Maciej; Kościołek, Joanna; Kordylewska-Kubus, Angelika; Litewka, Joanna; Przybyłowski, Piotr; Zawadzki, Fryderyk; Wajda-Pokrontka, Marta; Pyrć, Krzysztof; Zembala, Marian; Ochman, Marek

doi:10.1016/j.transproceed.2020.03.050

Cited by 3 publications

(1 citation statement)

References 14 publications

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“…The survival rate varies significantly among patients with non-IPF ILD and exceeds that of IPF by many years, especially in cases of sarcoidosis, connective tissue disease, NSIP and occupational ILD (14). According to the International Society of Heart and Lung Transplantation, the median survival rate after a lung transplant is 5.2 years for patients with idiopathic interstitial lungs and almost 7 years for ILDs that are not idiopathic (15). In this case, depending on the follow-up, if the progression is maintained, lung transplantation should be considered as an viable option.…”

Section: Discussionmentioning

confidence: 99%

Interstitial lung disease with genetic susceptibility: case report

Ana,

Trușculescu

2022

Pneumologia

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Introduction Interstitial lung disease (ILD) includes more than 200 progressive conditions classified based on common clinical, imaging or pathophysiological factors. Case presentation A 37-year-old male, former smoker, with unknown exposure and a family history of incompletely identified ILD, underwent functional and imaging investigations that raise the suspicion of an ILD with a pattern of non-specific interstitial pneumonia (NSIP). High-resolution computer tomography (HRCT) imaging detects the progression of lesions. The severely altered functional status does not allow a lung biopsy to be performed to elucidate the aetiology and establish the optimal therapeutic approach. Bronchoscopy with bronchial aspirate sampling and bronchoalveolar lavage does not suggest a specific ILD aetiology. A diagnosis of diffuse fibrosing and progressive ILD – an unclassifiable phenotype – was established, and after a multidisciplinary discussion, antifibrotic treatment was initiated. A genetic test was performed for a possible familial ILD with a genetic component. The test identified the presence of an autosomal recessive combined immunodeficiency due to NF-κB inducing kinase (NIK) deficiency associated with the MAP3K14 gene leading to the suspicion of a familial ILD. Discussion Genetic testing is essential for diagnosis of ILD, especially in young patients with a family history. Antifibrotics are the only available option for such cases; if immunosuppressive therapy should be initiated still remains a question. Is a lung transplant a realistic solution in such cases? Conclusions Familial aggregation and genetic changes should be sought for in diffuse ILD diagnosis.

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Section: Discussionmentioning

confidence: 99%

Interstitial lung disease with genetic susceptibility: case report

Ana,

Trușculescu

2022

Pneumologia

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Assessing the Severity of Connective Tissue-Related Interstitial Lung Disease Using Computed Tomography Quantitative Analysis Parameters

