Non-pharmacological Management of Fibrosing Interstitial Lung Diseases
Article information
Abstract
Non-pharmacological interventions are integral to the multidisciplinary management of fibrosing interstitial lung diseases (ILDs), complementing pharmacological therapies by addressing functional decline, symptom burden, and quality of life. Palliative care is fundamental, offering a structured approach to symptom control—particularly dyspnea— and facilitating advance care planning. Oxygen therapy may provide symptomatic benefit in patients with resting, nocturnal, or exertional hypoxemia, though evidence remains limited and extrapolated mainly from idiopathic pulmonary fibrosis cohorts. Pulmonary rehabilitation, encompassing supervised exercise and patient education, has demonstrated short-term improvements in exercise tolerance; however, long-term effects on dyspnea and health-related quality of life are inconsistent. Nutritional status is an emerging area of interest, with data linking low body mass index and unintentional weight loss, underscoring the potential value of dietary assessment and intervention. Psychological support is critical, as ILD patients frequently experience anxiety, depression, and psychological distress. Although peer-led interventions remain under-investigated, they appear to address significant unmet needs in patient education and emotional support. Occupational therapy provides tailored strategies to maintain functional independence and manage fatigue, thereby improving daily living. Immunization against influenza, pneumococcus, and severe acute respiratory syndrome coronavirus- 2 (SARS-CoV-2) is essential, given the elevated risk of infection-related morbidity and mortality. For selected patients with progressive disease refractory to medical therapy, lung transplantation offers a potential survival advantage, necessitating timely referral and evaluation. Although results are promising, evidence is still limited for some interventions and further research is warranted to establish robust, evidence-based guidelines for non-pharmacological management in fibrosing ILD.
Introduction
Interstitial lung disease (ILD) is an umbrella term to describe a number of diseases affecting the lung parenchyma. Their aetiology is yet to be fully understood, but it involves both inflammatory and fibrotic processes [1]. Fibrotic ILDs may develop a progressive phenotype, characterized by self-perpetuating fibrosis and decline in lung function. Structural damage to the lungs leads to progressive and relentless respiratory and systemic manifestations, including dyspnea, cough, loss of weight, loss of independence, and eventually death [2]. Patients with ILD report that these diseases have a profound impact on their lives, leading not only to deteriorating mood but also to significant effects on their relatives and caregivers.
While some pharmacological treatments have been approved and used over the last 10 years, such as immunomodulators or antifibrotics, for most of these conditions, current pharmacological therapies have not been shown to improve short-term survival or quality of life. Therefore, it is essential to focus on comprehensive, patient-centred care to enhance overall clinical outcomes. While the search for a cure is still a key priority for stakeholders, it has been proven that similarly important are symptom management and improved quality of life [3,4]. Supplemental oxygen therapy, pulmonary rehabilitation (PR), and palliative care have been shown to reduce symptom burden and improve daily functioning [5-7].
In this review, we will examine non-pharmacological treatment options (Figure 1) that should be considered as part of a holistic approach for ILD patients. Several groups of healthcare professionals are involved beside the medical team, to ensure a 360° support (Figure 2).
Non-pharmacological management for fibrotic interstitial lung diseases (ILDs).
Multidisciplinary team roles in non-pharmacological management for fibrotic interstitial lung diseases.
Certain non-pharmacological interventions, including PR, long-term oxygen therapy (LTOT), and palliative care, are supported by a robust body of evidence demonstrating their benefit. For others—such as dietary interventions, occupational therapy, and psychological support—the evidence remains limited, underscoring the need for further research. Most of these interventions proposed are relatively low-cost in comparison to their potential benefits. However, due to the absence of large, dedicated cohort studies, their overall effectiveness and cost-efficiency cannot be thoroughly evaluated. It should also be acknowledged that the available evidence for fibrosing ILD remains limited and is largely extrapolated from studies in patients with idiopathic pulmonary fibrosis (IPF).
Non-pharmacological management strategies can be broadly categorized as follows: (1) palliative care; (2) oxygen therapy; (3) PR; (4) nutrition; (5) psychological support; (6) occupational therapy; (7) lung transplantation; and (8) vaccination.
Palliative Care
Palliative care is an approach aimed at improving the quality of life of patients and their families who face challenges associated with life-threatening illnesses, encompassing physical, psychological, social, and spiritual aspects, as defined by the World Health Organization (WHO)8. The 2023 European Respiratory Society (ERS) Clinical Practice Guideline of palliative care for chronic obstructive pulmonary disease (COPD) or ILD defines palliative care as ‘A holistic and multidisciplinary, person-centred approach aiming to control symptoms and improve quality of life of people with serious health-related suffering because of ILD, and to support their informal caregivers’ [7]. By this definition, palliative care is required not only for patients approaching the end-of-life but also for their caregivers. Palliative care is especially important as ILD patients carry a significant symptoms burden [9]. Timely identification of patients at risk of deteriorating or rapidly progressing is important for an effective referral as they will have higher mortality and higher healthcare resource utilization [10,11].
