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1.
Therapeutic Advances in Musculoskeletal Disease ; 14, 2022.
Article in English | EMBASE | ID: covidwho-2009255

ABSTRACT

Background: The results of randomized controlled (RCT) and retrospective studies have expanded the armamentarium of drugs for systemic sclerosis (SSc) – interstitial lung disease (ILD) treatment. The correct positioning of these drugs is not yet clarified. Objectives: Systemic literature review (SLR) on rituximab (RTX), tocilizumab (TCZ), nintedanib and abatacept (ABT) for the treatment of SSc-ILD. The results of the SLR were used to create a dedicated survey. Design: The study was performed as a systematic review. Data sources and methods: the SLR was performed using the following terms: “(systemic sclerosis OR scleroderma) AND (interstitial lung disease OR lung fibrosis OR pulmonary fibrosis) AND (rituximab OR tocilizumab OR abatacept OR nintedanib)”. The results of the SLR were integrated in a survey including 8 domains. These were sent to all EUSTAR members and to the participants of the 2020 Scleroderma World Congress. Results: 41 studies (34 on RTX, 5 on TCZ, 2 on ABT, and 1 on nintedanib) were identified. RCTs supported the use of TCZ and nintedanib, while retrospective studies supported the use of RTX for SSc-ILD. No clear data were obtained about ABT. The survey showed that RTX is the most available option (96%) whereas the most frequent reason for targeted therapy introduction is lung progression while on csDMARDs (86% RTX, 59% TCZ and 63% nintedanib). Combination therapy was the most frequently mentioned therapeutic scheme for nintedanib (75%) and RTX (63%). Physicians’ perception of safety was similar for all drugs, while drug efficacy was the same for RTX and nintedanib, followed by TCZ (4.8 ± 2). The most frequently raised concerns pertained to efficacy, safety and combination regimens. Conclusion: Our SLR supports the use of RTX, TCZ and nintedanib for SSc-ILD patients and underlines the need for more data about upfront combination versus monotherapy. It also highlighted the need to identify predictors supporting drug choice according to both pulmonary and extra-pulmonary manifestations.

2.
Annals of the Rheumatic Diseases ; 81:599, 2022.
Article in English | EMBASE | ID: covidwho-2009016

ABSTRACT

Background: Interstitial Lung Disease (ILD) is the most common lung involvement in rheumatoid arthritis (RA) and leads to increased morbidity and mortality. Some retrospective observational studies suggest that abatacept (ABT) could be effective and safety, although there are no clinical trials and prospectively collected data are scarce. Objectives: To evaluate prospective the effectiveness and safety of ABT in patients with ILD associated RA (ILD-RA). Methods: Design and Protocol: We performed a multicenter, prospective, observational study of patients with interstitial lung disease secondary to rheumatoid arthritis (ILD-RA) receiving ABT between 2015 and 2021. The patients were assessed using high-resolution computed tomography and lung function tests at the beginning of treatment (V0), at 12 months (V12), and at the end of follow-up in 2021 (fV). The study was approved by the Ethics Committee (Code 1719-N-15). Main variable: effectiveness of ABT according to evolution of ILD at the end of follow-up: (1) improvement (ie improvement of FVC ≥10% or DLCO ≥15% and no radiological progression), (2) no progression (stabilization or improvement in FVC ≤ 10% or DLCO <15% and no radiological progression), (3) progression (worsening of FVC >10% or DLCO >15% and radiological progression) or (4) death. Other variables: clinical and analytical characteristics, treatments and safety (infections, hospitalization and mortality). Statistical analysis: Cox regression analysis to identify factors associated with worsening of ILD-RA treated with ABT. Results: Thirty-eight ILD-RA patients started ABT treatment during prospective follow-up. A total of 22/38 (57.9%) were men and the mean (SD) age was 66.1 (9.1) years. The mean (SD) evolution of ILD was 43.9 (30.0) months and the median (IQR) time with ABT was 17.0 (12.1-34.8) months. The baseline clini-cal-epidemiological characteristics and pulmonary progression of the patients are shown in Table 1. At the end of follow-up (fV) 28/38 (73.6%) had improvement/stabilization and 7/38 (18.4%) progressed and 3/38 (7.8%) of them died (COVID-19 pneumonia, respiratory infection and ILD progression, respectively). There were no signifcant differences in FVC (75.3 [8.7] vs 77.7 [14.6];p=0.775) or in FEV1 (83.9 [10.7] vs 84.7 [13.2];p=0.416) nor in the DLCO (61.0 [17.4] vs 60.7 [15.2];p=0.789) at the end of follow-up. There was a greater numberwith improvement/stabilization among the patients who were in combination with Methotrexate compared to those who were in monotherapy (83.3% vs 39.1%;p=0.046). The baseline variables that were independently associated with progression-mortality of ILD-RA in fV were: baseline FVC (OR [95% CI], 0.895 [0.805-0.996];p=0.042) and duration of ILD-RA (OR [95% CI], 1.204 [1.148-2.112;p=0.046]). Two patients discontinued ABT during follow-up due to insufficient joint and pulmonary response. Conclusion: More than half of the patients with ILD-RA treated with ABT manage to stabilize or improve their lung disease after a median follow-up of 17 months. Patients who worsen or die have lower baseline FVC values and ILD-RA with a longer evolution time.

