What symptoms and signs should prompt a GP to consider IPF?

The classic combination is: progressive exertional dyspnoea (often initially dismissed as deconditioning); a dry, persistent, debilitating cough; bibasal fine end-inspiratory crackles described as a Velcro-pulling sound on auscultation; and digital clubbing in 25–50% of cases. The key insight is that IPF is frequently missed in its early stages because each symptom in isolation is common in older adults. Think IPF in any patient over 60 with chronic dyspnoea plus dry cough not fully explained by COPD or heart failure — especially with a history of smoking, occupational dust exposure, or family history of pulmonary fibrosis.

What initial investigations should a GP request?

Arrange spirometry and DLCO — IPF produces a restrictive pattern (reduced FVC and FEV1 with preserved or increased FEV1/FVC ratio) plus reduced DLCO. A chest X-ray may show basal reticular markings but is insensitive early. High-resolution CT (HRCT) chest is the key investigation — a definite UIP pattern (subpleural basal-predominant honeycombing, reticular change, traction bronchiectasis) allows diagnosis without biopsy in the right clinical context. Blood workup to exclude alternative causes: ANA, ENA, RF, anti-CCP, ANCA, and antigen-specific hypersensitivity pneumonitis serology. Refer to a respiratory ILD multidisciplinary team for formal diagnostic review.

What are the antifibrotic medications available in Australia and what do they do?

Two antifibrotics are PBS-listed (Authority) for IPF in Australia. Pirfenidone (Esbriet) — a TGF-β-related antifibrotic, available since 2017, dosed at 801 mg three times daily with food. The CAPACITY and ASCEND trials demonstrated slowing of FVC decline by approximately 50% and reduced all-cause mortality. Nintedanib (Ofev) — a multi-targeted tyrosine kinase inhibitor, available since 2018, dosed at 150 mg twice daily (reduce to 100 mg if not tolerated). The INPULSIS trials showed equivalent FVC-decline slowing and reduced exacerbations. Both require specialist initiation; FVC 50–80% predicted is the typical PBS eligibility criterion. They do not reverse established fibrosis.

What complications of IPF does the GP need to monitor for?

Several complications require active GP surveillance. Pulmonary hypertension — echocardiogram if symptoms of right heart strain (exertional pre-syncope, ankle oedema, loud P2). Lung cancer — IPF carries a 4-fold increased risk; low-dose CT lung surveillance is reasonable in patients with life expectancy over 5 years. GORD — common; treat with PPI as subclinical micro-aspiration may drive fibrosis progression. Obstructive sleep apnoea — arrange sleep study if features present. Acute exacerbations — sudden severe worsening of dyspnoea over days to weeks carries approximately 50% in-hospital mortality; refer urgently. Anxiety, depression, and end-of-life planning should be addressed proactively.

When should a GP refer a patient with IPF for lung transplant assessment?

Referral to a lung transplant centre should happen early — lung transplant waiting lists in Australia are long and eligibility assessment takes time. Refer when: FVC or DLCO is declining despite antifibrotic therapy; exercise capacity is significantly impaired on 6-minute walk; or resting hypoxaemia is present. Ideal criteria include age under 70, BMI under 30, and absence of major comorbidity, though individual assessment varies. Australian lung transplant centres for IPF include the Alfred (Melbourne), RPA (Sydney), Prince Charles Hospital (Brisbane), and Sir Charles Gairdner (Perth). Bilateral lung transplant is preferred. Five-year post-transplant survival is approximately 50–60%.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis: recognition and AU general-practice care

By Dr HB Lo, FACRRM Reviewed 8 September 2026 7 min read

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrosing interstitial lung disease characterised by the UIP pattern on HRCT — subpleural, basal-predominant honeycombing. It predominantly affects adults over 60 years.

Without antifibrotic therapy, median survival is 3–5 years. PBS-listed pirfenidone and nintedanib slow FVC decline by approximately 50% and are standard of care for FVC 50–80% predicted.

The GP's role is to recognise progressive dyspnoea, dry cough, and bibasal Velcro crackles in an older adult; exclude alternative causes; and refer promptly to a respiratory ILD multidisciplinary team.

Idiopathic pulmonary fibrosis is a diagnosis GPs should hold in active consideration for any older adult presenting with progressive breathlessness and a dry cough — especially if bibasal crackles are present on auscultation and there is a history of smoking. The disease is commonly under-recognised in early stages, misattributed to COPD, cardiac disease, or deconditioning. Recognition matters because antifibrotic therapy, now PBS-listed, slows the rate of lung function decline by approximately 50% when started early — but it cannot reverse established fibrosis.

The ATS/ERS/JRS/ALAT 2022 diagnostic guideline underpins current practice. Australian-specific resources include the Lung Foundation Australia, the Thoracic Society of Australia and New Zealand (TSANZ), and the Australian IPF Registry (AIPFR).