1. Barriers of palliative care access and time of referral
Unfortunately, notwithstanding strong evidence, the process often remains suboptimal, with referrals often delayed until later stages of the disease [12-14]. There are various barriers to palliative care referral for patients with ILD. Barriers on the disease side included difficulty in predicting the prognosis and outcomes of ILD patients, the absence of established treatments for ILD symptoms, and a lack of tools to assess disease burden and determine the optimal timing for palliative care referral. In a survey of more than 100 physicians participated, over 60% of participants report the hesitation due to the unpredicted prognosis more than 50% of them experience of lack for multidisciplinary staff settings. 60% also found difficulty to spend time for discussion for palliative in a busy clinic [15]. On the healthcare system side, there is sometimes a lack of palliative care services, especially among community healthcare providers. On the patient or caregiver side, there may be a lack of knowledge or understanding about the prognosis of ILD and a misconception of palliative care role as only for dying patients [16-18]. Due to these barriers (Table 1), referrals to palliative services are often delayed, with up to 70% of IPF patients being referred within 6 months of death. Furthermore, the referral rate for ILD patients to palliative care ranges from 0% to 38% [13,17]. The guideline recommends considering a palliative care referral when ILD patients and their informal caregivers have physical, psychological, social or spiritual/existential unmet needs. Disease-based factors such as oxygen use, hospitalization and/or health service utilization may act as surrogate markers to help identify those likely to have unmet needs [7].
Barriers to palliative care service for ILD patients
2. Symptoms management
Non-pharmacological treatments mentioned in this review, like oxygen and PR, are essential to palliative care, besides symptom assessment and management. Medications to relieve symptoms are an important component of palliative care. Cough is one of the most troubling symptoms in patients with ILD, adversely affecting quality of life and prognosis [19,20]. The importance of cough control is now recognized. Recent randomized phase 2 studies have evaluated morphine and opioid agonist–antagonists Nalbuphine for cough control. Oral morphine reduced awake cough by about 40% and halved cough frequency. Nalbuphine reduced daytime objective cough frequency by 52.5% and 24-hour cough frequency by 50.8% compared with placebo treatment period. Both agents were associated with increased adverse effects, most commonly nausea, fatigue, constipation, and dizziness; however, these side effects were generally tolerated and only rarely led to treatment discontinuation in the trials [21,22]. Despite benefits for cough, oral morphine did not improve dyspnea related to fibrotic lung disease and was associated with increased constipation, nausea, and confusion [23]. One of the major adverse effects of morphine and other opioid medications is constipation [24]. In patients with end-stage lung disease—particularly those with pulmonary hypertension—constipation and the associated straining during the Valsalva manoeuvre can precipitate significant hemodynamic instability, cardiovascular complications, or even pneumothorax [25-27]. Therefore, the routine co-prescription of laxatives, such as stimulant agents (e.g., senna) and osmotic agents (e.g., lactulose), is recommended to prevent and manage opioid-induced constipation in this population [24]. Furthermore, non-pharmacological interventions, including the use of handheld or table fans to increase airflow and specific breathing techniques (such as pursed-lip or diaphragmatic breathing), have been demonstrated to alleviate dyspnea and improve quality of life [28,29]. A recent randomized controlled trial (RCT) of singing therapy demonstrated that online group singing interventions improved quality of life (assessed via Short Form-36 [SF-36]) in patients with COPD or ILD suffering from chronic breathlessness and reduced quality of life [30].
3. Psychological support to patient and their caregivers
There is a substantial and ongoing need for psychological support in patients with ILD and their caregivers. In one review, the prevalence of depression and anxiety in ILD reached approximately 57% and 61% in ILD patients, respectively, with demonstrable adverse effects on health-related quality of life [31]. This psychological burden affects not only patients but also informal caregivers, who frequently report high levels of distress and reduced well-being. Despite these extensive needs, only a minority of patients—around one-fifth in some reports—access professional psychological support, and up to 80% report that available support is inadequate [31-33]. Supportive care needs extend beyond emotional or psychological concerns and encompass physical symptoms, family-related issues (such as increasing caregiver burden and changing family roles), social difficulties (including social withdrawal), and challenges with activities of daily living (for example, managing household tasks and transportation). Comprehensive psychosocial support for patients and their informal caregivers is therefore essential to address anxiety, mood disturbance and spiritual distress [34,35]. RCT indicate that interventions such as community case conferences, structured disease-management programmes and nurse-led palliative care models can alleviate palliative symptoms and improve quality of life for both patients with fibrotic ILD and their caregivers [36-38]. The details of the psychological support provided will be described in greater depth in a later section of this article
4. Advance care planning and end-of-life care
Advanced care planning (ACP) and end-of-life care are other important parts of comprehensive palliative care. The definition suggested by the European Association for Palliative Care of ACP is a process to enable individuals to define goals and preferences for future medical treatment and care, to discuss these goals and preferences with family and healthcare providers, and to record and review these preferences if appropriate, so that their preference can be taken into account when they are unable to make their own decisions [39]. The goal of the discussion is to avoid futile interventions or other measures that do not match with patients’ values and preferences. Sometimes, due to the ‘unpredictability’ of the prognosis, discussions about sensitive issues related to end-of-life prognosis can be challenging, and different patients and caregivers may be more or less open to discussing these topics [18]. But earlier discussions with ACP showed it can improve communication, better understanding of the disease and hence reduce the uncertainty, improve future end-of-life management, and also future disease treatment decisions [7,35,40]. A retrospective study showed that ACP and palliative care bundle lead to more patients died at home or hospice (96% vs. 83%, p=0.02), more use of opioids (88% vs. 60%, p=0.002) and more caregiver engagement (adjusted odd ratio, 2.38) [41]. Another retrospective study showed after implantation of palliative care led to less unplanned visit and favour at home or in hospice death [42].