3.
Annals of the Rheumatic Diseases ; 81:1465-1466, 2022.
Article in English | EMBASE | ID: covidwho-2008962

ABSTRACT

Background: Interstitial lung disease (ILD) in connective tissue diseases (CTD) is an important cause of morbidity and mortalitiy. Objectives: To evaluate ILD in CTD (systemic sclerosis, myositis, Sjögren syndrome, rheumatoid arthritis, mixed connective tissue disease), sarcoidosis and interstitial pneumonia with autoimmune features and its progression in 12 months evaluated through high resolution computed tomography (HRCT) and pulmonary function test (PFT). Methods: A retrospective single tertiary center cohort study in CTD-ILD outpatients seen between 2012 and 2021. Clinical, serological data, PFT and HRCT results were collected. ILD patterns were classifed into: usual interstitial pneumonia (UIP), inconsistent UIP, nonspecifc interstitial pneumonia (NSIP), fbrosing NSIP, organizing pneumonia, interstitial lymphoid pneumonia and associated to sarcoidosis. Progression of ILD was defned as:->10% decline in FVC in PFT.->15% decline in DLCO in PFT.-Progression of fbrosis in HRCT. IBM SPSS v23 was used for statistical analysis. Results: 51 patients were collected. Baseline characteristics are shown in Table 1. Figure 1 shows ILD progression in 1 year. During follow up, 1 patient with sarcoidosis died of COVID19 bilateral pneumonia. Conclusion: In our series most patients were middle aged women. Anti-Ro antibodies and smoking status (former or current) were common among patients. Common clinical features were Raynaud (45%), skin affection (45%) and arthritis (40%). 47% of the patients expressed dyspnea at ILD diagnosis. 29,4% were treated with MP pulses, 23,5% with rituximab, 31,4% with mycofenolate mophetil. Fibrosing pattern in HRCT (UIP and fb-NSIP) was the most prevalent. 20% of the patients had progressive fbrosis under PFT criteria and 18% under HCRT. More studies of ILD-CTD are necessary to identify factors for progression and response to treatment and throw out more conclusions of prediction and prognosis of disease.

4.
Annals of the Rheumatic Diseases ; 81:1673, 2022.
Article in English | EMBASE | ID: covidwho-2008918

ABSTRACT

Background: Intersticial lung disease (ILD) is a common condition in patients with systemic autoinmune diseases (SAI) that is characterized by increased morbility and mortality. Coronavirus disease 2019 (COVID-19), caused by SARS-CoV-2, has posed huge challenges worldwide and previous studies suggest that ILD patients experience a more severe clinical course. Objectives: To analyze COVID-19 infection effects in patients with ILD associated systemic autoinmune disease (ILD-SAI) and to describe their vaccination status. Methods: Design We performed a multicenter, retrospective, observational study from 6 teaching hospitals in Andalusia, Spain. Study protocol We included ILD-SAI patients: rheumatoid arthritis (RA), systemic sclerosis (SS) and infam-matory myopathies (IM), assisted in reumatology consultations in 2021. Variables COVID-19 infection was the main variable that we collected and it was confrmed by a positive result on a PCR test. Secondary variables: (1) COVID-19 severity defned as hospitalization or death;(2) vaccination status;(3) temporary relationship between infection and vaccination. Other variables included: clinical, epidemiological characteristics, treatments received, type of ILD (UIP/NSIP), pulmonary function testing and high-resolution computed tomography. Statistic analysis Tw o multivariable logistic regression analysis to indentify the 'COVID-19' and 'severe COVID-19' associated factors. Results: We included 176 ILD-SAI, of whom: 105 (59.7%) had RA, 49 (27.8%) had SS and 22 (12.54%) had IM. The main baseline characteristics for the over-sall simple and te 3 subgroups are shown in Table 1. We recorded 22/179 (12.5%) SARS-CoV-2 infections, 7/22 (31.8%) of them were severe and 3/7 (42.85%) died. As to the vaccination, 163/176 (92.6%) patients received the complete dosis. Among those correctly vaccinated 18/163 (11%) had the SARS-CoV-2 infection, 4/18 (22.2%) after the vaccinated date and 14/18 (77.7%) when they still dint't have the complete vaccination. From the 13 not vaccinated patients, 4/13 (30.7%) had COVID-19. As to frequency, COVID-19 severity and vaccination, there were no difference between subgroups of CTD-ILD patients. The risk factors associated with the COVID-19 infection were the last FVC (OR [CI 95%], 0.971 [0.944-0.998];p=0.048), the vaccination (OR [CI 95%], 0.185 [0.049-0.691];p=0.012) and the Rituximab treatment(OR [CI 95%], 3.172 [1.028-6.785];p=0.045). Moreover, the only variable associated independently with the severe COVID-19 was the protective effect of vaccination (OR [CI 95%], 0.020 [0.003-0.119];p<0.001). Conclusion: A total of 12.5% ILD-SAI patients were COVID-19 infected, most of them without the complete vaccine. Rituximab and a deterioration of FVC were risk factors for the COVID-19 wehreas the vaccination was a protective factor for the mild and severe infection.

5.
J Clin Med ; 11(15)2022 Aug 08.
Article in English | MEDLINE | ID: covidwho-1997677

ABSTRACT

Systemic sclerosis (SSc) is a chronic autoimmune disease with a poor prognosis. Among the various complications of SSc, treatment options for the fibrotic lesions, skin sclerosis, and SSc-associated interstitial lung disease (SSc-ILD) have been limited. However, since 2019, the efficacy and safety of nintedanib, tocilizumab, and rituximab for SSc or SSc-ILD have been demonstrated in double-blind, randomized, placebo-controlled trials, respectively. The antifibrotic agent nintedanib was approved for SSc-ILD in all regions of the United States, Europe, and Japan after the SENSCIS study confirmed that it suppressed the reduction in forced vital capacity (FVC), a measure of SSc-ILD. Tocilizumab, an anti-interleukin-6 receptor antibody, was approved for the treatment of SSc-ILD in the United States after the FocuSSced study showed that it inhibited the decrease in FVC. Rituximab, an anti-CD20 antibody, showed improvement in both modified Rodnan skin score, a measure of skin sclerosis, and FVC in the DESIRES study, and was approved in Japan for the treatment of SSc itself. With the development of these three drugs, SSc treatment is entering a new era. This paper outlines the latest advances in SSc therapeutics, focusing on nintedanib, tocilizumab, and rituximab.

