Palliative Care in Interstitial Lung Disease and Pulmonary Fibrosis: A Comprehensive Clinical Review

 Palliative Care in Interstitial Lung Disease and Pulmonary Fibrosis: A Comprehensive Clinical Review

Authors: Dr. Shekhar Ingle and Team, Doctor's Forum for All 🏥

Co author - Dr. Ankit. 

Copyright: © 2026 Dr. Shekhar Ingle and Team, Doctor's Forum for All. All rights reserved.

Disclaimer: This article is for educational and clinical reference purposes only. It does not replace individualized clinical judgment, local protocols, or specialist consultation. Medication doses should be verified with current formularies and adjusted to patient-specific factors.

Abstract

Background: Interstitial lung disease (ILD) and pulmonary fibrosis are progressive, life-limiting conditions. They carry a symptom burden that rivals or exceeds many cancers. Dyspnoea, cough, fatigue, anxiety, and depression dominate the clinical picture. Yet palliative care remains underused in this population, often offered only in the final weeks of life.

Objective: This review provides a clinically verified framework for integrating palliative care into ILD and pulmonary fibrosis management. It covers symptom assessment, pharmacological and non-pharmacological interventions, advance care planning, models of care delivery, and end-of-life management.

Methods: A structured narrative review was conducted using peer-reviewed guidelines, systematic reviews, randomized controlled trials, and consensus statements published between 2024 and 2026. Key sources included the European Respiratory Society (ERS) clinical practice guideline, the SEPAR-SECPAL consensus document, a Swiss position paper, and several 2025 narrative reviews and meta-analyses.

Results: The ERS guideline recommends palliative care when physical, psychological, social, or existential needs are identified through holistic needs assessment. A needs-based approach, not prognosis-based, is now standard. Dyspnoea management includes non-pharmacological interventions, personalised self-management strategies, and selective use of low-dose opioids in advanced disease. Chronic cough may respond to neuromodulators and low-dose opioids. Fatigue and psychological distress require routine screening and targeted interventions. Advance care planning is inconsistently implemented but essential. Multiple models of proactive palliative care exist, including multidisciplinary integrated services, nurse-led care, and telemonitoring.

Conclusion: Palliative care belongs in ILD management from diagnosis. Early integration improves quality of life, reduces anxiety and depression, and minimises emergency hospitalisations. A multidisciplinary, needs-based approach is essential.

Keywords: Interstitial lung disease, pulmonary fibrosis, palliative care, dyspnoea, chronic cough, advance care planning, end-of-life care, integrated care.

1. Introduction

Interstitial lung disease is a beast. It's not one disease. It's a heterogeneous group of irreversible, life-limiting conditions. Idiopathic pulmonary fibrosis. Connective tissue disease-related ILD. Fibrotic hypersensitivity pneumonitis. Sarcoidosis. The list goes on.

The prognosis is grim. IPF carries a median survival of just 3 to 5 years from diagnosis. The global incidence is 5.8 per 100,000 persons. Prevalence is 17.7 per 100,000. Regional variations exist—9.0 per 100,000 in North America, 5.1 in Europe, 4.4 in Asia. These aren't just numbers. They're people. People who struggle for every breath. People whose lives shrink as their lungs stiffen.

The symptom burden is brutal. Dyspnoea. Chronic cough. Fatigue. Anxiety. Depression. These aren't just symptoms. They're thieves. They steal function. They steal joy. They steal identity. And they affect caregivers too—the hidden workforce doing most of the daily care, often with insufficient support.

Here's the paradox. In lung cancer, palliative care is well established. Early integration is validated. Specialist consultations are routine. But in nonmalignant chronic lung diseases like ILD? Palliative care is underused. Offered late. Sometimes not at all. Patients with COPD or ILD typically do not have access to the palliative care available to people with cancer. That's a problem. A big one.

The European Respiratory Society defines palliative care for ILD 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 COPD or ILD, and to support their informal caregivers." That's not end-of-life care. That's good care. Period.

2. Methods

This review was conducted using a structured search of PubMed, Cochrane Library, EMBASE, and clinical guideline databases. Search terms included "interstitial lung disease," "pulmonary fibrosis," "palliative care," "dyspnoea," "chronic cough," "advance care planning," "opioids," "pulmonary rehabilitation," and "end-of-life care."

Inclusion criteria: systematic reviews, randomized controlled trials, clinical practice guidelines, consensus statements, and large observational studies published in English between 2024 and 2026. Key guidelines consulted included the ERS clinical practice guideline on palliative care for COPD or ILD, the SEPAR-SECPAL consensus document (2026), the Swiss Position Paper on Palliative Care for Severe Chronic Lung Diseases (2025), and several 2025 systematic reviews and narrative reviews on symptom management and care models.

3. Results

3.1 The ERS Guideline: A Game Changer

The European Respiratory Society published a clinical practice guideline on palliative care for people with COPD or ILD. It's the first of its kind. A task force of 20 members—including patient representatives and caregivers—formulated eight questions. Four were addressed with full systematic reviews. The evidence-to-decision framework was used to formulate recommendations.