The ERS clinical practice guideline also emphasizes that palliative care should be integrated throughout the disease course and does not preclude the use of disease-modifying treatments, such as antifibrotics or immunosuppressants. It could be provided in different settings, like at patients’ homes, outpatient clinics or wards, and even intensive care units [7]. As the needs of palliative care are diverse, a multidisciplinary approach with different specialists, including respiratory physicians, palliative specialist, physiotherapist, psychologist to accommodate the needs of patients [7,43].
Key messages
• Palliative care should be introduced early to address physical, emotional, and spiritual needs, not just at end-of-life.
• Palliative care can be integrated with active treatment and delivered across various settings, including home and hospital.
• A multidisciplinary approach, including symptom management and ACP, improves patient and caregiver outcomes.
Oxygen Therapy
1. Resting hypoxemia
In patients with advanced ILD, resting hypoxemia is frequently observed due to diffusion defects, ventilation-perfusion mismatch, and pulmonary vascular abnormalities [44,45]. This condition reflects disease severity and impacts both quality of life and survival [46,47]. The cumulative incidence of resting hypoxemia is 16.5%, while exertional hypoxemia occurs in 40% of patients over 5-year [47]. Thus, oxygen assessment at rest and during exertion is essential for determining the need for ambulatory oxygen therapy (AOT) and LTOT. Resting hypoxemia is easily measured via pulse oximeter, whereas exertional hypoxemia requires other assessments such as the 6-minute walk test (6MWT), 1-minute sit-to-stand test, or the DeOX score.
2. Exertional hypoxemia
Patients with ILD may experience desaturation during physical activity or exercise. Exertional hypoxemia is defined as a drop in oxygen saturation (SpO2) ≤88% during 6MWT [48] and is more prevalent in those with severe disease [49]. Risk factors include disease progression, exercise limitation, reduced lung function parameters, polycythaemia, and pulmonary hypertension [50].
1. 6MWT: A standardized walking assessment used to evaluate walking capacity. Patients are instructed to walk on a flat corridor as much as could within 6 minutes [51]. Desaturation during 6MWT is associated with reduced physical activity, increased pulmonary hypertension risk, and higher mortality [48,52,53]. An official American Thoracic Society (ATS) guideline states recommends AOT for patients who desaturate to an SpO2 of ≤88% [54].
2. 1-minute sit-to-stand test: An alternative to 6MWT that requires only a chair when the 6MWT is not feasible. It has demonstrated consistency in detecting exercise-induced desaturation in patients with ILD [55,56].
3. DeOX score: A predictive tool for assessing desaturation risk during the 6MWT in fibrotic ILD when 6MWT is not feasible [57]. This score is developed from a study of 300 patients with ILD by using SpO2 and diffusing capacity of the lung for carbon monoxide (DLCO) to categorize patients into three groups: 0 (SpO2 >95% and DLCO >40%), 1 (SpO2 ≤95% or DLCO ≤40%), and 2 (SpO2 ≤95% and DLCO ≤40%). Patients with scores of 1 and 2 are significantly associated with 6MWT desaturation, with odds ratios of 8.1 (95% confidence interval [CI], 4.14 to 15.88) and 24.8 (95% CI, 11.78 to 57.04), respectively.
Oxygen supplementation aims to correct hypoxemia, improving oxygen delivery to tissues, relieve pulmonary vascular strain, and enhancing exercise tolerance in patients with ILD. According to ILD pathogenesis, fibrosis and thickening of the alveolar-capillary membrane impair gas exchange, resulting in reduced arterial oxygen tension (PaO2) and peripheral saturation (SpO2). Increasing the inspired oxygen fraction enhances diffusion, thereby improving arterial oxygen and tissue oxygenation. Furthermore, correction of chronic hypoxemia also alleviates hypoxic vasoconstriction, lowering pulmonary arterial pressures and potentially mitigating pulmonary hypertension [5,58].
3. Long-term oxygen therapy
Patients with chronic resting hypoxemia should receive LTOT, defined as oxygen supplementation for at least 15 hours per day. LTOT has been shown to alleviate breathlessness, improve oxygenation, and reduce the risk of pulmonary hypertension [5,54]. However, there is limited RCT data assessing its impact on mortality. One unpublished RCT reported no significant difference in mortality between patients receiving LTOT and those who did not [59]. Additionally, a population-based longitudinal study of patients with ILD on LTOT found no significant difference in transplant-free survival between those with moderate (PaO2 7.4 to 8.7 kPa) and severe hypoxemia (PaO2 <7.4 kPa) [60]. Due to the limited evidence regarding the effects of LTOT on mortality, dyspnea, and physical activity in patients with ILD, current recommendations are based on evidence from studies in patients with COPD and resting hypoxemia.