6.
Journal of Cystic Fibrosis ; 21:S93, 2022.
Article in English | EMBASE | ID: covidwho-1996782

ABSTRACT

Background: Respiratory management of cystic fibrosis (CF) relies on accurate monitoring of trends in lung function. The COVID-19 pandemic accelerated uptake of home spirometers at our paediatric & adult CF centres. Objectives: To establish the reliability and consistency of home-measured spirometry compared to clinic spirometry in children with CF. Methods: A single centre retrospective study. A timeline was constructed for each individual consisting of 3 pre-pandemic hospital clinic sessions and 3 subsequent virtual sessions. The acceptable period between sessions was 3–12weeks. Remote deviceswere Nuvoair® Next or Spirobank® Smart. Control data from CF adults with concurrent clinic and home Nuvoair® data in 2019. Accepted FVC & FEV1session values were used to calculate coefficient of variance (CoV). Sessions graded as ‘F’ (ATS guidelines) were noted but excluded. GLI percent predicted values were used, with height values interpolated from growth charts if necessary. Results: Sequential spirometry data and baseline demographics are shown in table 1 (n = 139). The proportion of acceptable and unacceptable spirometry (ATS) did not differ between Nuvoair® and hospital measurements. There were more A grades and fewer F grades with hospital spirometry. (Table Presented) Conclusions: Routine home spirometry had acceptable variability and quality compared to hospital measures. The step-up in home spirometry measurements for children using Nuvoair® was not seen in pre-pandemic adult data. The differences between home and hospital measures in children suggest an influence of isolation above that of equipment differences.

7.
Journal of Cystic Fibrosis ; 21:S82, 2022.
Article in English | EMBASE | ID: covidwho-1996774

ABSTRACT

Objectives: To analyse characteristics of people with cystic fibrosis (PwCF) who were using home spirometry devices (HS) during 2020–2021 Methods: During the COVID-19 pandemic, the CF Foundation (CFF) partnered with a technology vendor, ZephyRx, to distribute MIR HS devices to eligible PwCF. During 04/2020–12/2021, 20,157 spirometers were shipped to PwCF. PwCF enrolled in the CFF patient Registry (CFFPR) provided an additional consent to have their HS values linked to their CFFPR data. An application programming interface (API) was built to allow transfers of HS data (FEV1, FVC, FEF25–75, sex, date of birth, height) from each device. Each record contained a CFFPR ID to enable its linkage to the CFFPR. This analysis uses CFFPR data to describe the HS cohort and the data obtained through API to characterise HS utilisation trends. Demographic and clinical characteristics between the HS cohort and the 2019–2020 CFFPR population ages 7 and older were also compared. Results: 272 (94.4%) CF programs participated in the HS program. Records of 1,537 patients, who had activated their device by January 10, 2021, or earlier were linked to CFFPR. The cohort was 69.8% adult, 89.5% Caucasian, 57.8% female, and had a mean age of 27.8, and mean FEV1 of 79.9% predicted. When compared to the CFFPR population, the HS cohort was older, contained more Caucasians and females, and had lower lung function. The median number of acceptable FEV1 measurements supplied per PwCF was 4 (IQR 2–8). 1065 (69%) PwCF in the HS cohort continued to use their device 6 months from activation. Conclusions: HS data has the potential to augment care and research databases like the CFFPR. Little is known about PwCF’s long-term usage of HS devices in a real-world setting. While the HS cohort is small and may be biased compared to the CFFPR population, we have established a reliable channel for collecting HS data and that PwCF’s usage patterns suggests that most are using the devices on a regular basis.

8.
Journal of Cystic Fibrosis ; 21:S46, 2022.
Article in English | EMBASE | ID: covidwho-1996756

ABSTRACT

Objectives: The use of digital technology for remote monitoring has increased within CF care in recent years, including the use of remote spirometry. The challenges posed by the COVID-19 pandemic have also meant that delivery of CF care virtually has been required. The NuvoAir platform allows for remote lung function monitoring and is validated for spirometry, however there is no real-world data comparing results to hospital spirometry within an adult CF cohort. FEV1 is an established marker of disease progression within CF. Accurate spirometry results are therefore key in guiding appropriate decision-making. Repeatability of FEV1 and FVC is defined as results being within 150 mls of each other. Methods: FEV1 (L) completed by CF patients at St Bartholomew’s Hospital, on the same day on the NuvoAir Air Next spirometer and SentrySuite Vyaire Medical software, were recorded. Spirometry was completed at either an outpatient review, MDT clinic or during admission. Results were collected between August 2020 and December 2021. Results: A total of 46 sets of results were recorded. The mean difference between FEV1 (L) on the 2 devices was 0.035L (1.6%). The correlation between the FEV1 (L) on the NuvoAir Air Next and SentrySuite was statistically significant (p = 0, r = 0.97). The number of FEV1 results that varied by more than 150mls between devices was 22/46 (47.8%). Conclusion: FEV1 results from the 2 devices showed a statistically significant correlation and a small mean difference. However, as almost half of the sets of results varied by more than is acceptable for determining repeatability, the 2 devices cannot consistently be perceived to be comparable in clinic practice. Further data collection is needed to review the sensitivity to change on the NuvoAir Air Next device compared with hospital spirometry software within a CF cohort.