The recommendations are clear. Consider palliative care when physical, psychological, social, or existential needs are identified through holistic needs assessment. Offer palliative care interventions, including support for informal caregivers, in accordance with such needs. Offer advance care planning in accordance with preferences. Integrate palliative care into routine COPD and ILD care.

This isn't prognosis-based. It's needs-based. That distinction matters. It means you don't wait for a patient to be "terminal" before offering palliative care. You offer it when needs arise. Which could be at diagnosis. Or any point along the trajectory.

3.2 Symptom Assessment and Management

3.2.1 Dyspnoea

Dyspnoea is the cardinal symptom. It's what patients fear most. It's what wakes them at night. It's what makes them stop doing the things they love.

The evidence supports a stepwise approach. Non-pharmacological interventions first. Personalised self-management strategies. Pulmonary rehabilitation. Then, in advanced disease, selective use of low-dose opioids. But here's the nuance—overall benefit may be modest. A 2025 meta-analysis found that morphine did not significantly reduce breathlessness. Standardized mean difference: −0.26. Confidence interval crossed zero. No impact on quality of life, oxygen saturation, or respiratory rate. Fatigue was the only side effect reaching statistical significance.

A 2026 critical review by Vozoris argued that opioids should not be used for dyspnoea management in ILD, citing respiratory harms. The debate is live. Clinicians need to weigh benefits against risks on a case-by-case basis. Start low if you're going to use them. Monitor closely. Have a plan for titration and de-escalation.

Non-pharmacological interventions matter. Fans. Neuromuscular electrical stimulation. Chest wall vibration. Acupuncture. Acupressure. These can relieve dyspnoea. A Cochrane review concluded there's no evidence for or against benzodiazepines for breathlessness in advanced ILD. So don't reach for them reflexively.

3.2.2 Chronic Cough

Cough is relentless. It's exhausting. It disrupts sleep. It causes urinary incontinence. It makes social situations unbearable. It affects 50 to 70 percent of ILD patients.

Management focuses on treating reversible causes. Gastro-oesophageal reflux disease. Postnasal drip. Then antitussive therapies. Neuromodulators. Low-dose opioids for refractory symptoms. Behavioural and speech therapy may help. The evidence is limited but growing.

A 2026 review in Thorax summarised the current evidence. Chronic cough may be addressed through behavioural and speech therapy, treatment of contributing comorbidities, and antitussive therapies including neuromodulators and low-dose opioids for refractory symptoms.

3.2.3 Fatigue and Psychological Distress

Fatigue is pervasive. It's not just tiredness. It's a bone-deep exhaustion that makes everything harder. It affects 80 to 90 percent of ILD patients. Anxiety and depression affect 30 to 50 percent. They worsen outcomes. They make everything worse.

Routine screening is essential. Use PHQ-9 or HADS. Evaluate modifiable factors. Anaemia. Hypothyroidism. Depression. Medication side effects. Then target interventions. Exercise helps. So does energy conservation. Cognitive behavioural therapy works. Mindfulness helps. SSRIs are first-line pharmacotherapy when indicated.

3.2.4 Oxygen Therapy

Oxygen remains a standard of care for patients with resting or exertional hypoxaemia. It improves oxygenation. But its effect on dyspnoea and symptoms is variable. That's the honest truth. Individualised assessment and patient-centred decision making are essential.

3.2.5 Pulmonary Rehabilitation

Pulmonary rehabilitation is evidence-based. It improves exercise capacity. It reduces symptom burden. It improves health-related quality of life. It also offers psychological benefits. It should be integrated early and maintained as feasible throughout the disease course.

3.3 Advance Care Planning

Advance care planning is inconsistently implemented in ILD care. That's a problem. It leads to end-of-life care unaligned with the patient's wishes. Increased distress amongst patients and their care partners. A 2025 "call to action" paper argued for early and routine integration of advance care planning into IPF management. It reviewed the multifaceted patient-, provider-, and system-level barriers. Proposed actionable strategies to normalise, document, and operationalise patient-centred advance care planning across the disease trajectory.

A 2024 retrospective audit in a national referral centre found that optimal care requires collaboration to define goals and preferences for future medical treatment and care with the patient and their families. Knowledge of illness, goals of treatment and care, and fears and concerns should be documented.

The EU PAL-COPD project is trying to fix this. It's the first large-scale international trial integrating palliative care into respiratory care for ILD. The intervention focuses on identification, advance care planning, and ongoing review of palliative care needs. It starts when the patient is hospitalised for an exacerbation. Why is this important? Because hospitalisations are a critical moment. Patients are sick. They're scared. They're thinking about their mortality, even if they don't say it. It's the perfect time to start conversations about goals of care.

3.4 Models of Care

A 2025 narrative review identified five models of proactive palliative care for ILD. Multidisciplinary integrated services. Nurse-led care. Hospice and residential aged care. Home-based care. Telemonitoring and telehealth. Each model shares common elements. Normalisation of palliative principles within routine care. Diverse delivery settings. Flexibility.

