565 results found
Gupta R, Baughman RP, Nathan SD, et al., 2022, The six-minute walk test in sarcoidosis associated pulmonary hypertension: Results from an international registry, Virtual International Conference of the American-Thoracic-Society, Publisher: W B SAUNDERS CO LTD, ISSN: 0954-6111
Khanna D, Tashkin DP, Wells AU, et al., 2021, STRATUS: A Phase II Study of Abituzumab in Patients With Systemic Sclerosis-associated Interstitial Lung Disease, JOURNAL OF RHEUMATOLOGY, Vol: 48, Pages: 1295-1298, ISSN: 0315-162X
Stock CJW, Hoyles RK, Daccord C, et al., 2021, Serum markers of pulmonary epithelial damage in systemic sclerosis-associated interstitial lung disease and disease progression, Respirology, Vol: 26, Pages: 461-468, ISSN: 1323-7799
Background and objectiveThe course of systemic sclerosis‐associated interstitial lung disease (SSc‐ILD) is highly variable, and accurate prognostic markers are needed. KL‐6 is a mucin‐like glycoprotein (MUC1) expressed by type II pneumocytes, while CYFRA 21‐1 is expressed by alveolar and bronchiolar epithelial cells. Both are released into the blood from cell injury.MethodsSerum KL‐6 and CYFRA 21‐1 levels were measured in a retrospective (n = 189) and a prospective (n = 118) cohort of SSc patients. Genotyping of MUC1 rs4072037 was performed. Linear mixed‐effect models were used to evaluate the relationship with change in lung function parameters over time, while association with survival was evaluated with Cox proportional hazard analysis.ResultsIn both cohorts, KL‐6 and CYFRA 21‐1 were highest in patients with lung involvement, and in patients with extensive rather than limited ILD. KL‐6 was higher in patients carrying the MUC1 rs4072037 G allele in both cohorts. In patients with SSc‐ILD, serum KL‐6, but not CYFRA 21‐1, was significantly associated with DLCO decline in both cohorts (P = 0.001 and P = 0.004, respectively), and with FVC decline in the retrospective cohort (P = 0.005), but not the prospective cohort. When combining the cohorts and subgrouping by severity (median CPI = 45.97), KL‐6 remained predictive of decline in DLCO in both milder (P = 0.007) and more severe disease (P = 0.02) on multivariable analysis correcting for age, gender, ethnicity, smoking history and MUC1 allele carriage.ConclusionOur results suggest serum KL‐6 predicts decline in lung function in SSc, suggesting its clinical utility in risk stratification for progressive SSc‐ILD.
Wells AU, Devaraj A, Desai SR, 2021, Interstitial Lung Disease after COVID-19 Infection: A Catalog of Uncertainties COMMENT, RADIOLOGY, Vol: 299, Pages: E216-E218, ISSN: 0033-8419
Chaudhuri N, George PM, Kreuter M, et al., 2021, Hospitalization outcomes for COVID-19 in patients with interstitial lung disease: a potential role for aerodigestive pathophysiology? reply, American Journal of Respiratory and Critical Care Medicine, Vol: 203, Pages: 522-524, ISSN: 1073-449X
Invernizzi R, Wu BG, Barnett J, et al., 2021, The respiratory microbiome in chronic hypersensitivity pneumonitis is distinct from that of idiopathic pulmonary fibrosis, American Journal of Respiratory and Critical Care Medicine, Vol: 203, Pages: 339-347, ISSN: 1073-449X
RATIONALE: Chronic hypersensitivity pneumonitis (CHP) is a condition that arises following repeated exposure and sensitisation to inhaled antigens. The lung microbiome is increasingly implicated in respiratory disease but to date, no study has investigated the composition of microbial communities in the lower airways in CHP. OBJECTIVE: To characterise and compare the airway microbiome in subjects with CHP, idiopathic pulmonary fibrosis (IPF) and controls. METHODS: We prospectively recruited individuals diagnosed with CHP (n=110), IPF (n=45) and controls (n=28). Subjects underwent bronchoalveolar lavage and bacterial DNA was isolated, quantified by qPCR and the 16S rRNA gene was sequenced to characterise the bacterial communities in the lower airways. MAIN MEASUREMENTS AND RESULTS: Distinct differences in the microbial profiles were evident in the lower airways of subjects with CHP and IPF. At the phylum level, the prevailing microbiota of both IPF and CHP subjects included Firmicutes, Bacteroidetes, Proteobacteria and Actinobacteria. However, in IPF, Firmicutes dominated while the percentage of reads assigned to Proteobacteria in the same group was significantly lower compared to CHP subjects. At the genus level, Staphylococcus was increased in CHP and Actinomyces and Veillonella in IPF. The lower airway bacterial burden in CHP subjects was higher than controls but lower than those with IPF. In contrast to IPF, there was no association between bacterial burden and survival in CHP. CONCLUSIONS: The microbial profile of the lower airways in subjects with CHP is distinct from that of IPF and, notably, bacterial burden in individuals with CHP fails to predict survival.