9.
European Journal of Molecular and Clinical Medicine ; 9(2):438-458, 2022.
Article in English | EMBASE | ID: covidwho-1981086

ABSTRACT

Introduction: Pulmonary Function Tests (PFTs), especially spirometry is an established mode of assessing chronic lung diseases especially Asthma. Spirometric reference values are essential in assessing pulmonary function. Normative values of these tests differ from population to population and with difference in methods and apparatus used. The normal standards for pulmonary function measurements among the hilly areas of Himachal Pradesh is not reported yet. Aim: To measure the normative values of spirometry (FEV1, FVC, FEV1/FVC, PEFR) in children of 5- 18 years age range living in and around Solan district of Himachal Pradesh, India. Material and Methods: This cross-sectional study was carried out at M.M Medical College and Hospital, Kumarhatti, Solan, HP, India and comprised school-going children and nearby community aged 5-18 years. After noting their gender, age, height and weight, the pulmonary function test measures, Forced vital capacity (FVC), Forced expiratory volume in 1 second (FEV1), FEV1/FVC%, FEF 25-75% and peak expiratory flow rate were taken. Simple and multiple regression models were used for the prediction of pulmonary function test values. SPSS 20 was used for statistical analysis Results: Of the 200 participants, 110(55%) were boys and 90 (45%) were girls. The mean age was 12.47±3.27 years. The means height, weight, forced expiratory volume in 1 second, force vital capacity, peak expiratory flow rate FEV1/FVC% and FEF 25-75% were 147.39±16.07cm, 41.30±12.38kg, 2.54±0.70, 2.90±0.8, 5.42±1.30, 87.41±3.85% and 2.90±0.84 respectively. All the three variables - Age, Height and Weight-had significant linear relationship and positive correlation with the pulmonary function test values (p0.7). Among these three variables maximum correlation was found with height (r=>0.8). Conclusion: Age, height and weight had statistically significant and positive correlation with the PFT values, both for boys and girls. Height was found to be most strongly and positively correlated with the PFT values. Overall significant difference was seen in FEV1/FVC and FEF 25-75 among boys and girls except FEV1, FVC and PEFR of the same age group. The fitted regression equations would help to predict the PFT values for the Indian children living in hilly areas at given age, height and weight. This study should be seen as a pilot study and will require data from a large population to establish normal values for our population.

10.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927926

ABSTRACT

The COVID-19 let sequelae not only in the respiratory system but also in several other systems, for instance in the skeletal muscle and in the immune response. This study evaluated the effects of 12 weeks of pulmonary rehabilitation (PR), constituted by aerobic (30 minutes of treadmill training at 75% of maximum heart rate) and resistance training (30 minutes at 75% of 1 maximum repetition) on 33 moderate and severe post-COVID-19 patients. The results demonstrated that PR effectively improved lung function, denoted by improved FVC (p<0.02), FEV1 (p<0.02), FEV1/FVC (p<0.01), MEF25% (p<0.006), MEF50% (p<0.03), and MEF75% (p<0.02). PR also improved lung mechanics, denoted by improved respiratory impedance (Z5hz, p<0.03);respiratory reactance (X5Hz, p<0.01), resistance of the whole respiratory systems (R5Hz, p<0.03), central airway resistance (RCentral, p<0.03), and peripheral airway resistance (RPeripheral, p<0.02). In addition, PR improved peripheral muscle strength denoted by increased right (p<0.02) and left (p<0.01) hand grip strength and the respiratory muscle strength, denoted by increased maximum inspiratory pressure (p<0.02) and maximum expiratory pressure (p<0.03). Of note, PR reduced pulmonary (breath condensate) inflammation, as observed by reduced levels of the pro-inflammatory cytokine IL-1beta (p<0.0001), IL-6 (p<0.0001), while increased the levels of the anti-inflammatory cytokine IL- 1RA (p<0.0004) and IL-10 (p<0.003), beyond to increase the levels of the cytokines with anti-viral properties, IFN-gamma (p<0.0002) and IFN-beta (p<0.008). Such response was also observed in the serum, as denoted by reduced levels of pro-inflammatory IL-1beta (p<0.006), and IL-6 (p<0.01), while increased the levels of anti-inflammatory cytokine IL-1RA (p<0.0001), and IL-10 (p<0.0001). PR also increased the serum levels of the cytokines with anti-viral properties, IFN-gamma (p<0.02) and IFN-beta (p<0.001). In conclusion, PR reveals to be beneficial for post-COVID-19 patients, mitigating the sequelae observed in the respiratory system, skeletal muscle and in the immune response.

11.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927925

ABSTRACT

Introduction: PrecISE is an ongoing Phase II clinical trial sponsored by the National Heart, Lung, and Blood Institute to investigate the efficacy of several treatments for severe asthma. The threat of COVID-19 has raised interest in obtaining reliable spirometry data for asthma research and clinical care in a remote, “no-touch” fashion. Prior studies of the accuracy of remote spirometry have not included real-time coaching. The PrecISE investigators hypothesized that remote spirometry with real-time video coaching could provide an accurate FEV1 for use as a study endpoint in a clinical trial setting. Methods: PrecISE network participants had remote spirometry post-bronchodilator (4 puffs of albuterol) measured with video coaching from trained research coordinators using the ZEPHYRx platform connected to MIR Spirobank Smart handheld spirometers. Remote spirometry measurements occurred within a +/- 3-day window from scheduled in-person PrecISE visits during which in-person spirometry with bronchodilator challenge was measured with standard equipment (Vyaire Medical). All measurements occurred during the screening/run-in period of the PrecISE protocol. Both remote and in-person spirometry was overread by the PrecISE Spirometry Core and only included in analysis if sessions met ATS acceptability and reproducibility criteria. Correlations between remote and in-person FEV1 and FVC were analyzed, and Bland-Altman plots generated. As a comparison, within subject biological variability was measured using data from separate in-person visits during the screening/run-in period. Results: A total of 128 pairs of remote/in-person spirometry data were obtained. The mean FEV1 for remote spirometry was 2.50 L (SD 0.81) and for inperson spirometry was 2.42 L (SD 0.80), with an estimated correlation of 0.95 (95% CI: 0.93, 0.97). The mean difference in FEV1 (in-person - remote) was -0.07 L (95% CI: -0.11, -0.03, SD 0.25). The mean FVC for remote spirometry was 3.72 L (SD 1.01) and for in-person spirometry was 3.53 L (SD 0.93), with an estimated correlation of 0.91 (95% CI: 0.87, 0.93). The mean difference in FVC (in-person - remote) was -0.19 L (95% CI: -0.27, -0.12, SD 0.42). A total of 142 pairs of repeated in-person spirometry measurements were performed (median time between measurements: 43 days), with mean difference in FEV1 of -0.01 L (95% CI: -0.06, 0.03) and FVC of -0.02 L (95% CI: -0.07, 0.03). Bland-Altman plots for FEV1 differences are shown in Figure 1. Conclusions: Remote spirometry with real-time video coaching provides a reliable FEV1 measurement which correlates closely with in-person spirometry and is suitable for use in clinical trials. (Figure Presented).