The indications for LTOT are [54,61]: (1) Resting hypoxemia in patients with ILD: Partial pressure of oxygen in arterial blood (PaO2) ≤55 mm Hg (7.3 kPa) or SpO2 ≤88%; (2) Patients with less severe resting hypoxemia PaO2 ≤60 mm Hg (8 kPa) or SpO2 ≤89% in the presence of hypoxic organ damage, such as pulmonary hypertension, right-sided heart failure, or polycythaemia.
4. Ambulatory oxygen therapy
AOT is defined as the use of a portable oxygen supplement during exercise or daily activities. The Ambulatory Oxygen in Fibrotic Lung Disease (FLD) (AmbOx) trial, a randomized 2-week crossover multicentre study on patients with fibrotic ILD, demonstrated that ambulatory oxygen significantly improved breathlessness and chest symptoms domains of the King’s Brief ILD Questionnaire (KBILD) in patients with exertional desaturation (resting SpO2 ≥94% on room air but SpO2 ≤88% after the 6MWT) compared to those without ambulatory. Notably, at the end of study, more than half of patients continue using ambulatory oxygen [62]. Another RCT, the Pulmonary Fibrosis Ambulatory Oxygen (PFOX) trial is underway to evaluate the efficacy of AOT in exertional hypoxemia in pulmonary fibrosis [63]. During exertion, supplemental oxygen helps maintain saturation, delays the onset of anaerobic metabolism, and improves exercise tolerance and quality of life. In summary, AOT is recommended in patients with ILD who experience exertional desaturation [54,61].
5. Nocturnal oxygen therapy
Patients with fibrotic ILD may experience nocturnal hypoxemia, defined as SpO2 <90% for ≥10% of total sleep time. The pooled prevalence of nocturnal hypoxemia is approximately 37% and may occur even without obstructive sleep apnoea (OSA), resting hypoxemia, or exertional hypoxemia [64]. Furthermore, data from small cohorts also found that nocturnal hypoxemia is related to poor clinical outcomes [65,66]. However, studies on the benefits of nocturnal oxygen supplementation remain limited. The 2015 British Thoracic Society (BTS) guideline does not recommend nocturnal oxygen therapy for patients with nocturnal hypoxemia unless they already meet the criteria for LTOT [61]. Meanwhile, the ATS guideline does not address the role of nocturnal oxygen therapy in ILD [54]. Further studies are needed to determine the potential benefit of nocturnal oxygen therapy.
6. ILD and OSA
The incidence of OSA in patients with ILD is high, with meta-analyses and cohort studies consistently reporting a prevalence of 61% to 72% [67-69]. Rates are even higher in IPF, reaching up to 76% in some studies [67,69]. Moderate-to-severe OSA affects approximately 36% to 40% of individuals with ILD [67,69]. Identified risk factors include male sex, older age, higher body mass index (BMI), reduced forced vital capacity (<80% predicted), and shorter 6-minute walk distance (<372 m) [67,70].
Continuous positive airway pressure (CPAP) is the first-line therapy for OSA in ILD. CPAP has been shown to improve sleep quality, daytime sleepiness, fatigue, and overall quality of life, particularly when adherence is good (≥4–6 hours/night) [71,72]. While some evidence suggests that CPAP may improve progression-free survival in ILD patients requiring supplemental oxygen, it has not consistently demonstrated a mortality benefit or slowed disease progression in the wider ILD population [71-73]. Despite this, routine screening and early management of OSA in ILD remain recommended due to its high prevalence and significant impact on quality of life [67,71,74].
Key messages
• Hypoxemia is common in advanced ILD and should be assessed at rest and with exertion to guide oxygen therapy.
• AOT can improve symptoms in patients with ILD who have exertional desaturation.
• LTOT is indicated for resting hypoxemia, though evidence for survival benefit remains limited.
• OSA is highly prevalent in ILD, highlighting the need to identify risk factors and screen early.
Pulmonary Rehabilitation
PR is defined as a comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies that include but are not limited to exercise training, education, and behaviour change, designed to improve the physical and psychological condition of patients with chronic respiratory problems and to promote the long-term adherence to health-enhancing behaviours [75]. Patients with ILD often have reduced exercise capacity and shortness of breath during exercise, causing deterioration in the quality of life and poor survival [76-78]. The mechanism of reduced exercise capacity is multifactorial, involving impaired diffusion capacity, ventilation-perfusion inequality, and muscle dysfunction. Sarcopenia is not uncommon in patients with ILD, affecting 30% to 40% of individuals and being associated with poor clinical outcomes [79]. Some patients may have difficulty taking part in scheduled PR. Those with significant mobility problems, such as a joint pain or other physical limitations, maybe unable to manage the required exercises or attend session regularly. Recognizing these challenges highlights the need for flexible or adapted rehabilitation options that better suit each patients’ abilities.
1. Components of PR
Exercise training is an essential part of PR. The training should include both endurance training and resistance training. The endurance (aerobic) training is targeted to improve the aerobic exercise capacity, increase endurance, and hence improve daily function, reduce breathlessness and fatigue, and, ultimately, increase physical activity. Resistance training aims to improve local muscle strength and endurance [80]. Breathing exercises such as diaphragmatic breathing, inspiratory muscle training, and chest expansion exercises serve as direct training for the respiratory muscles [81]. These interventions have been shown also improve lung function and enhance exercise capacity [80,82]. Other components, including education, nutrition and psychological support, should not be omitted [80]. Exercise prescription of ILD may be different with more exertional dyspnea and desaturation. Oxygen supplements during exercise help to relieve dyspnea and improve exercise capacity. It should be considered with patients who have desaturation on exertion [62].
