Interstitial lung diseases

Wijsenbeek, Marlies; Suzuki, Atsushi; Maher, Toby

doi:10.1016/s0140-6736(22)01052-2

Cited by 122 publications

(64 citation statements)

References 164 publications

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“…El patrón de presentación de la enfermedad es heterogéneo y está en función de la causa subyacente, por lo tanto, es fundamental establecer el diagnóstico oportuno y con certeza [2]. Lo complejo de la evaluación radica en la existencia de más de 200 causas que contribuyen con la EPI, lo cual, contribuye con un retraso significativo en el diagnóstico temprano, y en este tiempo, los casos reciben intervenciones ineficaces por tiempos prolongados y el retraso en el diagnóstico o referencia a un centro especializado en la atención de EPI se ha relacionado de manera consistente con una menor tasa de supervivencia [3, 4].…”

Section: Transferencia En La Práctica De Dr Arturo Cortés-télles Y Dr...unclassified

Estructurar centros especializados en el diagnóstico y tratamiento de enfermedades pulmonares intersticales – una necesidad no resuelta

Cortés-Télles¹,

Ortíz‐Farias²

2022

Kompass Neumol

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Background: Early appropriate diagnosis and treatment of interstitial lung diseases (ILD) is crucial to slow disease progression and improve survival. Yet it is unknown whether initial management in an expert centre is associated with improved outcomes. Therefore, we assessed mortality, hospitalisations and health care costs of ILD patients initially diagnosed and managed in specialised ILD centres versus non-specialised centres and explored differences in pharmaceutical treatment patterns. Methods: An epidemiological claims data analysis was performed, including patients with different ILD subtypes in Germany between 2013 and 2018. Classification of specialised centres was based on the number of ILD patients managed and procedures performed, as defined by the European Network on Rare Lung Diseases. Inverse probability of treatment weighting was used to adjust for covariates. Mortality and hospitalisations were examined via weighted Cox models, cost differences by weighted gamma regression models and differences in treatment patterns with weighted logistic regressions. Results: We compared 2022 patients managed in seven specialised ILD centres with 28,771 patients managed in 1156 non-specialised centres. Specialised ILD centre management was associated with lower mortality (HR: 0.87, 95% CI 0.78; 0.96), lower all-cause hospitalisation (HR: 0.93, 95% CI 0.87; 0.98) and higher respiratory-related costs (€669, 95% CI €219; €1156). Although risk of respiratory-related hospitalisations (HR: 1.00, 95% CI 0.92; 1.10) and overall costs (€ -872, 95% CI € -75; €1817) did not differ significantly, differences in treatment patterns were observed. Conclusion: Initial management in specialised ILD centres is associated with improved mortality and lower all-cause hospitalisations, potentially due to more differentiated diagnostic approaches linked with more appropriate ILD subtype-adjusted therapy.

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Section: Transferencia En La Práctica De Dr Arturo Cortés-télles Y Dr...unclassified

Estructurar centros especializados en el diagnóstico y tratamiento de enfermedades pulmonares intersticales – una necesidad no resuelta

Cortés-Télles¹,

Ortíz‐Farias²

2022

Kompass Neumol

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“…Interstitial lung diseases (ILDs) represent a group of more than 200 diverse parenchymal pulmonary disorders that share similar clinical, radiographic, physiologic or pathologic manifestations. ILDs are mainly characterized by alveolar and interstitial inflammation and/or fibrosis, usually leading to decreased lung function that may eventually be fatal [ 1 , 2 ]. Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive and fatal disease of unknown etiology and it is considered one of the most representative types of lung fibrosis within the ILDs [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…The radiologic pattern of UIP consists of bilateral reticulation and honeycombing specifically in the periphery and in the lower lobes, with a correspondent histologic UIP pattern consisting of heterogeneous paraseptal fibrosis with architectural distortion associated with the typical presence of fibroblast foci [ 2 ]. IPF usually occurs in older adults (68 years is the mean age of diagnosis) and its prevalence, incidence and mortality is higher not only in the aged population but also among males [ 1 , 3 , 5 , 6 , 7 ]. Furthermore, with an incidence of 0.09–1.30 per 10,000 people, an estimated prevalence of 0.33–4.51 per 10,000 people and a median survival of 3–5 years following diagnosis, IPF is regarded as a rare disease that is more lethal than most cancers [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Promises and Challenges of Cell-Based Therapies to Promote Lung Regeneration in Idiopathic Pulmonary Fibrosis