12.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927895

ABSTRACT

Rationale: Fibrotic hypersensitivity pneumonitis (FHP) is an irreversible lung disease with high morbidity and mortality. We sought to evaluate the safety and effect of pirfenidone on disease progression in such patients.Methods: In a phase 2 double-blind, single-center trial, we randomly assigned, in a 2:1 ratio, adults with FHP to receive either oral pirfenidone (2403 mg/day) or placebo for 52 weeks. Patients had to have CT lung fibrotic abnormalities affecting ≥5%, worsening respiratory symptoms, and either an increase in the extent of fibrosis on CT or relative decline in the FVC% of ≥5% within the 24-months before screening. The primary endpoint was the mean change from baseline to week 52 in %FVC. Secondary endpoints included progression-free survival (PFS, time to the first occurrence of any one of the following: a relative decline of ≥10% in FVC and/or DLCO, acute exacerbation, a decrease of ≥50 m in the 6-minute walk distance, increase in background prednisone by ≥10 mg or introduction of corticosteroids and/or steroid-sparing drugs, or death), change from baseline to week 52 in FVC slope and mean %DLCO, all-cause hospitalizations, CT progression of lung fibrosis, and safety. Results: After 40 patients had been randomized (mean age 67.1 years, 42.5% males) the study was stopped due to slow recruitment due to the COVID-19 pandemic. At baseline, demographics, smoking and inciting antigen exposure history, lung function, 6-minute walk distance, extent of CT lung fibrosis, and immunosuppressive therapy were balanced in both groups. There was no significant difference between the pirfenidone and placebo groups after adjusting for baseline %FVC and concomitant immunosuppressive therapy (p=0.88) in mean change from baseline to week 52 in %FVC. Secondary endpoints showed no difference between groups in change from baseline to week 52 in FVC slope, mean %DLCO, all-cause hospitalization and CT progression of lung fibrosis. However, a decrease in PFS favored pirfenidone (Table). The percentages of patients with any adverse events (AE) were similar in both groups. Nausea and rash, respectively, led to transient dose reduction of study treatment in 2 patients in the pirfenidone group. There were no treatment-related serious AE or AE leading to discontinuation of study treatment. No death occurred in the pirfenidone group and one death (respiratory) occurred in the placebo group. Conclusions: The trial was under powered to detect a difference in the primary endpoint. Pirfenidone was found to be tolerable and safe and compared to placebo reduced PFS in patients with FHP.

13.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927888

ABSTRACT

Background: COVID-19 disease can cause a wide variety of long-term health problems, including impaired lung function, reduced exercise performance, and decreased quality of life. Objectives: Our study aimed to investigate the efficacy, feasibility and safety of pulmonary rehabilitation in patients with COVID-19 and to compare the results between patients with a mild / moderate and severe / critical course of the disease. Methodology: Patients in the post-acute phase of a mild to critical course of COVID-19 admitted to a comprehensive pulmonary rehabilitation program were included in this prospective and observational cohort study, 43 patients were included in the study (20 with mild / moderate COVID and 23 with severe / critical COVID-19). Main measures: Several measures of exercise performance, 6-minute walking distance, lung function (forced vital capacity (FVC)), and quality of life (36-question short-format health survey (SF-36 )). Results: Results: At admission, the patients had a reduced walking distance (mild: median 401 m, interquartile range (IQR) 335-467 m;severe: 108 m, 84-132 m), a deteriorated FVC (mild: 72 % ,;severe: 35%,) and a low SF-36 mental health score (mild: 52 points, severe: 32 points. The patients received sessions adjusted to their physical capacities, the patients of both subgroups improved in the test of 6 minute walk (mild / moderate: +54 m, severe / critical: +117 m, both p <0.002), FVC (mild / moderate: + 8.9%, p = 0.004;severe / critical: + 12, 4%, p <0.003) and mental component SF-36 (mild / moderate: +6.8 points, p = 0.062;severe / critical: +16.7 points, -p <0.005). Conclusions: An exercise program Well structured results in a benefit in the areas of aerobic capacity, lung volumes and quality of life, in this sense it is recommended to expand population samples to be able to apply our protocol to other centers in charge in the rehabilitation of COVID-19 patients. Key Words: Exercise, Coronavirus, Rehabilitation.

