Progressive pulmonary fibrosis: an expert group consensus statement.

This expert group consensus statement emphasises the need for standardising the definition of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis being of paramount importance in ensuring appropriate initial management. Equally, case-by-case decisions on monitoring and management are essential, given the varying presentations of F-ILDs and the varying rates of progression. The value of diagnostic tests in risk stratification at presentation and, separately, the importance of a logical monitoring strategy, tailored to manage the risk of progression, are also stressed. The term "progressive pulmonary fibrosis" (PPF) exactly describes the entity that clinicians often face in practice. The importance of using antifibrotic therapy early in PPF (once initial management has failed to prevent progression) is increasingly supported by evidence. Artificial intelligence software for high-resolution computed tomography analysis, although an exciting tool for the future, awaits validation. Guidance is provided on pulmonary rehabilitation, oxygen and the use of non-invasive ventilation focused specifically on the needs of ILD patients with progressive disease. PPF should be differentiated from acute deterioration due to drug-induced lung toxicity or other forms of acute exacerbations. Referral criteria for a lung transplant are discussed and applied to patient needs in severe diseases where transplantation is not realistic, either due to access limitations or transplantation contraindications. In conclusion, expert group consensus guidance is provided on the diagnosis, treatment and monitoring of F-ILDs with specific focus on the recognition of PPF and the management of pulmonary fibrosis progressing despite initial management.

Integrating Clinical Probability into the Diagnostic Approach to Idiopathic Pulmonary Fibrosis: An International Working Group Perspective.

Background: When considering the diagnosis of idiopathic pulmonary fibrosis (IPF), experienced clinicians integrate clinical features that help to differentiate IPF from other fibrosing interstitial lung diseases, thus generating a "pre-test" probability of IPF. The aim of this international working group perspective was to summarize these features using a tabulated approach similar to chest HRCT and histopathologic patterns reported in the international guidelines for the diagnosis of IPF, and to help formally incorporate these clinical likelihoods into diagnostic reasoning to facilitate the diagnosis of IPF. Methods: The committee group identified factors that influence the clinical likelihood of a diagnosis of IPF, which was categorized as a pre-test clinical probability of IPF into "high" (70-100%), "intermediate" (30-70%), or "low" (0-30%). After integration of radiological and histopathological features, the post-test probability of diagnosis was categorized into "definite" (90-100%), "high confidence" (70-89%), "low confidence" (51-69%), or "low" (0-50%) probability of IPF. Findings: A conceptual Bayesian framework was created, integrating the clinical likelihood of IPF ("pre-test probability of IPF") with the HRCT pattern, the histopathology pattern when available, and/or the pattern of observed disease behavior, into a "post-test probability of IPF." The diagnostic probability of IPF was expressed using an adapted diagnostic ontology for fibrotic interstitial lung diseases. Interpretation: The present approach will help incorporate the clinical judgment into the diagnosis of IPF, thus facilitating the application of IPF diagnostic guidelines and, ultimately improving diagnostic confidence and reducing the need for invasive diagnostic techniques.

Home monitoring in interstitial lung diseases.

The widespread use of smartphones and the internet has enabled self-monitoring and more hybrid-care models. The COVID-19 pandemic has further accelerated remote monitoring, including in the heterogenous and often vulnerable group of patients with interstitial lung diseases (ILDs). Home monitoring in ILD has the potential to improve access to specialist care, reduce the burden on health-care systems, improve quality of life for patients, identify acute and chronic disease worsening, guide treatment decisions, and simplify clinical trials. Home spirometry has been used in ILD for several years and studies with other devices (such as pulse oximeters, activity trackers, and cough monitors) have emerged. At the same time, challenges have surfaced, including technical, analytical, and implementational issues. In this Series paper, we provide an overview of experiences with home monitoring in ILD, address the challenges and limitations for both care and research, and provide future perspectives. VIDEO ABSTRACT.

Placing the results of the SOLIDARITY trial with regards to remdesivir in perspective.

Remdesivir, a repurposed antiviral, was first accorded approval by the US Food Drug Administration (FDA) for the treatment of COVID-19 which necessitates hospitalization. However, the interim data of SOLIDARITY trial revealed no benefits with remdesivir for COVID-19 patients which led immediate debates in social media and the press about the utility of the drug. Both preclinical and clinical data demonstrated its efficacy in COVID-19. The recently concluded ACTT-1 trial showed its efficacy in reducing the duration of hospital stay which is of utmost importance for a country like India where reduction in bed occupancy can save lives of many and eases the financial burden of patient and government. Our benefit-risk analysis of ACTT-1 trial also favored the use of remdesivir over standard of care. The SOLIDARITY trial was fundamentally different from other clinical trials on remdesivir with respect to its design, adaptive nature, and selection of endpoints. Moreover, the success of antiviral therapy also depends on the timing of initiation and combination with other drugs. Hence we believe that drugs like Remdesivir are very important for countries like India where soft end points such as time to recovery and clinical improvement and early discharge become extremely significant during a pandemic.

The emerging role of mycophenolate mofetil in interstitial lung diseases.

INTRODUCTION: Mycophenolate mofetil (MMF), initially approved to prevent rejection in solid organ allograft, is now being increasingly used for other conditions. Over the last decade, MMF has emerged as a useful therapy for a variety of immune-mediated diseases. AREAS COVERED: There has been a growing interest in the clinical use of MMF in the treatment of ILDs due to its versatile anti-inflammatory, immunomodulatory, anti-fibrotic and anti-proliferative properties. In this focussed review, we summarize the available literature using the Pubmed, Science Direct and EMBASE databases published until June 2021 on the efficacy and tolerability of MMF in various ILDs. EXPERT OPINION: Other than idiopathic pulmonary fibrosis (IPF) and its broader category of progressive fibrosing ILD, there have been no drugs approved by relevant regulatory agencies for the treatment of the multiple other forms of ILD. Though results are limited, immunosuppressants such as MMF have shown promise as an effective and well-tolerated steroid-sparing agent, providing hope that the limited treatment armamentarium for ILDs can be expanded.

Ease-of-use, preference, confidence, and satisfaction with Revolizer(®), a novel dry powder inhaler, in an Indian population.

CONTEXT: While prescribing an inhaler device, it is important to take into account the usability, preference, confidence, and satisfaction of the patients. AIMS: The present study assessed these parameters with Revolizer(®), a novel dry powder inhaler (DPI), in patients with obstructive airway diseases and in device-naïve healthy participants. SETTINGS AND DESIGN: In this open-label, prospective, multicentre study with 100 participants [n = 50 healthy participants, n = 45 mild asthma patients, and n = 5 mild chronic obstructive pulmonary disease (COPD) patients], all participants were instructed and trained on the use of Revolizer and then the participants subsequently demonstrated the inhalation technique at two visits. MATERIALS AND METHODS: The average time required to execute three correct consecutive attempts and the number of errors (including critical errors) were recorded. Participants were asked about the ease of use, preference, confidence, and satisfaction by means of a questionnaire at each visit. RESULTS: The average time required by the participants to achieve three correct consecutive attempts at visit 1 was 3.75 ± 2.10 min, which significantly reduced at visit 2 (3.07 ± 1.32 min, P < 0.01). The number of errors decreased from visit 1 to visit 2. More than 85% participants found the Revolizer easy to use, and it was preferred by more than 75% participants. Revolizer scored high on the confidence and satisfaction of all participants at both visits. CONCLUSIONS: Revolizer is an easy-to-use and a preferred device in patients with mild asthma and COPD, as well as in healthy participants with no previous experience of using inhalation devices. The participants felt confident and satisfied using the Revolizer.