Figure - available from: Chronic Respiratory Disease
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HRCT score models and network analyses for FEV1 (a) and FEV1/FVC (b). Top lines show SBC-defined HRCT score groups with predicted PFT values; PAD, MOS and VOL are given as present (1) or absent (0), p values are for differences between the respective HRCT score groups. Network analyses depict figures for Spearman correlation coefficients, with red lines for positive, blue lines for negative correlations. Correlation coefficients ≤±0.1 are represented as thin gray lines without figures. FEV1: forced expiratory volume in 1 second; FEV1/FVC: FEV1/forced vital capacity; HRCT: high-resolution computed tomography; UVA: univariate analysis; MVA: multivariate analysis; BRK: traction bronchiectasis; RET: reticulation; HON: honeycombing; EMP: emphysema; GGO: ground glass opacities; NDL: noduli; CON: consolidations; PAD: pulmonary artery: aortic diameter </≥1; MOS: mosaic attenuation; VOL: volume reduction; SBC: Schwarz Bayesian Information Criterion; PFT: pulmonary function test.
Source publication
The aim was to evaluate the impact of multiple high-resolution computed tomography (HRCT) features on pulmonary function test (PFT) biomarkers in fibrotic interstitial lung disease (FILD) patients. HRCT of subsequently ILD-board-discussed FILD patients were semi-quantitatively evaluated in a standardized approach: 18 distinct lung regions were scor...
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Background
Early diffuse cutaneous systemic sclerosis (dcSSc) has the highest case fatality among rheumatic diseases. We report baseline characteristics, current immunosuppressive therapies, progression of skin and internal organ involvement, and mortality in a multicenter prospective cohort from the United States (US) of America.
Methods
We perfo...
Citations
... [13,14] In addition, presence and extent of fibrosis on HRCT have been identified as highly significant risk factors for disease progression in various ILD subtypes. [10,15] However, not all patients presenting with fibrotic ILD will experience progression, but a significant proportion may even demonstrate functional improvement over time, reflecting the heterogeneous nature of the disease, like shown in SSC-ILD. ...
... This approach resulted in a simple scoring system from one to six, as described in our previous publications. [15,19] Blood samples were analyzed using a Sysmex ® XN-3000 hematology analyzer (Sysmex Europe GmbH, Norderstedt, Germany) for cell counts and a Cobas ® 8,000 modular analyzer (Roche Diagnostics International AG, Rotkreuz, Switzerland) for C-reactive protein (CRP), lactate dehydrogenase (LDH), and rheumatoid factor measurement. Autoimmune serology testing was 3 performed using a EuroPatternMicroscope ® , a Dynex ® , and a EuroBlotOne ® platform by Euroimmun (EUROIMMUN Medizinische Labordiagnostik AG, Lübeck, Germany) for anti-nuclear (ANA), antineutrophil cytoplasmatic (ANCA) and other disease-specific antibodies. ...
Background: The clinical spectrum of fibrotic interstitial lung diseases (ILD) is highly heterogeneous. We aimed to evaluate the prognostic value of widely available baseline biomarkers for improvement of lung function in patients with fibrotic ILD. Methods: This registry-based study included 142 patients with fibrotic ILD as defined by presence of reticulation, traction bronchiectasis or honeycombing on initial high-resolution computed tomography (HRCT). Functional improvement at 1 year was defined as relative increase of 5% in forced vital capacity (FVC) or of 10% in diffusion capacity for carbon monoxide (DLCO). The prognostic value of baseline biomarkers was evaluated for all patients and the subgroup with anti-inflammatory treatment. Results: At one year, 44 patients showed improvement while 73 progressed. Multivariate analyses found prognostic significance for age <60 years (OR 5.4; 95%CI 1.9-15.4; p=0.002), lactate dehydrogenase (LDH) >250U/L (OR 2.5; 95%CI 1.1-5.8; p=0.043) and blood monocyte count <0.8G/L (OR 3.5; 95%CI 1.1-11.3; p=0.034). In 84 patients undergoing anti-inflammatory treatment, multivariate analysis revealed age <60 years (OR 8.5 (95%CI 2.1-33.4; p=0.002) as the only significant variable. Conclusion: Younger age, higher LDH and lower blood monocyte count predicted functional improvement in fibrotic ILD patients, while in those treated with anti-inflammatory drugs, only age had significant implications.
... To address this unmet need, high-resolution computed tomography (HRCT) may be a helpful tool as it plays a crucial role in diagnosing and monitoring IPF [1,16]. Furthermore, the extent of lung abnormalities on HRCT has been shown to correlate with lung function parameters and survival in these patients [17,18]. Preliminary studies revealed that certain CT findings, such as fibrosis and ground-glass opacities (GGOs), tend to increase more significantly in patients with unsatisfactory response [19,20]; in turn, these findings highlight the prognostic value of CT in determining their treatment response. ...
Antifibrotic therapy has changed the treatment paradigm for idiopathic pulmonary fibrosis (IPF); however, a subset of patients still experienced rapid disease progression despite treatment. This study aimed to determine whether CT-based radiomic features can predict therapeutic response to antifibrotic agents. In this retrospective study, 35 patients with IPF on antifibrotic treatment enrolled from two centers were divided into training (n = 26) and external validation (n = 9) sets. Clinical and pulmonary function data were collected. The patients were categorized into stable disease (SD) and progressive disease (PD) groups based on functional or radiologic criteria. From pretreatment non-enhanced high-resolution CT (HRCT) images, twenty-six radiomic features were extracted through whole-lung texture analysis, and six parenchymal patterns were quantified using dedicated imaging platforms. The predictive factors for PD were determined via univariate and multivariate logistic regression analyses. In the training set (SD/PD: 12/14), univariate analysis identified eight radiomic features and ground-glass opacity percentage (GGO%) as potential predicators of PD. However, multivariate analysis found that the single independent predictor was the sum entropy (accuracy, 80.77%; AUC, 0.75). The combined sum entropy-GGO% model improved the predictive performance in the training set (accuracy, 88.46%; AUC, 0.77). The overall accuracy of the combined model in the validation set (SD/PD: 7/2) was 66.67%. Our preliminary results demonstrated that radiomic features based on pretreatment HRCT could predict the response of patients with IPF to antifibrotic treatment.
Introduction
Progression 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.
Methods
Of 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.
Results
In 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%.
Conclusion
The extent of honeycombing and traction bronchiectasis, as well as elevated blood monocyte count predicted progression within 1 year in fibrotic ILD patients.
Purpose of review:
This review aims to describe how the clinical manifestations of sarcoidosis may be shaped by the effects of sex hormones and by age dependent changes in immune functions and physiology This review is intended to highlight the need to consider the effects of sex and sex in future studies of sarcoidosis.
Recent findings:
The clinical manifestations of sarcoidosis differ based on sex and gender There is emerging evidence that female and male hormones and X-linked genes are important determinants of immune responses to environmental antigens, which has important implications for granuloma formation in the context of sarcoidosis Furthermore, sex hormone levels predictably change throughout adolescence and adulthood, and this occurs in parallel with the onset immune senescence and changes in physiology with advanced age.
Summary:
Recent studies indicate that sex and age are important variables shaping the immune response of humans to environmental antigens We posit herein that sex and age are important determinants of sarcoidosis clinical phenotypes Many gaps in our understanding of the roles played by sex and gender in sarcoidosis, and these need to be considered in future studies.
