scholarly journals Progressive fibrosing interstitial lung disease: prevalence and clinical outcome

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Byoung Soo Kwon ◽  
Jooae Choe ◽  
Eun Jin Chae ◽  
Hee Sang Hwang ◽  
Yong-Gil Kim ◽  
...  

Abstract Background The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in a real-world cohort and assess the prognostic significance of the PF-ILD diagnostic criteria. Methods Clinical data of patients with fibrosing ILD other than idiopathic pulmonary fibrosis (IPF) consecutively diagnosed at a single centre were retrospectively reviewed. A PF phenotype was defined based on the criteria used in the INBUILD trial. Results The median follow-up duration was 62.7 months. Of the total of 396 patients, the mean age was 58.1 years, 39.9% were men, and rheumatoid arthritis-ILD was the most common (42.4%). A PF phenotype was identified in 135 patients (34.1%). The PF-ILD group showed lower forced vital capacity and total lung capacity (TLC) than the non-PF-ILD group. The PF-ILD group also showed poorer survival (median survival, 91.2 months vs. not reached; P < 0.001) than the non-PF-ILD group. In multivariable Cox analysis adjusted for age, DLCO, HRCT pattern, and specific diagnosis, PF phenotype was independent prognostic factor (hazard ratio, 3.053; P < 0.001) in patients with fibrosing ILD. Each criterion of PF-ILD showed similar survival outcomes. Conclusions Our results showed that approximately 34% of patients with non-IPF fibrosing ILD showed a progressive phenotype and a poor outcome similar to that of IPF, regardless of the diagnostic criteria used.

2019 ◽  
pp. 1-3
Author(s):  
Julyan Al Fori

OBJECTIVE: To report the prevalence of interstitial lung disease in Systemic Sclerosis patients at the Royal Hospital and compare our data with the literature. METHODS:All adult Omani patients with Systemic Sclerosis (SSc) who are under regular follow-up at the Royal Hospital were retrospectively enrolled from January 2006 to January 2014. RESULTS: A total of 49 cases of Systemic Sclerosis (SSc) patients were included. The mean age was 44.06 ± 11.9 years. There was a predominance of females (48 cases; 98%). Interstitial lung disease present in 30 cases (61%). The most frequent symptoms were dyspnea 47%, cough 33%, and others (e.g. atypical chest pain) 20%. There was no association with smoking, non-smoker (47 cases, 95.5%) versus ex. smoker (2 cases, 4%). The most high resolution computed tomography (HRCT) finding was traction bronchiectasis (21 cases, 42.9%) followed by honey comb appearance (19 cases, 38.8%). Pulmonary Function Test (PFT) was done in 33 cases. The mean total lung capacity (TLC) was 81. 06 ± 26.2. The mean diffusion lung capacity (DLCO) was 61.8± 28.3. Pulmonary hypertension was found in 10 cases only (20.4%) based on echocardiography. CONCLUSIONS: ILD was present in 30 cases out of 49 cases of SSc (61.2%) with female predominance. The most frequently observed HRCT change was traction bronchiectasis. Similarities and differences were found with respect to the previous reports from other countries


PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0244114
Author(s):  
Ui Won Ko ◽  
Eun Jung Cho ◽  
Heung-Bum Oh ◽  
Hyun Jung Koo ◽  
Kyung-Hyun Do ◽  
...  

Disease progression (DP) in interstitial lung disease (ILD) is variable and difficult to predict. In previous reports, serum Krebs von den Lungen-6 (KL-6) was suggested to be useful in diagnosing and predicting survival in ILD. The aim of our study was to investigate the usefulness of serum KL-6 as a predictor of DP in ILD. Clinical data of 199 patients with ILD (idiopathic pulmonary fibrosis: 22.8%) were prospectively collected and serum KL-6 levels were measured. DP was defined as a relative decline in forced vital capacity (FVC) ≥ 10%, acute exacerbation, or death during follow-up. The median follow-up period was 11.1 months. The mean age of the subjects was 62.2 years, and 59.8% were male. DP occurred in 21.6% of patients. The progressed group showed lower FVC, lower diffusing capacity for carbon monoxide, lower the minimum oxygen saturation during the 6-minute walk test, higher fibrosis scores on high-resolution computed tomography, and higher KL-6 levels (826.3 vs. 629.0 U/mL; p < 0.001) than those of the non-progressed group. In receiver operating characteristic curve analysis, serum KL-6 levels were a significant predictor of DP in ILD (area under the curve = 0.629, p = 0.009, and the optimal cut-off level was 811 U/mL). In multivariable Cox analysis, high serum KL-6 levels (≥ 800 U/mL) were only independently associated with DP in ILD (HR 2.689, 95% CI 1.445–5.004, P = 0.002). Serum KL-6 levels might be useful to predict DP in patients with ILD.