2. Benefit of PR
A systematic review of more than 21 studies showed that PR can be performed safely in patients with ILD [6]. The systematic review found that PR improved the 6MWD by 40 m and improved the maximum exercise capacity. Besides the exercise performance, dyspnea and quality of life improved in terms of the Chronic Respiratory Disease Questionnaire (CRQ) and the St. George’s Respiratory Questionnaire (SGRQ). Furthermore, the effect of PR is sustained for 6 to 12 months. ILD patients undergoing PR can achieve a 6MWD of 32 m more and report less dyspnea and a better quality of life. The effect of PR on survival at long‐term follow-up is uncertain, but there are also no side effects of PR that have been identified in all studies [6]. A recent meta-analysis showed that the component of the PR programme should be more than 8 weeks, fully supervised and including high-intensity interval training (HIIT) had a better clinical impact on the exercise capacity and quality of life [82]. Based on consistent evidence supporting the benefits of PR, and the absence of adverse events—including cardiovascular complications, musculoskeletal injuries, or death—reported across 10 RCTs during training, the ATS Clinical Practice Guideline on PR in adults with chronic respiratory disease therefore strongly recommends PR for patients with ILD, supported by moderate-quality evidence [83].
Based on consistent evidence supporting the benefits of PR, and the absence of adverse events—including cardiovascular complications, musculoskeletal injuries, or death—reported across 10 RCTs during training, the ATS Clinical Practice Guideline for PR in adults with chronic respiratory diseases therefore strongly recommends PR for patients with ILD, supported by moderate-quality evidence [83].
3. Location of PR
The traditional approach is hospital and clinic-based rehabilitation. Nevertheless, with recent technological breakthroughs, home-based or telerehabilitation programs have become more popular. This may help tackle patient-related barriers to PR, like transportation and caring responsibilities. A Cochrane review showed telerehabilitation had similar efficacy on the exercise capacity and quality of health compared to in-person rehabilitation in patients with chronic respiratory disease. Moreover, the completion rate of telerehabilitation is 93% compared to a 70% completion rate for in‐person rehabilitation, which is much higher [84]. Even though in the study, 99% of patients had COPD instead of ILD. The result showed the great potential of telerehabilitation in chronic respiratory disease. The evidence of telerehabilitation on ILD is limited, but some studies have shown that it can improve exercise capacity, respiratory function and shortness of breath through remote rehabilitation [85-87].
4. Duration and maintenance of PR
In most studies, rehabilitation for ILD patients usually lasts 6 to 12 weeks. Maintenance sessions of PR has limited evidence on ILD but more on COPD. The evidence, however, is not strong enough for ATS guidelines to recommend either supervised maintenance PR after the initial programme [83]. The study showed that an at-home rehabilitation programme for 40 weeks after the initial 12 weeks of rehabilitation showed no improvement in 6MWD. Other studies showed that a 6-month rehabilitation programme improved 6MWD, exercise capacity and muscle force [88], and improved endurance time [89]. To date, there is no supporting data on whether repeating PR is helpful. Although the optimal mode and program of PR remain unknown, it should not be omitted in the management of patients with ILD.
Key messages
• PR improves exercise capacity, breathlessness, and quality of life in ILD.
• Effective PR includes endurance and resistance training, HIIT, education, psychological support, and oxygen supplementation when needed.
• Telerehabilitation is a promising alternative to centre-based PR, though evidence in ILD is still emerging.
Nutrition
Research into nutrition within ILD remains limited. Malnutrition is defined as a state of nutrition in which a deficiency or excess of energy, protein and other nutrients causes measurable adverse effects on tissue/body form (body shape, size, and composition) and function and clinical outcome [90]. This can be broken into two categories: undernutrition and overnutrition.
1. Undernutrition
The prevalence of undernutrition for patients with ILD ranges between 9% and 55% [91,92]. The variability of this data is secondary to a consensus on specific nutritional screening tools to use for patients with ILD. This means that many of the definitions of undernutrition vary between cut-offs for BMI, percentage weight loss, oral intake and biochemical markers.
Research suggests that as little as 5% unintentional weight loss is a strong predictor for reduced survival [93,94]. Additionally, patients who are underweight, classified as a BMI <18.5 kg/m2, also have an increased risk of mortality [95]. Underweight is also a contraindication for lung transplant due to the mortality risk. One study in patients with IPF showed an 11% lower mortality risk with every one unit increase in BMI [96].
Patients with ILD who experience reduced appetite should be provided with tailored dietary strategies to support adequate nutritional intake. It is recommended that they consume small, frequent meals—ideally six to seven per day—as large portions may be difficult to tolerate. When main meals cannot be completed, high-calorie or protein-rich snacks and oral nutritional supplements should be incorporated to compensate. Establishing consistent mealtimes, spaced approximately 2 or 3 hours apart, can help regulate intake. Additionally, keeping energy-dense snacks and drinks readily accessible may encourage more frequent consumption. Patients should be encouraged to take advantage of periods during the day when their appetite is better, increasing intake during those times to help meet nutritional needs [97].