A 2025 scoping review on integrating rehabilitation and palliation found six overarching themes. Overlapping treatment goals. Timing of services. Inclusion of advance care planning. Attention to existential and spiritual dimensions. Barriers to integration. The role of informal caregivers. The overlap is obvious. Both aim to improve function and quality of life. Both address symptoms. Both support patients and families. Integrating them makes sense.

The Swiss Position Paper on Palliative Care for Severe Chronic Lung Diseases emphasised that general palliative care can be provided by nonspecialists. A specialised palliative care team is needed when symptoms become challenging to treat and care situations become increasingly complex. That's a practical distinction. Not every patient needs a specialist. But every patient needs a palliative approach.

3.5 End-of-Life Care

Recognising the terminal phase in ILD is hard. The trajectory is unpredictable. Death can come suddenly from an acute exacerbation. Or slowly from progressive respiratory failure.

Features suggesting imminent death include progressive decline despite maximal therapy. Refractory dyspnoea at rest. Worsening cachexia. Delirium. The patient expressing a desire for comfort-focused care.

Symptom control in the last days of life requires careful medication management. Morphine subcutaneously for dyspnoea. Midazolam for agitation and anxiety. Glycopyrronium for respiratory secretions. Haloperidol for nausea. Stop non-essential medications. Avoid intravenous fluids unless for comfort.

A 2025 systematic review of 32 studies found that early integration of palliative care improves survival rates, reduces anxiety and depression, and enhances quality of life. It minimises emergency hospitalisations and allows patients to choose their place of death. It promotes dignified end-of-life care. But barriers exist. Limited cons

ultation time. Provider discomfort with palliative care discussions. Logistical challenges. These hinder early access.

4. Discussion

4.1 Why Palliative Care Gets Ignored in ILD

Let's be honest. Palliative care in ILD is underused. Why?

First, the disease trajectory is unpredictable. Unlike cancer, where there's often a clear terminal phase, ILD can kill you suddenly or drag on for years. Clinicians struggle to know when to refer.

Second, there's a misconception that palliative care equals hospice. It doesn't. Palliative care is about symptom management and quality of life, alongside disease-directed therapy. It can start at diagnosis.

Third, there's a lack of trained palliative care clinicians in respiratory settings. And there's a lack of respiratory clinicians trained in palliative care.

Fourth, patients often don't want to talk about it. A 2025 qualitative study found that people with ILD had high symptom burden but limited knowledge regarding symptom management and palliative care. Misperceptions influenced acceptance. Opioids and palliative care were linked to end-of-life care. Yet, participants indicated a potential for acceptance of supportive care if reliable information was available.

4.2 The Opioid Debate

The evidence on opioids for dyspnoea in ILD is genuinely mixed. A 2025 meta-analysis found no significant benefit. But other studies suggest low-dose opioids can help. A critical review argued against their use entirely.

What's a clinician to do?

Individualise. Consider the patient's goals, the severity of dyspnoea, the risk of respiratory depression, and the availability of non-pharmacological alternatives. Start low if you're going to use them. Monitor closely. Have a plan for titration and de-escalation.

The debate isn't going away. More research is needed. But in the meantime, clinicians need to make decisions. And those decisions should be grounded in the patient's values and preferences, not just the latest meta-analysis.

4.3 The Role of Specialist Palliative Care

The ERS guideline recommends offering palliative care when needs are identified. General palliative care can be provided by nonspecialists. Specialist palliative care is needed when symptoms become challenging to treat and care situations become increasingly complex. That's the model. Primary palliative care by respiratory teams. Specialist palliative care when needed.

But implementation lags. A 2026 consensus document from SEPAR and SECPAL developed 70 evidence-based recommendations. The key message? Use a needs-based approach. Don't wait for a prognosis. Identify patients with palliative care needs early. Integrate palliative care alongside disease-directed therapies. Adoption of these recommendations is expected to improve quality of life and reduce symptom burden.

4.4 Gaps in Evidence

Despite advances, gaps remain. We need better evidence on opioid use for dyspnoea in ILD. We need to understand which models of care work best and for whom. We need to develop and test interventions that integrate palliative care into routine ILD management. We need to train clinicians. We need to overcome the cultural and systemic barriers that keep palliative care separate from respiratory care.

A 2025 scoping review on interventions to address supportive care needs found considerable heterogeneity. Multimodal interventions with a focus on symptom control, quality of life, peer support, and psychosocial input appear crucial. But generalisability is limited by small sample size, inconsistent evaluation measures, and study attrition. Further research is urgently required.

5. Conclusion

Palliative care in ILD is not optional. It's essential. The evidence supports early integration. The guidelines recommend it. The patients need it.

But implementation lags. Clinicians are unsure when to refer. Patients are reluctant to engage. Systems aren't designed for integrated care.

The solution requires effort at every level. Education. Training. Policy changes. Better research. And a cultural shift that recognises palliative care as part of good respiratory care, not something separate.

Start early. Use a needs-based approach. Address symptoms. Support families. Plan for the end. It's not complicated in principle. It's just hard in practice.

References

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Prepared By: Dr. Shekhar Ingle and Team, Doctor's Forum for All 🏥

Copyright: © 2026 Dr. Shekhar Ingle and Team, Doctor's Forum for All. All rights reserved.

Date: 2026