2021 ◽  
Vol 10 (12) ◽  
pp. 2548
Author(s):  
Marco Sebastiani ◽  
Fabrizio Luppi ◽  
Gianluca Sambataro ◽  
Diego Castillo Villegas ◽  
Stefania Cerri ◽  
...  

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.


Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3906-3916 ◽  
Author(s):  
Carlos Fernández-Díaz ◽  
Santos Castañeda ◽  
Rafael B Melero-González ◽  
Francisco Ortiz-Sanjuán ◽  
Antonio Juan-Mas ◽  
...  

Abstract Objective To assess the efficacy of abatacept (ABA) in RA patients with interstitial lung disease (ILD) (RA-ILD). Methods This was an observational, multicentre study of RA-ILD patients treated with at least one dose of ABA. ILD was diagnosed by high-resolution CT (HRCT). We analysed the following variables at baseline (ABA initiation), 12 months and at the end of the follow-up: Modified Medical Research Council (MMRC) scale (1-point change), forced vital capacity (FVC) or diffusion lung capacity for carbon monoxide (DLCO) (improvement or worsening ≥10%), HRCT, DAS on 28 joints evaluated using the ESR (DAS28ESR) and CS-sparing effect. Results We studied 263 RA-ILD patients [150 women/113 men; mean (s.d.) age 64.6 (10) years]. At baseline, they had a median duration of ILD of 1 (interquartile range 0.25–3.44) years, moderate or severe degree of dyspnoea (MMRC grade 2, 3 or 4) (40.3%), FVC (% of the predicted) mean (s.d.) 85.9 (21.8)%, DLCO (% of the predicted) 65.7 (18.3) and DAS28ESR 4.5 (1.5). The ILD patterns were: usual interstitial pneumonia (UIP) (40.3%), non-specific interstitial pneumonia (NSIP) (31.9%) and others (27.8%). ABA was prescribed at standard dose, i.v. (25.5%) or s.c. (74.5%). After a median follow-up of 12 (6–36) months the following variables did not show worsening: dyspnoea (MMRC) (91.9%); FVC (87.7%); DLCO (90.6%); and chest HRCT (76.6%). A significant improvement of DAS28ESR from 4.5 (1.5) to 3.1 (1.3) at the end of follow-up (P &lt; 0.001) and a CS-sparing effect from a median 7.5 (5–10) to 5 (2.5–7.5) mg/day at the end of follow-up (P &lt; 0.001) was also observed. ABA was withdrawn in 62 (23.6%) patients due to adverse events (n = 30), articular inefficacy (n = 27), ILD worsening (n = 3) and other causes (n = 2). Conclusion ABA may be an effective and safe treatment for patients with RA-ILD.


1994 ◽  
Vol 76 (1) ◽  
pp. 271-277 ◽  
Author(s):  
P. G. Hartley ◽  
J. R. Galvin ◽  
G. W. Hunninghake ◽  
J. A. Merchant ◽  
S. J. Yagla ◽  
...  

To assess the validity of computer-assisted methods in analyzing the lung parenchyma imaged with high-resolution computed tomography (HRCT), we compared computer-derived estimates of lung density to other, more traditional, measures of parenchymal injury in 24 subjects with idiopathic pulmonary fibrosis (IPF) and 60 subjects with extensive occupational exposure to asbestos. Gray scale density histograms were constructed from the HRCT images. The gray scale histogram of both study groups was of a skewed unimodal distribution. However, compared with the asbestos-exposed subjects, the patients with IPF had a gray scale distribution that was significantly shifted to the right (greater density) and flatter. In a multivariate analysis, after controlling for age and cigarette smoking, we found that the mean and median gray scale densities were independently associated with the presence of moderate-to-severe dyspnea, a higher International Labour Office chest X-ray category, a lower forced vital capacity, and a higher concentration of macrophages and eosinophils in the bronchoalveolar lavage fluid. These factors accounted for > 70% of the variance of the mean and median gray scale densities. Interestingly, no differences in gray scale density measures were noted between patients with IPF and patients with asbestosis when these other factors were taken into account. Our results suggest that computer-derived density analysis of the lung parenchyma on the HRCT scan is a valid, clinically meaningful, and objective measure of interstitial lung disease.