14.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927848

ABSTRACT

Introduction: Acute Exacerbation of Chronic Obstructive Pulmonary Disease (AECOPD) is a major cause of hospitalization and re-admissions. Lack of standardized management and non-adherence to guideline-directed treatment may lead to poor outcomes and increase cost. Interventions implemented by health systems to reduce readmissions have had varied success. Heterogeneity in the target patient population is a significant challenge. The Cleveland Clinic COPD Care Path consists of an admission order set that incorporates multi-disciplinary management, evidence-based medications, and postdischarge integrated care. In this study, we examined impact of this Care Path on quality metrics and 30-day readmissions of patients with proven COPD on spirometry. Methods: We studied patients with spirometry proven persistent airflow obstruction (postbronchodilator FEV1/FVC<70) admitted to the general nursing floor with AECOPD during the 3 years prior to the COVID pandemic (February 2, 2017 to January 31, 2020), excluding those who left against medical advice, hospice and transplant patients. Patient's Care Path status (On vs Off), age, gender, BMI, baseline lung function and comorbidities were recorded. We measured process metrics such as appropriate use of antibiotics and corticosteroids, and post-discharge integrated disease management (rates of prescribing long-acting bronchodilator, follow-up appointments). 30-day readmission rate, length of stay (LOS) observed to expected (O: E) ratio and cost per case were recorded. For continuous variables, we used means and standard deviations and the ANOVA test for statistical analysis. For categorical variables, percentages, and the t- test were used. The level of statistical significance was set at p < 0.05. Results: Of the total of 857 patients with airflow obstruction, the Care Path was utilized in 52.8% and 21.94% were readmitted within 30 days. Lower re-admissions were associated with lower comorbidity index and completed follow-up appointments. Lung function, long acting bronchodilator prescription and cost or length of index hospitalization did not affect readmission. The care path was utilized more among patients with lower FEV1/FVC ratio but less in patients with concomitant heart failure. Use of the care path was associated with more follow-up appointments (scheduled and completed), long-acting bronchodilator prescription on discharge, lower cost but not length of stay. On-Care-Path patients did not have a reduced risk of readmission on univariate analysis. Conclusions: The findings from this retrospective study of patients with spirometry proven COPD suggest that using standardized care path for AECOPD hospitalizations is associated with lower cost and facilitates transitions of care. However, length of stay and 30-day readmission rates are unaffected. (Figure Presented).

15.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927815

ABSTRACT

Introduction: Interstitial lung disease (ILD) comprises a heterogeneous group of diseases affecting the lung interstitium often associated with significant morbidity and mortality. The Australasian Interstitial Lung Disease Registry (AILDR) launched in 2016 with the concurrent aims to: a) provide a valuable resource for high quality ILD research to further understanding of ILD and b) improve care for ILD patients across Australia and NZ. Consisting initially of four pilot sites, over time the registry has expanded to 21 sites across Australasia. Methods: Consecutive ILD patients attending any of the registered ILD centres across Australia and NZ are eligible to enrol in the AILDR following provision of informed consent. Comprehensive data including demographics, ILD diagnosis, objective functional markers (baseline and subsequent tests) and treatment parameters are collected and stored on a secure online platform. We report data from the AILDR since initiation in May 2016 to 30th September 2021 inclusive. Results: In total 2140 participants were enrolled from 16 sites at a mean rate of 43/month (mean age 65.8±13.3years;1185 (55.4%) male;982 (45.9%) ever-smokers;mean BMI 29.4±5.9kg/m2). Baseline functional parameters demonstrated mean FVC 85.6±21.7% predicted, mean DLCO 60.5±19.4%predicted, and mean six-minute walk test (6MWT) distance 434.3±126.5metres. ILD diagnoses included: idiopathic pulmonary fibrosis (IPF) n=545 (30.3%), connective tissue disease associated ILD (CTD-ILD) n=326 (18.1%), chronic hypersensitivity pneumonitis (CHP) n=155 (8.6%), sarcoidosis n=120 (6.7%) and unclassifiable ILD n=190 (10.6%). Patients with IPF were more likely to be male (n=403, 73.9%, p<0.001) and older (72.6±8.3years, p<0.001) compared to all other ILD subtypes. A female predominance was observed for CHP (n=92, 59%, p=0.001) and CTD-ILD (n=206, 63%, p<0.001). Baseline functional parameters were lowest for those with CHP (FVC 76.8±22.4% predicted, DLCO 54.1±16.9% predicted), significantly lower comparable to the IPF group (FVC 84.8±19.6%predicted, DLCO 58.7±17.8%predicted, p<0.001). The highest baseline functional parameters were observed in those with sarcoidosis. Conclusion: We demonstrate the feasibility of a bi-national ILD registry evidenced by steady recruitment despite the COVID-19 pandemic. In this study, lower functional baseline parameters were detected in the CHP group suggesting priority research should be afforded to this group. Through a routine approach across Australasia, the AILDR aims to improve standardisation of diagnosis and management of ILD patients.

16.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927779

ABSTRACT

RATIONALE. Duchenne muscular dystrophy (DMD) is the most common and severe muscular dystrophy recognized in childhood. Genetically determined progressive dystrophin dysfunction in skeletal and multiple organ muscles is fatal before the age of 20 years without multi-specialized care. The most common cause of death is respiratory muscle failure. The primary task in caring for patients with DMD is the early monitoring of pulmonary functions. This has become difficult during the pandemic SARS-CoV-2 as the procedures generate aerosols, i.e. high risk of SARS-CoV-2 virus transmission. The aim of the study was to evaluate the capacity of the patients with DMD to perform longitudinal daily spirometry examinations at home. METHODS. The patients with DMD were recruited from the Rare Disease Centre, Medical University of Gdask, Poland (a member of the TREAT NMD Alliance Neuromuscular Network). Physical examination with anthropometry measurement, Vignos scale (VS), Brooke scale (BS), and Pulmonary Function Tests were measured during 3 months of monitoring. Participants used home spirometry (AioCare System, Healthup), twice every day. The adherence and correctness (separately for FEV1 and FVC) of spirometry examinations were evaluated. RESULTS. We recruited 20 boys with mean age 11.7(3.0) years (range 7-16 years) and BMI 22.3(5.5) kg/m∧2 with a median VS of 2.5 (IQR 6.5) and BS of 2 (IQR 4.0). During 3 months of monitoring 1301 spirometry examinations were performed;32% of them meet ATS/ERS criteria for FEV1 and FVC, 35% for FEV1 and 33% for FVC, however 50% of patients performed examinations with at least one maneuver with correct FVC. The mean adherence considering performance spirometry examinations twice a day was 39(26)% (range: 6%-98%). We observed a significant increase of FVC values during the monitoring period in 4/20 of patients based on linear regression model estimation. The study is still ongoing. CONCLUSIONS. The systematic pulmonary function monitoring at home in patients with restrictive diseases is an innovative project. The results showed that patients with DMD are able to monitor the lung function using a mobile spirometry system. We observed that in some cases, based on patient feedback, systematic spirometry tests were a form of pulmonary exercises and could be one of the factors that increase FVC after 3 months of monitoring. Personal spirometers could be used as a part of the therapy of patients with muscular dystrophy.

