Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive devastating lung disease with substantial morbidity. It is associated with cough, dyspnea and impaired quality of life. If left untreated, IPF has a median survival of 3 years. IPF affects ∼3 million people worldwide, with increasing incidence in older patients. The current concept of pathogenesis is that pulmonary fibrosis results from repetitive injury to the lung epithelium, with fibroblast accumulation, myofibroblast activation, and deposition of matrix. These injuries, in combination with innate and adaptive immune responses, dysregulated wound repair and fibroblast dysfunction, lead to recurring tissue remodeling and self-perpetuating fibrosis as seen in IPF. The diagnostic approach includes the exclusion of other interstitial lung diseases or underlying conditions and depends on a multidisciplinary team-based discussion combining radiological and clinical features and well as in some cases histology. In the last decade, considerable progress has been made in the understanding of IPF clinical management, with the availability of two drugs, pirfenidone and nintedanib, that decrease pulmonary lung function decline. However, current IPF therapies only slow disease progression and prognosis remains poor. Fortunately, there are multiple clinical trials ongoing with potential new therapies targeting different disease pathways. This review provides an overview of IPF epidemiology, current insights in pathophysiology, diagnostic and therapeutic management approaches. Finally, a detailed description of current and evolving therapeutic approaches is also provided.</p

Respiratory mechanics in patients with COPD on ventilatory support

Chronic Obstructive Pulmonary Oisease (COPO) is a major cause of morbidity and mortality throughout the world. Approximately 6 % of deaths in men and 4 % of deaths in women are due to COP01. COPO currently ranks as number six in the global impact of disease scale and is expected to rise to number three by the year 20202. In the Netherlands it already is the third cause of death3. The major cause of COPO is cigarette smoking. The percentage of smokers in the Netherlands is among the highest in Europe4 . COPO is defined physiologically as chronic airflow obstruction and may be due to a mixture of emphysema and peripheral airway obstruction from chronic obstructive bronchitis. Emphysema is a pathological diagnosis characterised by destruction of alveolar walls resulting in abnormal and permanent enlargement of airspaces and loss of lung elasticity, with consequent obstruction of peripheral airways1. Chronic obstructive bronchitis is due to obstruction of peripheral airways as a result of an inflammatory response 1. The population of patients studied in this thesis consists of patients in whom loss of elasticity of lung tissue is assumed to be present. However, this assumption is based on clinical and lung function findings, since destruction of elastic tissue is difficult to demo

Role of the internet of medical things in care for patients with interstitial lung disease

PURPOSE OF REVIEW: Online technologies play an increasing role in facilitating care for patients with interstitial lung disease (ILD). In this review, we will give an overview of different applications of the internet of medical things (IoMT) for patients with ILD. RECENT FINDINGS: Various applications of the IoMT, including teleconsultations, virtual MDTs, digital information, and online peer support, are now used in daily care of patients with ILD. Several studies showed that other IoMT applications, such as online home monitoring and telerehabilitation, seem feasible and reliable, but widespread implementation in clinical practice is lacking. The use of artificial intelligence algorithms and online data clouds in ILD is still in its infancy, but has the potential to improve remote, outpatient clinic, and in-hospital care processes. Further studies in large real-world cohorts to confirm and clinically validate results from previous studies are needed. SUMMARY: We believe that in the near future innovative technologies, facilitated by the IoMT, will further enhance individually targeted treatment for patients with ILD by interlinking and combining data from various sources.</p

Exhaled breath analysis in interstitial lung disease

PURPOSE OF REVIEW: There is a need for better noninvasive tools to diagnose interstitial lung disease (ILD) and predict disease course. Volatile organic compounds present in exhaled breath contain valuable information on a person's health and may be a novel biomarker in ILD. In this review, we will give an overview of the basic principles of breath analysis, summarize the available evidence in ILD, and discuss future perspectives. RECENT FINDINGS: An increasing number of studies on exhaled breath analysis were performed over the last decade in patients with ILD, using two methods for exhaled breath analysis: gas chromatography-mass spectrometry and electronic nose technology. Most studies showed high accuracy for diagnosis of ILD, but study design and methods widely varied. Studies investigating the potential of electronic nose technology to predict treatment response and disease behavior are ongoing. SUMMARY: The majority of studies using exhaled breath analysis in ILD show promising results for diagnostic purposes, but validation studies are lacking. Larger prospective longitudinal studies using standardized methods are needed to collect the evidence required for developing an approved diagnostic medical test.</p

“It Is a Moving Process”:Understanding the Evolution of Explainability Needs of Clinicians in Pulmonary Medicine

Clinicians increasingly pay attention to Artificial Intelligence (AI) to improve the quality and timeliness of their services. There are converging opinions on the need for Explainable AI (XAI) in healthcare. However, prior work considers explanations as stationary entities with no account for the temporal dynamics of patient care. In this work, we involve 16 Idiopathic Pulmonary Fibrosis (IPF) clinicians from a European university medical centre and investigate their evolving uses and purposes for explainability throughout patient care. By applying a patient journey map for IPF, we elucidate clinicians' informational needs, how human agency and patient-specific conditions can influence the interaction with XAI systems, and the content, delivery, and relevance of explanations over time. We discuss implications for integrating XAI in clinical contexts and more broadly how explainability is defined and evaluated. Furthermore, we reflect on the role of medical education in addressing epistemic challenges related to AI literacy.</p

“It Is a Moving Process”:Understanding the Evolution of Explainability Needs of Clinicians in Pulmonary Medicine

Clinicians increasingly pay attention to Artificial Intelligence (AI) to improve the quality and timeliness of their services. There are converging opinions on the need for Explainable AI (XAI) in healthcare. However, prior work considers explanations as stationary entities with no account for the temporal dynamics of patient care. In this work, we involve 16 Idiopathic Pulmonary Fibrosis (IPF) clinicians from a European university medical centre and investigate their evolving uses and purposes for explainability throughout patient care. By applying a patient journey map for IPF, we elucidate clinicians' informational needs, how human agency and patient-specific conditions can influence the interaction with XAI systems, and the content, delivery, and relevance of explanations over time. We discuss implications for integrating XAI in clinical contexts and more broadly how explainability is defined and evaluated. Furthermore, we reflect on the role of medical education in addressing epistemic challenges related to AI literacy.</p

Quality of life in sarcoidosis

Having sarcoidosis often has a major impact on quality of life of patients and their families. Improving quality of life is prioritized as most important treatment aim by many patients with sarcoidosis, but current evidence and treatment options are limited. In this narrative review, we describe the impact of sarcoidosis on various aspects of daily life, evaluate determinants of health-related quality of life (HRQoL), and provide an overview of the different patient-reported outcome measures to assess HRQoL in sarcoidosis. Moreover, we review the current evidence for pharmacological and non-pharmacological interventions to improve quality of life for people with sarcoidosis.</p