Author(s):  
Tamara Vojinovic ◽  
Ilaria Cavazzana ◽  
Paolo Ceruti ◽  
Micaela Fredi ◽  
Denise Modina ◽  
...  

Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511


2021 ◽  
Author(s):  
Bingpeng Guo ◽  
Qian Han ◽  
Wenjie Wang ◽  
Ziyi Zhang ◽  
Baoqing Sun ◽  
...  

Abstract Background: Krebs von den Lungen-6 (KL-6) is considered a sensitive biomarker for diagnosis of interstitial lung disease (ILD). We aimed to evaluate the diagnosis value of Nanopia® KL-6 (SEKISUI MEDICAL CO., LTD., Tokyo, Japan) in a Chinese cohort of patients with ILD.Methods: Totally 451 patients were enrolled in our multicenter study, including 166 (36.8%) ILD patients, 210 (46.6%) non-ILD patients and 75 (16.6%) health controls. All ILD patients underwent high-resolution computed tomography (HRCT) followed by pulmonary function test (PFT). Serum KL-6 concentrations were measured by latex particle enhanced turbidimetric immunoassay (LTIA).Results: KL-6 serum concentrations were significantly higher in ILD patients (911 U/ml, IQR 477-1790) than in non-ILD patients (225 U/ml, IQR 166-323) and health controls (196 U/ml, IQR 153-230, p<0.0001). Serum KL-6 higher than 435.5 U/ml appeared as the optimal cut-off value associated with ILD. KL-6 concentrations were inversely correlated with forced vital capacity (FVC) (rho=-0.515, p<0.001), total lung capacity (TLC) (rho=-0.563, p<0.001) and diffuse lung capacity of carbon monoxide (DLco) (rho=-0.544, p<0.001). ILD patients with more severe characteristics of HRCT including ground glass opacity, reticular pattern or honeycombing had significantly higher serum KL-6 levels. In the subgroup of ILD patients, serum KL-6 concentrations were higher in idiopathic interstitial pneumonia (IIP) patients (1024U/ml, IQR 697-2112.25) than in other ILD patients (743U/ml, IQR 702.75-2058.25, p<10-4). ILD and retained smoking were independent factors associated with higher KL-6 levels in multivariate analysis. Conclusions: Our study confirms that KL-6 is a credible biomarker for the diagnosis of ILD in a Chinese cohort of patients. High serum KL-6 concentration should call attention to physicians to assess ILD with HRCT and PFT.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 969.1-970
Author(s):  
M. DI Carlo ◽  
M. Tardella ◽  
E. Filippucci ◽  
F. Salaffi