Su,

Hou,

Zhu

et al. 2024

J Comput Assist Tomogr

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Objectives The aims of the study are to predict lung function impairment in patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) through computed tomography (CT) quantitative analysis parameters based on CT deep learning model and density threshold method and to assess the severity of the disease in patients with CTD-ILD. Methods We retrospectively collected chest high-resolution CT images and pulmonary function test results from 105 patients with CTD-ILD between January 2021 and December 2023 (patients staged according to the gender-age-physiology [GAP] system), including 46 males and 59 females, with a median age of 64 years. Additionally, we selected 80 healthy controls (HCs) with matched sex and age, who showed no abnormalities in their chest high-resolution CT. Based on our previously developed RDNet analysis model, the proportion of the lung occupied by reticulation, honeycombing, and total interstitial abnormalities in CTD-ILD patients (ILD% = total interstitial abnormal volume/total lung volume) were calculated. Using the Pulmo-3D software with a threshold segmentation method of −260 to −600, the overall interstitial abnormal proportion (AA%) and mean lung density were obtained. The correlations between CT quantitative analysis parameters and pulmonary function indices were evaluated using Spearman or Pearson correlation coefficients. Stepwise multiple linear regression analysis was used to identify the best CT quantitative predictors for different pulmonary function parameters. Independent risk factors for GAP staging were determined using multifactorial logistic regression. The area under the ROC curve (AUC) differentiated between the CTD-ILD groups and HCs, as well as among GAP stages. The Kruskal-Wallis test was used to compare the differences in pulmonary function indices and CT quantitative analysis parameters among CTD-ILD groups. Results Among 105 CTD-ILD patients (58 in GAP I, 36 in GAP II, and 11 in GAP III), results indicated that AA% distinguished between CTD-ILD patients and HCs with the highest AUC value of 0.974 (95% confidence interval: 0.955–0.993). With a threshold set at 9.7%, a sensitivity of 98.7% and a specificity of 89.5% were observed. Both honeycombing and ILD% showed statistically significant correlations with pulmonary function parameters, with honeycombing displaying the highest correlation coefficient with Composite Physiologic Index (CPI, r = 0.612). Multiple linear regression results indicated honeycombing was the best predictor for both the Dlco% and the CPI. Furthermore, multivariable logistic regression analysis identified honeycombing as an independent risk factor for GAP staging. Honeycombing differentiated between GAP I and GAP II + III with the highest AUC value of 0.729 (95% confidence interval: 0.634–0.811). With a threshold set at 8.0%, a sensitivity of 79.3% and a specificity of 57.4% were observed. Significant differences in honeycombing and ILD% were also noted among the disease groups (P < 0.05). Conclusions An AA% of 9.7% was the optimal threshold for differentiating CTD-ILD patients from HCs. Honeycombing can preliminarily predict lung function impairment and was an independent risk factor for GAP staging, offering significant clinical guidance for assessing the severity of the patient's disease.

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Expanding horizons: lung transplantation for non-IPF interstitial lung diseases

Citak,

Saribas,

Halis

et al. 2024

BMC Pulm Med

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Objective Interstitial lung diseases (ILDs) are diverse pulmonary disorders marked by diffuse lung inflammation and fibrosis. The variability in characteristics and treatment approaches complicates diagnosis and management. In advanced cases requiring transplantation, determining indications and selecting suitable candidates presents additional challenges. Methods Of all patients with non-IPF ILD between December 2016 to December 2022 were analyzed retrospectively. Patients were categorized into two groups: transplanted patients and deceased patients on the waiting list. Clinical data and survival outcomes were compared between groups. Results Of the 43 patients, 20 underwent lung transplantation while 23 died awaiting transplantation. Waiting list mortality was 53.4%, with median waiting times similar between groups (3 months for transplant patients and 6 months for those on the waiting list). There were no significant differences between groups in age, gender, height, BMI, 6-minute walk test (6MWT), or forced vital capacity (FVC). The prevalence of pulmonary hypertension (PH) was 76.7% in right heart catheterizations, similar in both groups. One single and 19 bilateral lung transplants were performed. Overall, 13 of the 20 patients survived to discharge from the hospital. One-year mortality was 7/20 (35%). The median follow-up was 34 months, with a 1-year conditional survival of 90.9% at 3 years and 70.7% at 5 years. Conclusions This study underscores the importance of further research into non-IPF ILDs. Lung transplantation remains a viable option that can significantly enhance both the quality and longevity of life for patients with advanced ILD.

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Effectiveness of Lung Transplantation in Patients With Interstitial Lung Diseases

Cited by 3 publications

References 14 publications

Interstitial lung disease with genetic susceptibility: case report

Interstitial lung disease with genetic susceptibility: case report

Assessing the Severity of Connective Tissue-Related Interstitial Lung Disease Using Computed Tomography Quantitative Analysis Parameters

Expanding horizons: lung transplantation for non-IPF interstitial lung diseases

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