Understanding the telehealth experience of care by people with ILD during the COVID-19 pandemic: what have we learnt?

INTRODUCTION: The COVID-19 pandemic resulted in a rapid transformation of health services. This study aimed to understand the experiences of healthcare by people with interstitial lung disease (ILD), to inform future service delivery. METHODS: Four specialist clinics in tertiary centres in Australia (Victoria:2 sites; New South Wales: 1 site; Western Australia: 1 site) recruited patients with ILD during an 8-week period from March 2021. Participants completed a COVID-specific questionnaire focused on health-related experiences during 2020. RESULTS: Ninety nine (65% of 153) participants completed the questionnaire. 47% had idiopathic pulmonary fibrosis or connective tissue disease-associated ILD, 62% were female and the average age was 66 years. Whilst 56% rated their overall health in 2020 as the same as months prior, 38% indicated a worsening in health attributed to reduced physical activity and fear of contracting the virus. Access to healthcare professionals was 'good' in 61%, and 'fair-to-poor' for 37% due to missed respiratory assessments, with telehealth (mainly telephone) being perceived as less effective. 89% had contact with respiratory physicians, 68% with general practitioners, predominantly via telephone, with few video consultations. High satisfaction with care was reported by 78%, with lower satisfaction attributed to delays in assessments, disruption to usual services such as pulmonary rehabilitation, and dissatisfaction with telehealth. CONCLUSION: People with ILD were generally satisfied with their care during 2020, however reduced access to healthcare professionals was challenging for those experiencing a deterioration in health. Telehealth was largely well received but did not always meet the needs of people with ILD particularly when unwell.

[SARS-CoV-2-Infection and Interstitial Lung Disease: Position paper of the German Respiratory Society]. / SARS-CoV-2-Infektion und interstitielle Lungenerkrankungen ­ Positionspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin.

The SARS-CoV-2 pandemic had a tremendous impact on diagnosis and treatment of interstitial lung diseases (ILD). Especially in the early phase of the pandemic, when the delta variant was prevailling, a huge number of viral pneumonias were observed, which worsened pre-existing, triggered de novo occurence or discovery of previously subclincal interstitial lung diseases. The effect of SARS-CoV-2 infection - without or with accompanying viral pneumonia - on the further development of pre-existing ILD as well of new pulmonary inflitrates and consolidiations is difficult to predict and poses a daily challenge to interdisciplinary ILD boards. This position paper of the German Respiratory Society (DGP e.V.) provides answers to the most pressing questions based on current knowledge.

Post-COVID Interstitial Lung Disease and Other Lung Sequelae.

As the world emerges from the COVID-19 pandemic, clinicians and researchers across the world are trying to understand the sequelae in patients recovered from COVID-19 infection. In this article, the authors review post-acute sequelae of SARS-COV-2, interstitial lung disease, and other lung sequelae in patients recovering from COVID-19 infection.

Effects of different exercise training programs on the functional performance in fibrosing interstitial lung diseases: A randomized trial.

OBJECTIVES: We aimed to compare the effects of different aerobic exercise training (ET) programs on respiratory performance, exercise capacity, and quality of life in fibrosing interstitial lung diseases (f-ILD). METHODS: A case-control study where 31 patients with f-ILD diagnosis based on chest high-resolution computed tomography were recruited from Main Alexandria University hospital-Egypt. Ten patients were randomly assigned for only lower limbs (LL) endurance training program, and 10 patients for upper limbs, lower limbs, and breathing exercises (ULB) program for consecutive 18 sessions (3 sessions/week for 6 consecutive weeks). Eleven patients who refused to participate in the ET program were considered as control. All patients were subjected for St George's respiratory questionnaire (SGRQ), 6-minute walk test (6-MWT), forced spirometry and cardiopulmonary exercise testing (CPET) before and after ET programs. RESULTS: Fibrosing non-specific interstitial pneumonia (NSIP) and collagenic associated-ILD were the commonest pathologies among the ET groups (30% each) with mean age of 44.4±12.25 and 41.90±7.58 years for LL and ULB groups respectively and moderate-to-severe lung restriction. 6-MWT and SGRQ significantly improved after both ET programs (p<0.001). Peak oxygen consumption (VO2) improved significantly after both LL training (median of 22 (interquartile range (IQR) = 17.0-24.0) vs. 17.5 (IQR = 13.0-23.0) ml/kg/min, p = 0.032) and ULB training (median of 13.5 (IQR = 11.0-21.0) vs. 10.5 (IQR = 5.0-16.0) ml/kg/min, p = 0.018). Further, maximal work load and minute ventilation (VE) significantly improved after both types of ET training (p<0.05); however, neither ventilation equivalent (VE/VCO2) nor FVC% improved after ET (p = 0.052 and 0.259 respectively). There were no statistically significant important differences between LL and ULB training programs regarding 6-MWT, SGRQ or CPET parameters (p>0.05). CONCLUSIONS: ET was associated with improvements in exercise capacity and quality of life in f-ILD patients irrespective of the type of ET program provided.