Background:In recent years, a growing interest has grown around interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). While high resolution computed tomography (HRCT) of the chest remains the diagnostic method of choice, increasing attention has been directed towards lung ultrasound (LUS) in the diagnosis of ILD in connective tissue diseases. LUS allows the detection of artifacts (B-lines) characteristic of ILD, without the need to use ionizing radiation. However, it is not yet well defined how to interpret the LUS findings under suspicion of RA-ILD.Objectives:To determine the cut-off number of LUS B-lines that identifies a significant RA-ILD.Methods:A cross sectional study was conducted on consecutive RA patients with suspected RA-ILD. The inclusion criteria were clinical (dyspnea, velcro sounds), instrumental (suggestive anomalies on conventional radiography, DLco reduction), or in presence of at least two of the following risk factors for RA-ILD: smoking habit, male sex, advanced age, and ACPA presence.Patients underwent LUS, chest HRCT, pulmonary function tests, and clinical evaluation. The diagnosis of RA-ILD was based on a semi-quantitative evaluation of HRCT using a computer-aided method (CaM). The 10% of fibrosis, measured with this method, was considered as a cut-off for the presence of significant RA-ILD. The LUS was carried out in 14 defined intercostal spaces using a linear multifrequency probe 6-18 MHz (MyLab Class C, Esaote S.p.A., Genoa, Italy) and the number of B-lines present in each intercostal space was counted and summed up (Figure 1). The discriminating validity of the LUS versus HRCT has been studied by using the receiver operating characteristic (ROC) curve analysis.Figure 1.LUS B-line illustrative.Results:72 consecutive RA patients (21 male, 51 female) were evaluated, with a mean age of 63.0 (SD 11.5 years), a mean ACPA titre of 327.6 (SD 633.3) U/ml and a rheumatoid factor of 324.6 (SD 748.7) U/ml. The mean estimate of pulmonary fibrosis using the CaM was 11.2% (SD 7.5) at HRCT, while at LUS the mean number of B-lines was 10.6 (SD 15.1). Pulmonary fibrosis of 10% as measured by the CaM at HRCT was detected in 25 patients (34.72%). Applying this HRCT cut-off point as an estimate of significant fibrosis, the presence of 9 B-lines was found to be the optimal cut-off at ROC curve analysis. This LUS cut-off defines the presence of significant fibrosis with a sensitivity of 70.0%, a specificity of 97.62%, and a positive likelihood ratio of 29.4 (Figure 2).Figure 2.Area under the ROC curve to determine the number of B-lines at LUS to define a significant RA-ILD, applying the 10% of fibrosis at chest HRCT measured by OsiriX as external criterion.Conclusion:The present study provided data to determine the number of B-lines to identify a significant RA-ILD. LUS may represent a useful technique to select RA patients to be assessed by chest HRCT.References:[1] Tardella M, et al. Ultrasound B-lines in the evaluation of interstitial lung disease in patients with systemic sclerosis: Cut-off point definition for the presence of significant pulmonary fibrosis. Medicine (Baltimore). 2018;97(18):e0566.[2] Salaffi F, et al. High-resolution computed tomography of the lung in patients with rheumatoid arthritis: Prevalence of interstitial lung disease involvement and determinants of abnormalities. Medicine (Baltimore). 2019;98(38):e17088.Disclosure of Interests:Marco Di Carlo: None declared, Marika Tardella: None declared, Emilio Filippucci Speakers bureau: Dr. Filippucci reports personal fees from AbbVie, personal fees from Bristol-Myers Squibb, personal fees from Celgene, personal fees from Roche, personal fees from Union Chimique Belge Pharma, personal fees from Pfizer, outside the submitted work., Fausto Salaffi: None declared


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yun Jae Kim ◽  
Jooae Choe ◽  
Ho Jeong Kim ◽  
Jin Woo Song

AbstractInterstitial lung disease (ILD) is the most common lung manifestation in patients with Sjögren syndrome (SJS) and is associated with poor outcomes. This study aimed to investigate the long-term clinical course and prognostic factors in patients with SJS-ILD. Clinical data and high-resolution computed tomography (HRCT) images of 62 patients with primary SJS-ILD were retrospectively analyzed (biopsy-proven cases, n = 16). The mean patient age was 59.8 years; 83.9% of the patients were females, and 38.7% showed a usual interstitial pneumonia (UIP) pattern on HRCT. The median follow-up period was 61.5 months. During follow-up, 15 patients (24.2%) died, 7 (11.3%) experienced acute exacerbation (AE), and 27 (43.5%) progressed. The 1-, 3- and 5-year survival rates were 93.5%, 85.8%, and 81.1%, respectively. Age (hazard ratio [HR]: 1.158, P = 0.003), C-reactive protein (CRP) level (HR: 1.212, P = 0.045), FVC (HR: 0.902, P = 0.005), and a UIP pattern on HRCT (HR: 4.580, P = 0.029) were significant prognostic factors in multivariable Cox analysis. In conclusion, death, AE, and ILD progression occurred in 25%, 10%, and 50% of the patients with SJS-ILD, respectively. Older age, higher CRP level, lower FVC, and a UIP pattern on HRCT indicated poor prognosis.


2021 ◽  
pp. 239719832110244
Author(s):  
Ramya Janardana ◽  
Aparna Irodi ◽  
Pramod P Chebbi ◽  
Ruchika Goel ◽  
Leena R Vimala ◽  
...  

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients. Results: Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud’s symptoms of 36 months (13.5–60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0–1.3) and 1 (0–1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5–49), the percentage of forced vital capacity improved by 1.8% (–3.82 to 9.07) as compared with baseline visit ( p = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively. Conclusion: Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period.


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