Speaker's Highlight

• Don Sin University of British Columbia, St. Paul Hospital (Canada)
  Kenneth R. Chapman Toronto General Hospital Research Institute (Canada)
• Parameswaran Nair McMaster University (Canada)
  Carolyn Calfee UCSF (U.S.A.)
• Gregory P. Downey University of Colorado School of Medicine (U.S.A.)
  David A. Schwartz University of Colorado School of Medicine (U.S.A.)
• Neil Schluger Tuberculosis Control Branch, California Department of Public Health (U.S.A.)
  Nick Kim Critical Care & Sleep Medicine, University of California San Diego (U.S.A.)
• Nicola Hananiah Baylor College of Medicine (U.S.A.)
  Jae-Joon Yim Seoul National University College of Medicine (Republic of Korea)
• Koichiro Asano Tokai University School of Medicine (Japan)
  Diahn-Warng Perng Taipei Veterans General Hospital (Taiwan)
• Konstantinos Kostikas University of Ioannina (Greece)
  Karin Klooster University Medical Center Groningen (Kingdom of the Netherlands)

• Managing Cough in Idiopathic Pulmonary Fibrosis: Practical Approaches

  Cough is often the earliest and most stubborn symptom reported by people living with idiopathic pulmonary fibrosis, sometimes preceding the formal diagnosis by months or years. For many Australians receiving their care through tertiary ILD clinics in Sydney, Melbourne, Brisbane, or Perth, the impact of a persistent dry cough reaches well beyond physical discomfort, affecting sleep, social engagement, and overall quality of life.

  Until recently, treatment options were limited and mostly borrowed from general chronic cough guidelines. The landscape has shifted with the wider availability of antifibrotic therapies on the Pharmaceutical Benefits Scheme, alongside a growing body of evidence targeting cough reflexes directly. This article outlines contemporary, evidence-based approaches while reflecting the realities of clinical practice in Australia.

  Why Cough Persists in IPF

  In idiopathic pulmonary fibrosis, cough arises from a combination of mechanical, inflammatory, and neurogenic mechanisms. Fibrotic remodelling distorts airway architecture, while increased sensory nerve density within the lung parenchyma lowers the threshold for cough reflexes. Neurotransmitter imbalances, particularly involving ATP, substance P, and transient receptor potential channels, amplify the signals that reach the brainstem.

  This explains why cough often resists suppression with simple measures and tends to worsen as disease progresses. Patients frequently describe a dry, hacking cough that is exhausting, socially isolating, and resistant to over-the-counter remedies found at the local pharmacy.

  Assessment Before Treatment

  A structured assessment is essential before any escalation of therapy. Beyond a careful history and physical examination, baseline investigations should include high-resolution CT chest, pulmonary function testing, and arterial blood gas where indicated. In Australian practice, ambulatory oxygen assessment and overnight oximetry are often arranged through hospital-based home oxygen programs.

  Co-existing contributors must be ruled out or managed. Gastro-oesophageal reflux, post-nasal drip, ACE inhibitor use, and coexistent airways disease commonly aggravate cough in IPF and should be screened for during the initial work-up. The Lung Foundation Australia's resources and TSANZ position statements provide useful clinical frameworks for primary carers and specialists alike, particularly in bushfire-affected regions where seasonal air quality can worsen symptoms.

  Pharmacological Approaches

  Antifibrotic agents remain the cornerstone of IPF management and may indirectly reduce cough frequency in a subgroup of patients. Both pirfenidone and nintedanib are listed on the Pharmaceutical Benefits Scheme with specific authority requirements, including confirmation of FVC decline patterns and multidisciplinary team review.

  Beyond antifibrotics, neuromodulators such as low-dose slow-release morphine, gabapentin, and pregabalin are frequently used off-label for refractory cough. Low-dose morphine has accumulated the strongest evidence base and is now recommended in several international guidelines, although prescribers in Australia must navigate state-specific regulations for opioid prescribing and ensure shared decision-making. A short trial of corticosteroids may be considered during acute exacerbations but is not appropriate for chronic cough suppression in stable disease.

  Non-Pharmacological Strategies

  Speech pathology interventions have a meaningful role. Techniques such as cough control breathing, hydration strategies, and laryngeal desensitisation exercises can reduce cough frequency and the distress it causes. Many Australian ILD centres now embed a speech pathologist within their multidisciplinary team.