Wells AU, 2021, New insights into the treatment of CTD-ILD, NATURE REVIEWS RHEUMATOLOGY, Vol: 17, Pages: 79-80, ISSN: 1759-4790
Behr J, Nathan SD, Wuyts WA, et al., 2021, Efficacy and safety of sildenafil added to pirfenidone in patients with advanced idiopathic pulmonary fibrosis and risk of pulmonary hypertension: a double-blind, randomised, placebo-controlled, phase 2b trial, LANCET RESPIRATORY MEDICINE, Vol: 9, Pages: 85-95, ISSN: 2213-2600
Bajwah S, Colquitt J, Loveman E, et al., 2021, Pharmacological and nonpharmacological interventions to improve symptom control, functional exercise capacity and quality of life in interstitial lung disease: an evidence synthesis, ERJ OPEN RESEARCH, Vol: 7
Tzouvelekis A, Antoniou K, Kreuter M, et al., 2021, The DIAMORFOSIS (DIAgnosis and Management Of lung canceR and FibrOSIS) survey: international survey and call for consensus, ERJ OPEN RESEARCH, Vol: 7
Avasarala SK, Wells AU, Colby TV, et al., 2021, Transbronchial Cryobiopsy in Interstitial Lung Diseases State-of-the-Art Review for the Interventional Pulmonologist, JOURNAL OF BRONCHOLOGY & INTERVENTIONAL PULMONOLOGY, Vol: 28, Pages: 81-92, ISSN: 1944-6586
Hetzel J, Wells AU, Costabel U, et al., 2020, Transbronchial cryobiopsy increases diagnostic confidence in interstitial lung disease: a prospective multicentre trial, EUROPEAN RESPIRATORY JOURNAL, Vol: 56, ISSN: 0903-1936
Brown KK, Schlenker-Herceg R, Wells AU, 2020, Reply to comment on "The natural history of progressive fibrosing interstitial lung diseases", EUROPEAN RESPIRATORY JOURNAL, Vol: 56, ISSN: 0903-1936
Walsh SLF, Humphries SM, Wells AU, et al., 2020, Imaging research in fibrotic lung disease; applying deep learning to unsolved problems, LANCET RESPIRATORY MEDICINE, Vol: 8, Pages: 1144-1153, ISSN: 2213-2600
Drake TM, Docherty AB, Harrison EM, et al., 2020, Outcome of hospitalization for COVID-19 in patients with interstitial lung disease: an international multicenter study., American Journal of Respiratory and Critical Care Medicine, Vol: 202, Pages: 1656-1665, ISSN: 1073-449X
RATIONALE: The impact of COVID-19 on patients with Interstitial Lung Disease (ILD) has not been established. OBJECTIVES: To assess outcomes in patients with ILD hospitalized for COVID-19 versus those without ILD in a contemporaneous age, sex and comorbidity matched population. METHODS: An international multicenter audit of patients with a prior diagnosis of ILD admitted to hospital with COVID-19 between 1 March and 1 May 2020 was undertaken and compared with patients, without ILD obtained from the ISARIC 4C cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished IPF from non-IPF ILD and used lung function to determine the greatest risks of death. MEASUREMENTS AND MAIN RESULTS: Data from 349 patients with ILD across Europe were included, of whom 161 were admitted to hospital with laboratory or clinical evidence of COVID-19 and eligible for propensity-score matching. Overall mortality was 49% (79/161) in patients with ILD with COVID-19. After matching ILD patients with COVID-19 had higher mortality (HR 1.60, Confidence Intervals 1.17-2.18 p=0.003) compared with age, sex and co-morbidity matched controls without ILD. Patients with a Forced Vital Capacity (FVC) of <80% had an increased risk of death versus patients with FVC ≥80% (HR 1.72, 1.05-2.83). Furthermore, obese patients with ILD had an elevated risk of death (HR 2.27, 1.39-3.71). CONCLUSIONS: Patients with ILD are at increased risk of death from COVID-19, particularly those with poor lung function and obesity. Stringent precautions should be taken to avoid COVID-19 in patients with ILD. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Trachalaki A, Irfan M, Wells AU, 2020, Pharmacological management of Idiopathic Pulmonary Fibrosis: current and emerging options, EXPERT OPINION ON PHARMACOTHERAPY, Vol: 22, Pages: 191-204, ISSN: 1465-6566