Incidental discovery of interstitial lung disease: diagnostic approach, surveillance and perspectives.

The incidental discovery of pre-clinical interstitial lung disease (ILD) has led to the designation of interstitial lung abnormalities (ILA), a radiological entity defined as the incidental finding of computed tomography (CT) abnormalities affecting more than 5% of any lung zone. Two recent documents have redefined the borders of this entity and made the recommendation to monitor patients with ILA at risk of progression. In this narrative review, we will focus on some of the limits of the current approach, underlying the potential for progression to full-blown ILD of some patients with ILA and the numerous links between subpleural fibrotic ILA and idiopathic pulmonary fibrosis (IPF). Considering the large prevalence of ILA in the general population (7%), restricting monitoring only to cases considered at risk of progression appears a reasonable approach. However, this suggestion should not prevent pulmonary physicians from pursuing an early diagnosis of ILD and timely treatment where appropriate. In cases of suspected ILD, whether found incidentally or not, the pulmonary physician is still required to make a correct ILD diagnosis according to current guidelines, and eventually treat the patient accordingly.

Contemporary Concise Review 2021: Interstitial lung disease.

The last 2 years have presented previously unforeseen challenges in pulmonary medicine. Despite the significant impact of the SARS-CoV-2 pandemic on patients, clinicians and communities, advances in the care and understanding of interstitial lung disease (ILD) continued unabated. Recent studies have led to improved guidelines, better understanding of the role for antifibrotics in fibrosing ILDs, prognostic indicators and novel biomarkers. In this concise contemporary review, we summarize many of the important studies published in 2021, highlighting their relevance and impact to the management and knowledge of ILD.

Longstanding Phenytoin Use as a Cause of Progressive Dyspnea.

CASE PRESENTATION: A 54-year-old South African man with a medical history of type 2 diabetes mellitus, seizure disorder, OSA, and latent TB presented to the ER with gradually progressive dyspnea over months. He also reported occasional dry cough and fatigue at presentation but denied fever, chills, chest pain, leg swelling, palpitations, or lightheadedness. He was treated with a course of levofloxacin for presumed community-acquired pneumonia as an outpatient without improvement and had tested negative for COVID-19. He denied occupational or environmental exposures or sick contacts, though he had traveled back to South Africa 1 year before presentation. He had complex partial seizures for the past 22 years, which had been well controlled on phenytoin (300 mg daily). His other home medications included dulaglutide, sertraline, and atorvastatin and had no recent changes. He quit smoking 30 years ago after smoking one pack per day for 10 years.

Telehealth for patients with interstitial lung diseases (ILD): results of an international survey of clinicians.

