ERS 2022:Comorbidities and Symptoms in Interstitial Lung Diseases: Think Out of the Box

The panel of experts discussed comorbidities, predictors, and symptoms of interstitial lung diseases which can complicate care using clinical data trials.

Chronic cough affects up to 87% of patients with fibrosing interstitial lung disease (ILD), is frequently refractory to treatment, may predict disease progression and significantly impacts the quality of life. Few studies have examined the triggers and impact of cough in patients with ILD. Patients with ILD and persistent cough were asked to complete a structured questionnaire about the triggers and impact of cough. The profile of cough triggers and abnormal laryngeal sensation suggests the hypersensitivity of the cough reflex in many ILD patients. Cough has a wide range of adverse impacts, the most common of which are stress incontinence and social embarrassment.

Weight loss has been related to worse outcomes in IPF patients. Therefore, the study analyzed associations between weight change and outcomes in patients with progressive fibrosing ILDs. In the INBUILD trial, weight loss significantly impacted the risk of acute ILD exacerbation or death; however, it did not impact the risk of ILD progression or death.

Patients with ILDs often have comorbidities that complicate their care and may lower treatment adherence. Therefore, the study aimed to examine the efficacy and safety of nintedanib in patients with progressive fibrosing ILDs (other than IPF) in subgroups based on comorbidity burden. In the INBUILD trial, there was no heterogeneity in the effect of nintedanib on lowering the rate of forced vital capacity (FVC) decline between subgroups based on comorbidity burden.

Clinic consultations frequently fail to capture patients' broader perspectives on their disease. The study aimed to understand better patients' perceptions of their disease to develop meaningful outcome measures. ILD has a significant impact on both physical and emotional well-being. However, patients have a limited understanding of their disease, including treatment options. Additional inputs from support groups, specialist ILD nurses and engagement with pulmonary rehabilitation may improve patient care.

Vaccination provides the most effective influenza protection. Patients with ILD are at high-risk for influenza complications. The study aimed to compare vaccinated versus unvaccinated ILD patients in terms of all-cause mortality and all-cause and influenza-related hospitalizations. Promising results were observed regarding improved all-cause mortality in half of the observed seasons.

Leading health organizations have identified psychological and physical Health-Related Quality of Life (HRQoL) in Interstitial Lung Disease (ILD) as an essential patient-centred outcome. This led to a review to determine how the symptom burden affects the day-to-day life of the patient group. Most patients attending ILD clinics have a significant burden of psychological and physical symptoms that affect their HRQoL. Unfortunately, traditional care models do not address these issues. To bridge this gap, psychological support delivered by experts at the time of diagnosis can be offered to these patients. To address the "care gap" holistic care models must be developed for patients attending ILD clinics.

A study aimed to investigate the link between ILD and oesophageal function by high-resolution oesophageal manometry (HROM). The study demonstrated that 72% of ILD patients investigated with HROM had some degree of oesophageal dysmotility, with a lower prevalence of GORD. The findings may indicate that oesophageal motility disorders, rather than acid reflux, should be studied further for their role in the development and/or progression of ILD.

In chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) candidates for lung transplantation (LTx), the effect of oesophagal pressure (PES) as a surrogate of intrathoracic pressure on the assessment of the central pulmonary pressure (CPP) during the right heart catheterization (RHC) at rest was investigated. In COPD and ILD candidates for LTx, intravascular CPP measured at end-expiration overestimates mean respiratory cycle pressures. Therefore, averaging intravascular measurements over the respiratory cycle would provide accurate results in COPD patients, but it could underestimate transmural values in ILD patients.

In idiopathic pulmonary fibrosis (IPF), a high prevalence of obstructive sleep apnea has been reported. However, the clinical features of sleep-related breathing disorders (SRDB) and their impact on the disease have not been thoroughly studied. A prospective study showed that IPF patients have a high prevalence of obstructive, non-obstructive apneas and persistent nocturnal hypoxemia, despite the lack of symptomatology. These findings may have biological and quality-of-life implications.