Stock CJ, Conti C, Montero-Fernandez Á, et al., 2020, Interaction between the promoter MUC5B polymorphism and mucin expression: is there a difference according to ILD subtype?, Thorax, Vol: 75, Pages: 901-903, ISSN: 0040-6376
The MUC5B promoter variant rs35705950 is associated with idiopathic pulmonary fibrosis (IPF). MUC5B glycoprotein is overexpressed in IPF lungs. We examined immunohistochemical expression of MUC5B in different interstitial lung disease patterns according to rs35705950 T-allele carriage. We observed increased expression of MUC5B in T-allele carriers in both distal airways and honeycomb cysts in patients with IPF (n=23), but no difference in MUC5B expression according to T-carrier status in the distal airways of patients with idiopathic non-specific interstitial pneumonitis (n=17), in scleroderma-associated non-specific interstitial pneumonitis (n=15) or in control lungs (n=20), suggesting that tissue overexpression in MUC5B rs35705950 T-carriers is specific to IPF.
Krauss E, Tello S, Dartsch RC, et al., 2020, Assessing the effectiveness of pirfenidone in changing the natural course of idiopathic pulmonary fibrosis: the data from European IPF Registry (eurIPFreg), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Price LC, Kouranos V, Dimopoulos K, et al., 2020, Sarcoidosis-associated Pulmonary Hypertension: A London Cohort, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Richeldi L, Wells AU, Cottin V, et al., 2020, Does excluding subjects with features similar to IPF affect the results of the INBUILD trial of nintedanib?, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Bonifazi M, Sverzellati N, Negri E, et al., 2020, Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Flaherty KR, Wells AU, Cottin V, et al., 2020, Effects of nintedanib on progression of ILD in patients with fibrosing ILDs and a progressive phenotype: further analyses of the INBUILD trial, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Kokosi M, Trachalaki A, Stock C, et al., 2020, Serum KL-6 identifies ILD patients with a progressive fibrosing phenotype, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Behr J, Nathan SD, Harari S, et al., 2020, Outcomes at Week 12 from three RCTs of sildenafil with and without antifibrotics in advanced IPF, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Tomassetti S, Ravaglia C, Wells A, et al., 2020, Historical eye on idiopathic pulmonary fibrosis: redefining the mortality scenario, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Tsitoura E, Trachalaki A, Vasarmidi E, et al., 2020, Collagen1a1 mRNA expression in BAL cells is associated with disease progression and poor survival in ILDs, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Stock C, Molyneaux P, Saunders P, et al., 2020, MUC2MUC5B and TOLLIP variants: no association with disease progression and survival in an IPF cohort, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Tomassetti S, Pavone M, Ravaglia C, et al., 2020, Clinical management implications of histologic information provided by lung biopsy in the multidisciplinary evaluation of interstitial lung diseases, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Sweiss NJ, Korsten P, Syed HJ, et al., 2020, When the Game Changes Guidance to Adjust Sarcoidosis Management During the Coronavirus Disease 2019 Pandemic, CHEST, Vol: 158, Pages: 892-895, ISSN: 0012-3692
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