INTRODUCTION: Clinicians and policymakers are promoting widespread use of home technology including spirometry to detect disease progression for patients with interstitial lung disease (ILD); the COVID-19 pandemic has accelerated this. Data collating clinicians' views on the potential utility of telehealth in ILD are limited. AIM: This survey investigated clinicians' opinions about contemporary methods and practices used to monitor disease progression in patients with ILD using telehealth. METHODS: Clinicians were invited to participate in a cross-sectional survey (SurveyMonkey) of 13 questions designed by an expert panel. Telehealth was defined as home monitoring of symptoms and physiological parameters with regular automatic transmission of data from the patient's home to the clinician. Data are presented as percentages of respondents. RESULTS: A total of 207 clinicians from 23 countries participated in the survey. A minority (81, 39%) reported using telehealth. 50% (n=41) of these respondents completed a further question about the effectiveness of telehealth. A majority of respondents (32, 70%) rated it to be quite or more effective than face-to-face visit. There were a greater number of respondents using telehealth from Europe (94, 45%) than Asia (51, 25%) and America (24%). Clinicians reported the most useful telehealth monitoring technologies as smartphone apps (59%) and wearable sensors (30%). Telehealth was most frequently used for monitoring disease progression (70%), quality of life (63%), medication use (63%) and reducing the need for in-person visits (63%). Clinicians most often monitored symptoms (93%), oxygen saturation (74%) and physical activity (72%). The equipment perceived to be most effective were spirometers (43%) and pulse oximeters (33%). The primary barriers to clinicians' participation in telehealth were organisational structure (80%), technical challenges (63%) and lack of time and/or workload (63%). Clinicians considered patients' barriers to participation might include lack of awareness (76%), lack of knowledge using smartphones (60%) and lack of confidence in telehealth (56%). CONCLUSION: The ILD clinicians completing this survey who used telehealth to monitor patients (n=81) supported its' clinical utility. Our findings emphasise the need for robust research in telehealth as a mode for the delivery of cost-effective healthcare services in ILD and highlight the need to assess patients' perspectives to improve telehealth utility in patients with ILD.

Could pulmonary low-dose radiation therapy be an alternative treatment for patients with COVID-19 pneumonia? Preliminary results of a multicenter SEOR-GICOR nonrandomized prospective trial (IPACOVID trial).

PURPOSE: To evaluate the efficacy and safety of lung low-dose radiation therapy (LD-RT) for pneumonia in patients with coronavirus disease 2019 (COVID-19). MATERIALS AND METHODS: Inclusion criteria comprised patients with COVID-19-related moderate-severe pneumonia warranting hospitalization with supplemental O2 and not candidates for admission to the intensive care unit because of comorbidities or general status. All patients received single lung dose of 0.5 Gy. Respiratory and systemic inflammatory parameters were evaluated before irradiation, at 24 h and 1 week after LD-RT. Primary endpoint was increased in the ratio of arterial oxygen partial pressure (PaO2) or the pulse oximetry saturation (SpO2) to fractional inspired oxygen (FiO2) ratio of at least 20% at 24 h with respect to the preirradiation value. RESULTS: Between June and November 2020, 36 patients with COVID-19 pneumonia and a mean age of 84 years were enrolled. Seventeen were women and 19 were men and all of them had comorbidities. All patients had bilateral pulmonary infiltrates on chest X­ray. All patients received dexamethasone treatment. Mean SpO2 pretreatment value was 94.28% and the SpO2/FiO2 ratio varied from 255 mm Hg to 283 mm Hg at 24 h and to 381 mm Hg at 1 week, respectively. In those who survived (23/36, 64%), a significant improvement was observed in the percentage of lung involvement in the CT scan at 1 week after LD-RT. No adverse effects related to radiation treatment have been reported. CONCLUSIONS: LD-RT appears to be a feasible and safe option in a population with COVID-19 bilateral interstitial pneumonia in the presence of significant comorbidities.

Addressing Reduced Laboratory-Based Pulmonary Function Testing During a Pandemic.