Patients who have been prescribed oral nutritional supplements should adhere to guidance provided by their dietitian or other healthcare professionals. These supplements are specifically formulated to provide additional energy, protein, and micronutrients when dietary intake is insufficient to meet nutritional requirements. Importantly, oral nutritional supplements are intended to complement, not replace, regular meals and snacks. Supplements may be consumed after meals or incorporated into foods and beverages, particularly if taken alone, as they are not well tolerated. It is generally advised to avoid consuming oral nutritional supplements immediately prior to meals, as they may induce satiety and reduce subsequent food intake [98,99]. If the prescribed flavours or formats become unpalatable, patients should be encouraged to consult their healthcare provider to explore alternative options better suited to their preferences and tolerance [97].
2. Overnutrition
Obesity defined as a BMI >30 kg/m2 or >27.5 kg/m2 for those from an Asian, Chinese, Middle Eastern, Black African or African-Caribbean background, has been associated with reduced lung capacity due to the additional weight on the thorax, poorer lung function, shortness of breath and reduced quality of life [100]. Also, obesity is a contraindication for lung transplant, as it is an independent risk factor for mortality after lung transplantation [101]. However, for those not being considered for transplant, there is some conflicting evidence where some studies have found that obesity may have a protective effect on morbidity and mortality compared with normal or underweight patients [95,102]. This may be indicative of the obesity paradox being relevant to patients with ILD, and it is often seen in other respiratory conditions such as COPD and lung cancer [103].
3. Body composition
BMI is overall a blunt measurement. It is clear that anthropometric data alone do not provide a complete nutritional picture. Body composition refers to relative proportions of lean body mass and body fat mass with a body [104]. This is important as we know that fat-free mass (FFM) is associated with poorer outcomes in ILD. Body composition is increasingly recognized as important in ILD because differentiating between fat and FFM can help diagnose sarcopenia, which is characterized by low muscle mass, reduced strength, and impaired physical performance [105]. One study looking at FFM in IPF patients showed that every 1 unit increase in FFM measured by bioelectrical impedance analysis (BIA) was associated with a decreased risk of death by 36% [106]. An increasing body of research suggests that FFM may independently improve (or influence) survival outcomes in patients with ILD [92].
Nowadays, there are a variety of different methods available to assess body composition, including dual-energy X-ray absorptiometry, computed tomography (CT) scan, magnetic resonance imaging (MRI), air displacement plethysmography, positron emission tomography combined with CT or PET/MRI, quantitative magnetic resonance and BIA [107]. More hospitals are beginning to invest in BIA machines; however, a downside to measuring body composition is cost of the machinery/software.
Key messages
Nutritional care for patients with ILD is often limited despite the increasing evidence of need. Ideally, all hospitals should aim to:
• Screen patients with ILD for undernutrition using a validated malnutrition screening tool, and refer them to a registered dietitian for individualized nutritional support and advice.
• Whenever possible, measure and monitor body composition, particularly FFM.
• For patients who may require weight management support, seek multidisciplinary guidance from a registered dietitian and physiotherapist to prevent loss of muscle mass.
Psychological Support
Depression and anxiety are common among patients, with reported prevalence ranging from 14% to 49% for depression and 21% to 60% for anxiety, both of which contribute substantially to a decline in quality of life [31]. Patients and their caregivers frequently report experiences of anger, frustration, and a perceived loss of control. Psychological distress is often exacerbated by fears about disease progression and uncertainty regarding the future, including concerns about potential loss of employment or income. Furthermore, the loss of independence and the perception of becoming a burden to family members are common sources of emotional distress, with many patients expressing guilt over their inability to maintain usual social and familial roles. Importantly, approximately 80% of patients indicate a need for psychological support, while 23% report that such support is inadequate or absent within the current care framework [35,108]. In this context, mental health and psychological support play a crucial role in the comprehensive care of patients with ILD. A pilot study evaluated the feasibility and effectiveness of a five-session online group intervention for patients with ILD drawn from a psychology waiting list. The intervention integrated elements of cognitive behavioural therapy (CBT), acceptance and commitment therapy, and compassion-focused therapy, and included 10 participants [109]. Outcome measures, including the KBILD, EuroQol 5 Dimension 5 Level (EQ-5D-5L), and hospital anxiety and depression scale (HADS), were collected electronically at baseline, post-intervention, and 3-month follow-up. The study found significant improvements in EQ5D activity and anxiety/depression scores. Additionally, changes in visual analogue scale (VAS) health ratings, KBILD total scores, and KBILD psychological domains exceeded the minimum clinically important difference. VAS health ratings continued to improve at the 3-month follow-up, although statistical analysis at this time point was not feasible due to the small sample size [109].
A recent RCT evaluated the effectiveness of Almee, a personalised digital CBT programme delivered via smartphone or tablet, in reducing anxiety and improving quality of life in individuals with ILD [110]. A total of 108 participants were randomized equally into intervention and control groups, with the intervention group completing a 9-week digital therapy programme. The primary endpoint was a reduction in anxiety, measured by the Generalised Anxiety Disorder-7 (GAD-7) scale. Secondary endpoints included changes in quality of life as assessed by the KBILD questionnaire, specifically its psychological, breathlessness/activity, and chest symptom domains. This trial represents an important step in evaluating the potential of digital CBT interventions to address the psychological burden associated with chronic respiratory conditions [110].