17.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927748

ABSTRACT

RATIONALE The COVID-19 pandemic has disrupted national spirometric surveillance of active and former U.S. coal miners since March 2020. Consequently data collected by the Health Resources and Services Administration (HRSA)-funded Black Lung Clinics Program (BLCP) represents the only major source of recent health data on U.S. former coal miners. Using the first available year of national BLCP data we examined associations between mining region and radiographic disease and lung function impairment. METHODS We analyzed pre-bronchodilator spirometry and International Labour Office chest radiograph classifications from miners seen across 15 BLCP grantees from July 1 2020 to June 30 2021. We calculated percent predicted (PP) and lower limits of normal (LLN) for forced expiratory volume in one second (FEV1) forced vital capacity (FVC) and FEV1/FVC ratio. We determined prevalence of patterns of spirometric abnormality (restrictive obstructive/mixed) and moderate to severe impairment (FEV1<70PP). We classified miners who worked the majority of their coal mining career in Kentucky Virginia or West Virginia as Central Appalachian miners. We examined associations between region worked and lung function impairment using logistic regression. RESULTS The 2,891 miners were predominantly non- Hispanic white (98.1%) and male (99.4%) with mean age 66 years (SD9.3). Mean coal mining tenure was 26 years (SD10.7) and 66% (n=1,900) were Central Appalachian miners. Thirty-seven percent had never smoked. Among those with chest radiographs (n=2,464 85%) Central Appalachian miners had a significant three-fold increase in progressive massive fibrosis (PMF) prevalence compared to miners who worked elsewhere in the U.S. (9% vs 3% p<.0001). Smoking history and spirometry were available in 66% (n=1,918). Of these 40% of never-smokers had abnormal spirometry (obstruction/mixed 10%;restriction 30%);among ever-smokers, 54% had abnormal spirometry (obstruction/mixed 27%;restriction 27%). Abnormal FEV1 was present in 30% of never smokers and 42% of ever-smokers. Mean FEV1PP was significantly lower among Central Appalachian miners compared to miners from other regions. Controlling for age, tenure, and pack-years, Central Appalachian miners had significantly elevated odds of having FEV1 impairment compared to non-Central Appalachian miners (OR 1.31, 95%CI 1.06,1.62). A subanalysis controlling for category of radiographic disease showed that odds of impairment remained elevated among Central Appalachian miners (OR 1.24, 95%CI 0.97,1.60). CONCLUSIONS Controlling for smoking, age, and tenure, former miners who worked most of their career in Central Appalachia have significantly increased odds of disabling impairment. These findings highlight the important role of HRSA-funded black lung clinics in understanding work-related lung disease among U.S. coal miners.

18.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927718

ABSTRACT

Introduction: Recognition of myositis-associated interstitial lung disease (ILD) has been increasing due to the recent identification of specific antibodies that are linked to a particular phenotype of myositis-ILD such as antisynthetase syndrome. The anti-Ro52 antibody has been found in multiple connective tissue diseases but its association with ILD is unclear. In fact, the literature on the phenotype of patients with an isolated anti-Ro52 antibody is very scarce. Case Presentation: A 57-year-old retired firefighter with history of gastroesophageal reflux presented to the hospital with a 4-week history of cough and progressive dyspnea with no other symptoms. He had presented to an urgent care facility where he tested negative for SARs-CoV-2 and was given antibiotics and inhalers without a clinical response. Patient required 2L/min of oxygen on admission. He had no other signs of autoimmune disease or other organ involvement. Chest CT showed peripheral ground glass opacities with basilar reticulations and bronchiectasis (Figure 1). WBC and CPK were normal and aldolase was mildly elevated. Initial serologies revealed an ANA 1:320 and a weakly positive p-ANCA with negative PR3/MPO antibodies. Patient was started on prednisone 60 mg and discharged five days later on mycophenolate mofetil (MMF) and 2L/min of oxygen. At 2-week follow-up, his extensive autoimmune panel showed a high titer of Anti-Ro52 antibody. An in depth ILD questionnaire revealed a family history of lupus and the remote use of a short course of steroids 20 years ago after a biceps biopsy showed polymyositis. On chart review, 10 years ago the patient had elevated CPK levels of 255 to 404, in the context of a nonspecific flank pain that resolved without intervention. At his 3-month follow-up, the patient still required 40mg of prednisone, reported dyspnea on exertion and required oxygen with ambulation. Rituximab was added to his regimen and his symptoms significantly improved;at 6-month follow-up, his FVC% improved from 53% to 70% and the patient was no longer on oxygen. Prednisone was titrated down to 10mg and he was continued on MMF 3g/day and Rituximab every 6 months. Discussion: We present a patient with Anti-Ro52 antibody myositisassociated ILD who required 3 immunosuppressive agents to control his disease. This case adds to the very scarce literature on ILD secondary to Anti-Ro52 antibody and highlights the importance of an extensive antibody testing for patients with ILD of unclear etiology, for diagnostic and therapeutic purposes. Further studies describing the phenotype of these patients are warranted. (Figure Presented).