  Pulmonary rehabilitation, delivered through hospital programs or increasingly via telehealth in regional areas, improves exercise tolerance and often reduces cough-related breathlessness. Patients in rural and remote parts of Queensland, Western Australia, and the Northern Territory frequently access rehab remotely through services funded by state health departments. Airway clearance is less relevant in pure IPF than in bronchiectasis, but humidification and adequate hydration are simple measures that individuals can adopt at home.

  Cough in Advanced Disease

  In advanced IPF, cough takes on a heavier palliative dimension. It can signal disease progression, intercurrent infection, or the development of complications such as lung cancer or pneumothorax. Palliative care teams, working alongside respiratory physicians and supported by services such as Silver Chain in Western Australia or Melbourne's Royal Melbourne Hospital palliative program, focus on comfort, dignity, and family support.

  Opioid dosing is individualised, with careful titration and attention to bowel regimens, sedation, and respiratory depression. Discussions about goals of care, advance care directives, and the role of non-invasive ventilation or hospice referral become increasingly important. Symptom-management plans empower family members who, in many Australian households, provide most of the informal care.

  Australian Clinical Pathways

  Access to multidisciplinary ILD review is well established in major academic centres. Regional patients can be referred through telehealth-supported clinics operated by services such as the Prince Charles Hospital in Brisbane or the Alfred Hospital in Melbourne, often with outreach to Tasmania and the Northern Territory.

  PBS authority approvals for antifibrotics require specific spirometry and HRCT criteria, with renewals based on documented disease course. Patients who do not meet PBS criteria may access medication through compassionate access programs run by the manufacturers, an option worth raising during consultations. Clinical trials investigating novel anti-fibrotic and anti-cough agents are available at sites including the Institute for Breathing and Sleep in Melbourne and Royal Prince Alfred Hospital in Sydney, and local investigators frequently contribute to the Australian IPF Registry.

  Emerging Evidence and Where Practice Is Heading

  Research into cough-specific therapies is gaining momentum. P2X3 receptor antagonists such as gefapixant have shown reductions in objective cough frequency in phase 2 trials, although commercial development has been mixed globally. Other candidates targeting TRPV4, neurokinin-1, and sodium channels are earlier in development.

  Real-world data from Australian cohorts continue to refine clinical practice. Local findings suggest that early antifibrotic initiation, rather than waiting for FVC decline below 80 percent predicted, may improve long-term cough outcomes. Continued engagement with registries and trial sites will be vital for the next generation of patients.

  Comparing the practical options discussed above helps frame shared decision-making during consultations.

  Approach	Mechanism	Typical Role in IPF Cough	Key Australian Access Point
  Pirfenidone	Antifibrotic, anti-inflammatory	Disease-modifying; may reduce cough indirectly	PBS authority listing
  Nintedanib	Tyrosine kinase inhibition	Disease-modifying; limited direct cough effect	PBS authority listing
  Low-dose morphine	Central cough suppression	Refractory chronic cough; strong evidence base	Specialist prescribing with state opioid rules
  Gabapentinoids	Neuromodulation	Adjunct; useful where laryngeal hypersensitivity present	PBS general listing; off-label indication
  Speech pathology	Behavioural retraining	Cough control techniques, hydration support	Multidisciplinary ILD clinics; telehealth
  Pulmonary rehabilitation	Exercise and education	Improves tolerance, reduces cough-related distress	Hospital programs and state-funded telehealth

  A practical takeaway for clinicians and patients is to view cough in IPF as a treatable symptom embedded within a broader care plan. Combining disease-modifying therapy, targeted neuromodulation, behavioural strategies, and supportive palliation, all adapted to local access pathways and PBS frameworks, gives Australian patients the best chance of meaningful relief.

  Richard Russell Nuffield Department of Clinical Medicine, University of Oxford (United Kingdom)
• Mona Bafadhel King’s College London (United Kingdom)
  David Jackson Guy’s and St Thomas’ Hospital, King’s College London (United Kingdom)
• James Chalmers University of Dundee (United Kingdom)
  David Price University of Aberdeen (United Kingdom)