In patients with idiopathic pulmonary fibrosis, surgical treatment of lung cancer is associated with a high rate of postoperative pulmonary complications (PPCs), which may be fatal. A study was conducted to determine the prevalence and risk factors of PPCs and to develop a novel scoring system to predict them. The study concluded that PPCs were common in IPF patients who received curative resection for NSCLC. Predictors of PPC development included advanced age, diabetes, a high American Society of Anesthesiology (ASA) score, a low carbon monoxide diffusing capacity (DLco), and the extent of surgery.

Given the poor prognosis and the anticipated rise in the incidence of IPF worldwide, symptom relief should be at the core of management. The study was conducted to explore nurses' experiences in delivering palliative care in IPF. The study showed that advanced care planning and earlier implementation of palliative care will help nurses care for IPF patients.

Hypoxemia, a smoking history, advanced age, and decreased lung function contribute to the cognitive deficit in IPF patients. The purpose of this study was to examine neurocognitive functions in IPF patients. The majority of IPF patients had mild cognitive impairment. Cognitive impairment can result in a lower quality of life and difficulties understanding and remembering certain concepts about a patient's illness and/or treatment strategy.

Although the factors that influence 6-minute walking distance (6MWD) in idiopathic pulmonary fibrosis (IPF) are well established, they have not been studied in idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, the study investigated factors that cause a decrease in the 6-minute walk distance per cent predicted (%6MWD), including the disease entity IPF and IPPFE. The results of the study suggest that leanness, the severity of illness and shortness of breath are essential factors in determining exercise tolerance regardless of the disease entity.

Patients with IPF are treated with antifibrotic. Antifibrotics are safe; however, they have adverse effects and do not reduce dyspnea or improve quality of life. Therefore, a study was conducted with the objective of discussing the perceptions of individuals with IPF about antifibrotic treatment. The results obtained were constructed into four thematic categories: the will to live, perceptions about improvement, delayed progression or worsening of the clinical condition resulting from the treatment, gratitude for the care and treatment and perceptions about side effects of the treatment and their repercussions on daily life. The will to live appears to motivate patients to use antifibrotic medications even when no cure or change in clinical status is promised.

It is known that IPF impacts a patient's quality of life. Therefore, a psychological assessment and intervention were initiated for IPF patients and caregivers. As a result, anxiety and depression were confirmed as common findings in IPF patients.

The coexistence of emphysema and usual interstitial pneumonia (UIP) on CT is becoming more common. It is unknown whether combined pulmonary fibrosis and emphysema syndrome (CPFE) is a distinct clinical entity with an impact on survival. A study was conducted to compare the survival rates of patients with IPF, CPFE, and emphysema. The study concluded that co-existent emphysema and fibrosis are linked with worse survival rates than emphysema alone. CPFE has a survival trajectory comparable to IPF.

Loss of muscle mass (LOM) appears to be associated with poor prognosis in chronic illnesses but its significance in IPF is unknown. Thus, the authors aimed to investigate the role of muscle loss in patients with IPF receiving antifibrotic treatment. LOM is common in IPF patients at the time of diagnosis. Furthermore, male patients with muscle loss had a lower two-year survival rate. Low High Gray Level Run Emphasis at baseline and oxygen requirement at rest are independent predictors of mortality.

The relationship between statin uses and the incidence of ILD is still being debated. However, a population-based longitudinal cohort study concluded that Statin use might be associated with a lower incidence of ILD and the development of IPF.

Worldwide, ILD patients struggle to consult different physicians, receive an incorrect diagnosis, and consequently have unappropriated treatment due to a complex and multidisciplinary approach to reach an accurate diagnosis. In the United Kingdom, some patients may have symptoms more than 5 years prior to diagnosis. In Brazil, there is still a lack of data showing the patient's journey, even though a public consultation on systemic sclerosis with interstitial lung disease (SSc-ILD) treatment protocol held in April 2022 indicates that Brazilian patients face similar issues seen globally. Patients took an average of three years to receive the correct diagnosis of IPF or SSc-ILD. The average time for PF-ILD was four years. In addition, 62% of patients experienced worsening up to two years after the onset of symptoms, and 36% experienced worsening in less than a year. Identifying the barriers to early ILD diagnosis and strategies to overcome them is critical to benefit the patient, improve patient outcomes and quality of life, and increase survival.

European Respiratory Society (ERS) International Congress 2022, 3rd-6th Sept. 2022, Barcelona