Validation of Proposed Criteria for Progressive Pulmonary Fibrosis

Pugashetti, Janelle Vu; Adegunsoye, Ayodeji; Wu, Zhe; Lee, Cathryn T.; Srikrishnan, Anand; Ghodrati, Sahand; Vo, Vivian; Renzoni, Elisabetta; Wells, Adrian; Garcia, Christine Kim; Chua, Felix; Newton, Chad A.; Molyneaux, Philip L.; Oldham, Justin M.

doi:10.1164/rccm.202201-0124oc

Cited by 68 publications

(36 citation statements)

References 36 publications

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“…Thus, our applied thresholds for progression necessarily needed to be higher to differ between clinically significant deterioration and physiological variation with a reasonable sensitivity and specificity. In line with that, Pugashetti et al recently showed that a previous decline in FVC of ≥10% was the best biomarker for 5-year transplant-free survival in non-IPF ILD patients ( 50 ).…”

Section: Discussionmentioning

confidence: 57%

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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Section: Discussionmentioning

confidence: 57%

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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“…Fibrotic interstitial lung diseases (f-ILDs) are a heterogeneous group of diffuse parenchymal lung disorders that may share common overlapping clinical characteristics and, under not yet clearly identified circumstances, progression to pulmonary fibrosis [ 1 , 2 , 3 , 4 ]. Variable susceptibility and progression to diffuse lung fibrosis constitute the major determinant of their unpredictable clinical course and prognosis [ 5 , 6 , 7 , 8 ]. A major achievement of the last decades in the elucidation of their etiopathogenetic mechanisms, besides the role of environmental, aging-related, immune or other factors, is that genomics play an important, previously unsuspected, role in their development [ 9 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective

et al. 2022

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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.

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“…We have reviewed with great interest the article “Validation of Proposed Criteria for Progressive Pulmonary Fibrosis” by Pugashetti and colleagues ( 1 ). The authors have assessed progressive pulmonary fibrosis (PPF) criteria proposed in different studies and their association with transplant-free survival in patients with nonidiopathic pulmonary fibrosis (non-IPF).…”

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confidence: 99%

“…The authors have assessed progressive pulmonary fibrosis (PPF) criteria proposed in different studies and their association with transplant-free survival in patients with nonidiopathic pulmonary fibrosis (non-IPF). After different analyses, the authors conclude that an FVC decline of ⩾10% and six additional PPF criteria in the absence of such decline identify patients with non-IPF interstitial lung disease (ILD) at increased risk of death or lung transplant ( 1 ).…”

mentioning

confidence: 99%