Regular psychological counseling along with support from family, caregivers, and peer groups can significantly improve emotional well-being. Family involvement is essential in providing encouragement, assisting with medical decisions, and fostering a supportive environment [35].
Additionally, patient support groups and advocacy organizations offer a platform for sharing experiences, education, and emotional support. Patients with IPF consistently report that connecting with others who share the same diagnosis is highly beneficial, whether through peer support groups, online forums, or interactions during PR. These forms of peer engagement not only provide emotional reassurance but also foster a sense of shared understanding and validation. Many patients express a strong need for structured, patient-led advocacy groups that offer ongoing education and psychosocial support; however, such services are frequently perceived as insufficient or entirely lacking within current care frameworks. Additionally, patients often voice concern about the unmet support needs of their family members, underscoring the importance of inclusive support systems that extend beyond the individual to encompass the wider caregiving network [18,35].
In addition to formal psychological interventions, ILD nurses play an essential role in providing ongoing education, emotion support, and care coordination for patients and their families. Acting as a consistent point of contact, they help patients navigate complex treatment pathways, manage symptoms, and access appropriate services. Nurse-led interventions have been shown to reduce hospitalizations and emergency department visits, while improving patient satisfaction and continuity of care [111]. Furthermore, ILD nurse involvement enhances patient education and engagement, addressing unmet informational and emotional needs identified in patients [112].
Key messages
• Anxiety and depression are common in patients with ILD and affect their quality of life.
• Support from family, peers, and mental health services is essential.
• Multidisciplinary care improves emotional and clinical outcomes.
Occupational Therapy
An occupational therapist is a healthcare professional responsible for facilitating patients’ ability to perform essential activities of daily living and work-related tasks following illness, injury, or disability. Although no studies to date have specifically examined the role of occupational therapy in fibrotic ILD, evidence from a systematic review of 26 studies involving patients with chronic respiratory diseases—95% of whom had COPD—offers valuable insights. Occupational therapy interventions in this population commonly target three domains: body functions and structures (e.g., addressing fatigue, dyspnea, and reduced mobility), activities and participation (e.g., enabling self-care, domestic, and vocational tasks), and environmental factors (e.g., modifying home or workplace environments to support function). Simple home modifications, such as installing grab bars in bathrooms, using shower chairs, raising chair or bed heights, and rearranging frequently used items to minimize exertion–can significantly improve safety and functional capacity. Occupational therapists also provide guidance on the use of assistive devices and collaborate with multidisciplinary teams to optimize the patient’s home environment [113]. Reported outcomes range from improved physiological responses to enhanced health-related quality of life [114]. Extrapolating from these findings, it is reasonable to infer that occupational therapy may confer similar benefits in patients with fibrotic ILD. Within a multidisciplinary care framework, occupational therapists contribute to individualized assessment and intervention, promoting energy conservation, activity pacing, adaptive strategies, and environmental adjustments. Despite the absence of disease-specific data, the consistent integration of occupational therapy in chronic respiratory care underscores its potential utility in the management of fibrotic ILD. Future research is warranted to evaluate its efficacy in this specific patient population and to guide evidence-based implementation
Key messages
• Occupational therapy improves daily function by addressing symptoms, supporting activities, and adapting environments.
• No fibrotic ILD-specific studies exist, but COPD evidence suggests similar benefits.
• In ILD care, occupational therapy offers tailored strategies, though more research is needed.
Lung Transplantation
Lung transplants remain the last resort for end-stage pulmonary disease, and ILD is not an exception. However, only a few selected patients are suitable candidates for this major surgery. The prognosis of ILD is challenging to predict individually and can vary even with the same disease. Therefore, early referral of potential candidates is recommended to lessen the chance that an eligible patient may miss the opportunity for a lung transplant [115]. Table 2 shows the consensus document of the International Society for Heart and Lung Transplantation (ISHLT) for the timing of referral and listing for lung transplant. While for some ILDs we only need to consider the pulmonary condition, some others such as connective tissue disease associated ILD, may have extrapulmonary involvement of the systemic disease, which may affect the transplant decision, and the ISHLT had a separate statement to address these issues [116,117].
International Society for Heart and Lung 2021 consensus document for the selection of lung transplant candidates
There are more than 4,000 cases of lung transplants per year, and the trend of ILD patients having lung transplants is increasing, although there are regional differences among countries. Eligibility criteria may also vary. Idiopathic interstitial pneumonia (IIP) and non-IIP ILD are the most common indications for lung transplantation, with 32.4% and 8.1%, respectively, in the ISHLT registry in 2017 [118]. The median post-transplant survival was 5.2 years for patients with IIP and 6.7 years for other ILDs transplanted between 1992 and 2017 [118].