19.
American Journal of Respiratory and Critical Care Medicine ; 205(1), 2022.
Article in English | EMBASE | ID: covidwho-1927697

ABSTRACT

Introduction: We sought to determine whether a 6-week integrated rehabilitation program improved pulmonary function, physical and mental health outcomes in patients discharged alive after surviving a severe COVID-19 pneumonia. Methods: Parallel, open-label, feasibility randomized controlled trial in participants aged 18-75 years who were discharged for severe COVID-19 pneumonia. The intervention consisted of 12 in-person home respiratory rehabilitation sessions and 6 telephonebased, emotion-centered problem-solving therapy psychological sessions. We measured the 6- minute walk test (6MWT), forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), Patient Health Questionnaire (PHQ-9), General Anxiety Disorder (GAD-7), Impact of Event Scale- Revised (IES-R) and Short Form 36 health survey (SF-36). Following the recommendation by Cocks and Torgerson, we used a one-sided 80% confidence interval to determine if this feasibility trial should proceed to a phase III trial. Results: We randomized 103 participants (mean age, 48 years;71% men). There were no differences at baseline assessments of outcomes between the two groups. Intervention participants walked 15 and 45 meters more during a 6MWT at 7 and 12 weeks, respectively, than controls after accounting for baseline distance. In both instances, the onesided 80% lower bound was above 0. Intervention participants also had a greater improvement of lung function for week 7 (mean difference FEV1, 0.05 L;95% CI, -0.14 to 0.24;mean difference FVC, 0.10 L;95% CI, -0.12 to 0.31) and at week 12 (mean difference FEV1, 0.10 L;95% CI, -0.18 to 0.37;mean difference FVC, 0.15 L;95% CI, -0.17 to 0.47). Likewise, the prevalence of depression (percentage difference PHQ-9, 24.3%;95% CI, -4.3 to 44.3), anxiety (percentage difference GAD-7, 20.7%;95% CI, 0.8 to 40.6) and post-traumatic stress (percentage difference IES-R, 14.0%;95% CI, -5.7 to 33.7) were lower in the intervention group at week 2 after hospital discharge. At week 7, the intervention group had a greater improvement of physical (mean difference, 10.8;95% CI, -1.8 to 23.4) and social (mean difference, 6.43;95% CI, -0.65 to 13.5) function on the SF-36. Conclusion: We demonstrated that an integrated rehabilitation program administered over a 6-week after discharge in Lima, Peru was both feasible and acceptable, and had positive benefits on physical and pulmonary function and on mental health during the 12 weeks of follow-up. Measured improvements in the primary outcome of 6MWT distance recommend that we consider a phase III trial to test the efficacy of our integrated rehabilitation intervention in a larger sample.

20.
Chinese Journal of Radiology (China) ; 56(4):377-384, 2022.
Article in Chinese | EMBASE | ID: covidwho-1896938

ABSTRACT

Objective To explore the application value of CT pulmonary function imaging in patients with Coronavirus Disease 2019 (COVID‑19) in the convalescent phase. Methods The COVID‑19 patients who were clinically cured and discharged from Union Hospital, Tongji Medical College, Huazhong University of Science and Technology were prospectively collected from January to April 2020. Clinical pulmonary function tests (PFTs) and CT pulmonary function imaging were performed 3 months after discharge. The Philips IntelliSpace Portal image post‑processing workstation was used to obtain the paired inspiratory‑expiratory CT quantitative indexes of the whole lung, left lung, right lung and five lobes. The patients were divided into two groups according to whether residual lesions remain in inspiratory CT images: non‑residual lesion group and residual lesion group. The chi‑square test was used to compare the differences in the PFT results between groups;the Mann‑Whitney U test was used to compare the differences in PFT indexes [forced expiratory volume in the first second as percentage of predicted value (FEV1%), FEV1/forced vital capacity (FEV1/FVC), total lung capacity as percentage of predicted value (TLC%), FVC% ] and the differences in quantitative CT indexes [lung volume (LV), mean lung density (MLD), volume change in inspiratory phase and expiratory phase (∆ LV)] between groups. Multiple linear regression was used to analyze the relationship between CT pulmonary function imaging and PFT indexes of convalescent COVID‑19 patients. Results Of the 90 patients with COVID‑19, 35 were males and 55 were females;45 were included in the non‑residual lesion group and 45 were included in the residual lesion group. Fifty‑three patients had clinical pulmonary dysfunction 3 months after discharge, including 22 patients in the non‑residual lesion group and 31 patients in the residual lesion group. In patients with residual disease, left lower lobe and right lower lobe LV, left lower lobe and right lower lobe ∆ LV in the inspiratory and expiratory phase were smaller than those without residual disease;whole lung, left lung, right lung, left upper lobe, left lower lobe and right lower lobe MLD in the inspiratory phase and left lower lobe and right lower lobe MLD in the expiratory phase were greater than those without residual disease (P<0.05). Since there was no significant difference in FEV1/FVC and FVC% between residual and non‑residual lesion groups (P>0.05), FEV1/FVC and FVC% of two groups were combined. Multiple linear regression analysis showed FEV1/FVC= 91.765-0.016×LVin‑right middle lobe+0.014×MLDex‑left lower lobe (R2 =0.200, P<0.001), FVC% =-184.122-0.358× MLDin‑right lung-0.024× ∆ LVleft upper lobe (R2 =0.261, P<0.001). There was significant difference in TLC% between residual and non‑residual lesion groups (P<0.05), so multiple linear regression analysis was performed both in the two groups. In the non‑residual lesion group, TLC% =80.645+0.031× (R2 =0.132, P<0.001);In the residual lesion group, TLC% =-110.237-0.163× LVex‑right lower lobe MLDin‑right upper lobe-0.098×MLDex‑left upper lobe -0.025×LVex‑right lower lobe (R2 =0.473, P<0.001). Conclusion CT pulmonary function imaging can quantitatively analyze the whole lung, unilateral lung and lobulated lung, thus reflecting the regional pulmonary function, providing more valuable diagnostic information for the assessment of ulmonar function in convalescent atients with COVID‑19

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