To reduce the spread of the severe acute respiratory syndrome coronavirus 2, many pulmonary function testing (PFT) laboratories have been closed or have significantly reduced their testing capacity. Because these mitigation strategies may be necessary for the next 6 to 18 months to prevent recurrent peaks in disease prevalence, fewer objective measurements of lung function will alter the diagnosis and care of patients with chronic respiratory diseases. PFT, which includes spirometry, lung volume, and diffusion capacity measurement, is essential to the diagnosis and management of patients with asthma, COPD, and other chronic lung conditions. Both traditional and innovative alternatives to conventional testing must now be explored. These may include peak expiratory flow devices, electronic portable spirometers, portable exhaled nitric oxide measurement, airwave oscillometry devices, and novel digital health tools such as smartphone microphone spirometers and mobile health technologies along with integration of machine learning approaches. The adoption of some novel approaches may not merely replace but could improve existing management strategies and alter common diagnostic paradigms. With these options comes important technical, privacy, ethical, financial, and medicolegal barriers that must be addressed. However, the coronavirus disease 19 pandemic also presents a unique opportunity to augment conventional testing by including innovative and emerging approaches to measuring lung function remotely in patients with respiratory disease. The benefits of such an approach have the potential to enhance respiratory care and empower patient self-management well beyond the current global pandemic.

COVID-19 or clinical amyopathic dermatomyositis associated rapidly progressive interstitial lung disease? A case report.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has reach pandemic proportions globally. For patients with symptoms of fever and cough accompanied by rapid lung damage progression, COVID-19 needs to be distinguished from interstitial lung disease (ILD) attributed to connective tissue disease (CTD), especially dermatomyositis (DM)/clinical amyopathic dermatomyositis (CADM) associated rapidly progressive interstitial lung disease (RP-ILD). CASE PRESENTATION: We report a case of a woman observed with fever, cough, and rapid lung damage during the epidemic. The patient had a suspicious epidemiological history, and her chest CT scans showed lung damage similar to that caused by COVID-19, but anti-Ro52 antibody was strongly positive. She was diagnosed with CADM associated RP-ILD and died 1 month later. CONCLUSIONS: During the COVID-19 epidemic, it is critical to carefully assess patients with CTD related ILD, especially RP-ILD associated with CADM. Repeated nucleic acid tests for COVID-19 are necessary to achieve accurate case diagnosis. High-resolution CT (HRCT) of the chest is presently deemed an inefficient technique to distinguishing between COVID-19 and CADM associated RP-ILD. The characteristic rashes of dermatomyositis require careful observation and can often provide diagnostic clues. For patients with CADM, a high titers of anti-Ro52 antibody may be related to the pathogenesis of RP-ILD, suggesting a poor prognosis.

Fatal outcome of anti-MDA5 juvenile dermatomyositis in a paediatric COVID-19 patient: a case report.

Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three real-time RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.

[Expert recommendations for the diagnosis and treatment of interstitial lung disease caused by novel coronavirus pneumonia].

COVID-19 is an acute infectious disease caused by a newly discovered coronavirus (SARS-CoV2). COVID-19 may manifest bilateral interstitial pneumonia on imaging. About 30%-60% of patients present varying degrees of interstitial changes, while most patients have a good prognosis. Since there's little practical instruct on treating interstitial lung disease (ILD) caused by COVID-19, we present this file as references for all the colleagues fighting with this disease. The primary findings on CT are bilateral, peripheral ground-glass opacities (GGO) and consolidation. Inter-/intra-lobular septal thickening are also common. Subpleural lines and traction bronchiectasis can be seen in some cases which indicate the presence of interstitial fibrosis. Images of severe cases are similar with those in advanced stage of nonspecific interstitial pneumonia (NSIP) and organizing pneumonia (OP). COVID-19 could present the typical two phases of diffuse alveolar damage: acute and proliferative phase on pathology. Massive pulmonary interstitial fibrosis may also be present. HRCT is the best radiological approach for the diagnosis and differential diagnosis of COVID-19, and to assess the presence of ILD. Periodical CT following-up is recommended for patients who present interstitial manifestations. Biomarkers such as KL-6, SP-D, RAGE may also helpful on evaluating the severity of interstitial fibrosis and therapeutic response. We do not suggest applying pulmonary function tests and 6-minute walking test on patients in active stage of the disease. The primary treatments in acute phase are antiviral therapy and supportive treatment. We do not suggest routine use of corticosteroids, while on patients with excessive activation of inflammatory response or rapid progression of lung lesions, a low to medium dosage of corticosteroids could be applied for a short course. Pirfenidone and Nintedanib are encouraged to apply on patients in reparative phase with evidence of progressing fibrosis. Low to medium dosage of corticosteroids is also feasible on patients with NSIP or OP manifestation in this phase, with a relatively longer course. Chinese traditional medicine and rehabilitation medicine may also helpful. Lung transplant surgery is an option for severe pulmonary fibrosis patients. Patients should receive CT following-up after be discharged from hospital, especially those whose pulmonary exudation is not well absorbed. We suggest a routine following-up on month 1, 4 and 10 after discharging, and an extended period for those who have developed irreversible interstitial fibrosis.