Egea-Zorrilla

Vera

Sáez

et al. 2022

Cells

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The lung epithelium is constantly exposed to harmful agents present in the air that we breathe making it highly susceptible to damage. However, in instances of injury to the lung, it exhibits a remarkable capacity to regenerate injured tissue thanks to the presence of distinct stem and progenitor cell populations along the airway and alveolar epithelium. Mechanisms of repair are affected in chronic lung diseases such as idiopathic pulmonary fibrosis (IPF), a progressive life-threatening disorder characterized by the loss of alveolar structures, wherein excessive deposition of extracellular matrix components cause the distortion of tissue architecture that limits lung function and impairs tissue repair. Here, we review the most recent findings of a study of epithelial cells with progenitor behavior that contribute to tissue repair as well as the mechanisms involved in mouse and human lung regeneration. In addition, we describe therapeutic strategies to promote or induce lung regeneration and the cell-based strategies tested in clinical trials for the treatment of IPF. Finally, we discuss the challenges, concerns and limitations of applying these therapies of cell transplantation in IPF patients. Further research is still required to develop successful strategies focused on cell-based therapies to promote lung regeneration to restore lung architecture and function.

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“…After the advent of the anti-fibrotic drugs Pirfenidone and Nintedanib had fundamentally changed the therapeutic landscape in IPF (6,7), increasing evidence also suggested their use in systemic sclerosis (SSC)-ILD or progressive fibrosing ILD other than IPF (8)(9)(10)(11)(12). With regards to these advances, recent studies and guidelines support a treatment strategy based on disease phenotype, irrespective of the underlying ILD diagnosis (13). Patients with "inflammatory" ILD considered likely to respond to anti-inflammatory therapies should receive such treatment, however if progressive fibrosis occurs, anti-fibrotic agents should be used either as monotherapy or as an add-on (8,(13)(14)(15)(16)(17).…”

Section: Introductionmentioning

confidence: 99%

Clinical, imaging, and blood biomarkers to assess 1-year progression risk in fibrotic interstitial lung diseases—Development and validation of the honeycombing, traction bronchiectasis, and monocyte (HTM)-score

et al. 2022

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IntroductionProgression of fibrotic interstitial lung disease (ILD) leads to irreversible loss of lung function and increased mortality. Based on an institutional ILD registry, we aimed to evaluate biomarkers derived from baseline patient characteristics, computed tomography (CT), and peripheral blood for prognosis of disease progression in fibrotic ILD patients.MethodsOf 209 subsequent ILD-board patients enregistered, 142 had complete follow-up information and were classified fibrotic ILD as defined by presence of reticulation or honeycombing using a standardized semi-quantitative CT evaluation, adding up typical ILD findings in 0–6 defined lung fields. Progression at 1 year was defined as relative loss of ≥10% in forced vital capacity, of ≥15% in diffusion capacity for carbon monoxide, death, or lung transplant. Two-thirds of the patients were randomly assigned to a derivation cohort evaluated for the impact of age, sex, baseline lung function, CT finding scores, and blood biomarkers on disease progression. Significant variables were included into a regression model, its results were used to derive a progression-risk score which was then applied to the validation cohort.ResultsIn the derivation cohort, age, monocyte count ≥0.65 G/L, honeycombing and traction bronchiectasis extent had significant impact. Multivariate analyses revealed the variables monocyte count ≥0.65 G/L (1 point) and combined honeycombing or traction bronchiectasis score [0 vs. 1–4 (1 point) vs. 5–6 lung fields (2 points)] as significant, so these were used for score development. In the derivation cohort, resulting scores of 0, 1, 2, and 3 accounted for 1-year progression rates of 20, 25, 46.9, and 88.9%, respectively. Similarly, in the validation cohort, progression at 1 year occurred in 0, 23.8, 53.9, and 62.5%, respectively. A score ≥2 showed 70.6% sensitivity and 67.9% specificity, receiver operating characteristic analysis for the scoring model had an area under the curve of 71.7%.ConclusionThe extent of honeycombing and traction bronchiectasis, as well as elevated blood monocyte count predicted progression within 1 year in fibrotic ILD patients.

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Interstitial lung diseases

Cited by 122 publications

References 164 publications

Estructurar centros especializados en el diagnóstico y tratamiento de enfermedades pulmonares intersticales – una necesidad no resuelta

Estructurar centros especializados en el diagnóstico y tratamiento de enfermedades pulmonares intersticales – una necesidad no resuelta

Promises and Challenges of Cell-Based Therapies to Promote Lung Regeneration in Idiopathic Pulmonary Fibrosis

Clinical, imaging, and blood biomarkers to assess 1-year progression risk in fibrotic interstitial lung diseases—Development and validation of the honeycombing, traction bronchiectasis, and monocyte (HTM)-score

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