For patients on the lung transplant list, antifibrotic therapy is recommended to continue until transplant. Concerns have been raised that antifibrotic therapy, particularly nintedanib, could impair wound healing or increase the risk of post-transplant bleeding [119,120]. Previous clinical experience has indicated bleeding event rates of 15.8 versus 10.2 per 100 patient exposure-years in the INPULSIS trials, and 8.4 per 100 patient exposure-years in INPULSIS-ON [121]. However, evidence from retrospective cohort studies and meta-analyses demonstrates that there is no increased risk of bleeding or anastomotic airway complications associated with antifibrotic therapy [122-124]. Evidence indicates that discontinuation of antifibrotic therapy fewer than five medication half-lives before transplantation may be associated with a higher incidence of anastomotic and sternal dehiscence (0 vs. 11 patients [5.2%], p=0.031) [125]. Some studies found that high-dose pre-transplant corticosteroids (>0.42 mg/kg/m2/day) increased mortality [104], and doses <40 mg or 0.3 mg/kg/day are probably safe [123,126]. There were no other data on other immunosuppressants, but it is recommended to limit immunosuppressants for ILD to the lowest effective doses while a patient is listed for transplant [127].
Key messages
• Early referral for lung transplant should be considered in patients with disease progression.
• Antifibrotics are safe to continue pre-transplant, but high-dose corticosteroids may increase risk.
Vaccination
Patients with ILD are at an increased risk of severe clinical illness and complications following respiratory tract infections. Viral infections, such as influenza, pneumococcal pneumonia, coronavirus disease 2019 (COVID-19), and respiratory syncytial virus (RSV), can lead to acute exacerbation of ILD, respiratory failure, and increased mortality. While there is limited data on vaccination in patients with ILD, vaccination remains a critical preventive strategy to reduce the risk of severe infections and their associated complications. Given their vulnerability, vaccination in patients with ILD should follow current national immunization guidelines.
1. Influenza vaccine
A large epidemiologic study from Germany demonstrated variable influenza vaccination rates among patients with ILD and suggested a potential reduction in all-cause mortality in some seasons [128]. Annual influenza vaccination is strongly recommended because influenza virus strains change each year, requiring updated vaccines to provide effective protection.
2. Pneumococcal vaccine
Current evidence supports the use of pneumococcal vaccination to prevent invasive pneumococcal disease and pneumonia. Pneumococcal vaccines, including pneumococcal conjugated vaccine and pneumococcal polysaccharides vaccine, are recommended for adult aged 65 years and older, as well as for patients aged 19 to 64 years with chronic lung diseases, including ILD, in accordance with national immunization guidelines [129,130]. These vaccines help reduce the incidence of pneumonia and hospitalizations in high-risk populations.
3. COVID-19 vaccine
Vaccine against severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has been demonstrated to reduce the risk of severe disease, hospitalization, and death in patients with chronic lung diseases. COVID-19 vaccines should be administered according to local guidelines, with booster doses as recommended, to ensure optimal protection [131].
4. RSV vaccine
RSV vaccine is recommended for older adults and those with chronic respiratory disease to reduce the risk of severe RSV infection [132]. Overall, vaccination remains a cornerstone of preventive care in patients with ILD. Other vaccines, such as pertussis (Tdap) and herpes zoster (shingles), may also be considered based on the patient’s age and comorbidities.
Infection Prevention Strategies
In addition to vaccination, patients should be educated on essential infection prevention strategies. Emphasizing the importance of hand hygiene is crucial, as regular handwashing with soap and water or using hand sanitizers containing at least 60% alcohol can significantly reduce the transmission of respiratory infections. Furthermore, mask wearing serves as an effective barrier against respiratory pathogen. Wearing well-fitting masks can reduce the risk of infection, especially in crowded or high-risk environments. Patient should also be informed to recognize early symptoms of respiratory infections and enabling prompt medical consultation. Implementing these preventive measures is necessary in ILD management.
Key messages
• Vaccination is a key preventive strategy, and patients with ILD should receive vaccination to prevent severe complications.
Smoking Cessation
Smoking is a major risk factor for both the development and progression of ILD. Patients should be strongly encouraged to stop smoking, with support provided through counseling, nicotine replacement therapy, or pharmacological aids as appropriate.
Reduction of Environmental and Occupational Exposures
Environmental pollutants, occupational dusts, and chemical fumes can exacerbate lung injury. Patients should be advised to reduce exposure by using protective equipment, improving ventilation, and adhering to workplace safety protocols. Identifying and eliminating potential triggers is also essential for preventing disease progression.
Conclusion
The non-pharmacological management of fibrosing ILD represents a fundamental component of comprehensive care, targeting the physical, psychological, and social dimensions of illness that pharmacological therapies alone cannot adequately address. As fibrosing ILDs often follow a progressive and unpredictable trajectory, early and proactive integration of supportive interventions is crucial in optimizing quality of life and functional independence. Despite the evident value of these interventions, a consistent theme across all domains is the scarcity of evidence specific to ILD. Much of the current practice is informed by expert consensus, observational data, or studies in adjacent disease populations such as COPD or IPF. There is an urgent need for robust, prospective research to evaluate the efficacy, optimal timing, and long-term outcomes of non-pharmacological interventions across the ILD spectrum.
In conclusion, non-pharmacological management should be regarded not as ancillary but as central to the care of individuals living with fibrosing ILD. Integrating multidisciplinary, personalised, and evidence-informed approaches is essential to addressing the holistic needs of this complex and vulnerable patient population.
Notes
Authors’ Contributions
Conceptualization: all authors. Writing - original draft preparation: all authors. Writing - review and editing: all authors. Approval of final manuscript: all authors.
Conflicts of Interest
No potential conflict of interest relevant to this article was reported.
Funding
No funding to declare.