Outcome of Hospitalization for COVID-19 in Patients with Interstitial Lung Disease. An International Multicenter Study.

Rationale: The impact of coronavirus disease (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 the hospital with COVID-19 between March 1 and May 1, 2020, was undertaken and compared with patients without ILD, obtained from the ISARIC4C (International Severe Acute Respiratory and Emerging Infection Consortium Coronavirus Clinical Characterisation Consortium) cohort, admitted with COVID-19 over the same period. The primary outcome was survival. Secondary analysis distinguished idiopathic pulmonary fibrosis from non-idiopathic pulmonary fibrosis 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 the 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, patients with ILD with COVID-19 had significantly poorer survival (hazard ratio [HR], 1.60; confidence interval, 1.17-2.18; P = 0.003) than age-, sex-, and comorbidity-matched controls without ILD. Patients with an 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.

Atypical Chest Computed Tomography Finding of Predominant Interstitial Thickening in a Patient with Coronavirus Disease 2019 (COVID-19) Pneumonia.

BACKGROUND Coronavirus disease 2019 (COVID-19) is caused by a novel coronavirus, SARS-CoV-2, and is associated with severe respiratory disease. There are extensive publications on the chest computed tomography (CT) findings of COVID-19 pneumonia, with ground-glass opacities (GGO) and mixed GGO and consolidation being the most common findings. Those with interstitial thickening manifesting as reticular opacities typically show superimposed ground-glass opacities, giving a crazy-paving pattern. CASE REPORT We report the case of a 77-year-old man with a background of asthma-chronic obstructive pulmonary disease (COPD) overlap syndrome (ACOS) who presented with progressive cough and shortness of breath for 2 days. He was in close contact with a confirmed COVID-19 case. Reverse-transcription polymerase chain reaction analysis of a nasopharyngeal swab was positive for SARS-CoV-2. The initial chest radiograph was negative for lung consolidation and ground-glass opacities. During admission, he had worsening shortness of breath with desaturation, prompting a chest CT examination, which was performed on day 14 of illness. The chest CT revealed an atypical finding of predominant focal subpleural interstitial thickening in the right lower lobe. He was provided supportive treatment along with steroid and antibiotics. He recovered well and subsequently tested negative for 2 consecutive swabs. He was discharged after 34 days. CONCLUSIONS Interstitial thickening or reticular pattern on CT has been described in COVID-19 pneumonia, but largely in association with ground-glass opacity or consolidation. This case demonstrates an atypical predominance of interstitial thickening on chest CT in COVID-19 pneumonia on day 14 of illness, which is the expected time of greatest severity of the disease.

Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic.

The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2, has affected virtually all aspects of patient care. Health-care systems around the world are trying simultaneously to treat patients with COVID-19, prepare for its long-term impacts, and treat patients with other acute and chronic diseases. There are multiple ways that the COVID-19 pandemic will directly affect patients with fibrotic interstitial lung disease (ILD), particularly given their common risk factors for poor outcomes. Major issues for patients with ILD will include restricted access to key components of the diagnostic process, new uncertainties in the use of common ILD pharmacotherapies, limited ability to monitor both disease severity and the presence of medication adverse effects, and significantly curtailed research activities. The purpose of this review is to summarize how COVID-19 has impacted key components of the diagnosis and management of fibrotic ILD as well as to provide strategies to mitigate these challenges. We further review major obstacles for researchers and identify priority areas for future ILD research related to COVID-19. Our goals are to provide practical considerations to support the care of patients with ILD during the COVID-19 pandemic and to provide a road map for clinicians caring for these patients during future infectious disease outbreaks.