A. Core clinical — the AU general-practice framework

Recognising IPF

The symptom triad in a patient over 60 years:

Progressive exertional dyspnoea — insidious onset, often initially attributed to aging or deconditioning
Dry cough — chronic, persistent, often debilitating
Bibasal Velcro crackles on auscultation — fine, end-inspiratory crackles described as the sound of tearing Velcro

Clubbing is present in 25–50% of cases and, while non-specific, heightens suspicion when combined with the above.

Risk factors and exposure history

Cigarette smoking — 60–70% of IPF patients are current or ex-smokers; smoking is the strongest modifiable risk factor
Occupational exposures — metal and wood dust, livestock, mould, bird-keeping, and metal-working fluid (these may instead indicate chronic hypersensitivity pneumonitis, an important differential)
Drug-induced ILD — amiodarone, methotrexate, nitrofurantoin, bleomycin, and immune checkpoint inhibitors
Familial pulmonary fibrosis — TERT, TERC, SFTPA2, SFTPC, MUC5B genetic variants; consider in anyone under 50 or with family history
Connective tissue disease — ask about joint pains, Raynaud’s phenomenon, sclerodactyly, dysphagia, photosensitivity, and skin thickening; these suggest CTD-ILD rather than IPF

Initial workup in general practice

Spirometry and DLCO: Look for a restrictive pattern — reduced FVC and FEV1 with preserved or increased FEV1/FVC ratio — plus a disproportionately reduced DLCO. A 6-minute walk test demonstrating significant oxygen desaturation worsens prognosis.

Chest X-ray: Insensitive in early IPF. Basal reticular interstitial markings and volume loss are late findings. Normal CXR does not exclude IPF.

HRCT chest (MBS items 56301 and 56307): The gold standard imaging investigation. A definite UIP pattern on HRCT — subpleural and basal-predominant honeycombing plus reticular pattern plus traction bronchiectasis, without features inconsistent with UIP — allows diagnosis without surgical biopsy when clinical context is appropriate.

Connective tissue disease screen: ANA, ENA panel (Scl-70, anti-centromere, RNA Pol III, Jo-1, anti-MDA5), RF, anti-CCP, ANCA, and NT-proBNP (to exclude heart failure).

Hypersensitivity pneumonitis serology: Where relevant exposure is identified (avian, mould, metal-working fluid).

Referral

Refer to a respiratory ILD multidisciplinary team once IPF is suspected. An ILD-MDT diagnosis — combining clinical, radiological, and where necessary histological information — is mandatory per ATS/ERS/JRS/ALAT 2022. In Australia, expert ILD centres include: Royal Melbourne Hospital; The Alfred (Melbourne); Royal Prince Alfred (Sydney); Royal Brisbane and Women’s Hospital; Sir Charles Gairdner (Perth); and Royal Adelaide Hospital.

B. Evidence on antifibrotic therapy

Pirfenidone (Esbriet) — PBS-listed since 2017

Pirfenidone modulates TGF-β-related pathways central to fibrogenesis. The CAPACITY trials (Noble, Lancet 2011) and ASCEND trial (King, NEJM 2014) demonstrated a ~50% reduction in the rate of FVC decline plus reduced all-cause mortality. Dose: titrate up to 801 mg three times daily with food. Common adverse effects: nausea, photosensitivity, fatigue, anorexia, elevated LFTs, and rash.

Nintedanib (Ofev) — PBS-listed since 2018; extended to PPF since 2020

Nintedanib is a multi-targeted tyrosine kinase inhibitor (VEGFR, FGFR, PDGFR). The INPULSIS trials (Richeldi, NEJM 2014) showed a ~50% reduction in FVC decline and reduced acute exacerbations. The INBUILD trial (Flaherty, NEJM 2019) extended its indication to non-IPF progressive pulmonary fibrosis (PPF). Dose: 150 mg twice daily, reduce to 100 mg twice daily if not tolerated. Common adverse effects: diarrhoea, nausea, vomiting, weight loss, elevated LFTs, and bleeding risk — caution with anticoagulants.

What the evidence does not support

The PANTHER-IPF trial (NEJM 2012) showed that triple therapy with prednisone, azathioprine, and N-acetylcysteine significantly increased mortality and hospitalisation in IPF and was stopped early. Corticosteroids and immunosuppressants — which remain appropriate for non-IPF ILD including NSIP, COP, and CTD-ILD — are harmful in IPF.

C. Complications and co-management

GORD: Commonly associated with IPF and potentially implicated in micro-aspiration-driven fibrosis progression. Treat clinically symptomatic GORD with PPI (omeprazole, esomeprazole). Routine asymptomatic PPI prescription is debated but widely practised.

Acute exacerbations of IPF: Sudden worsening of dyspnoea over days to weeks; in-hospital mortality approximately 50%. Exclude PE (CTPA), pneumonia, and heart failure. Refer urgently; high-dose steroids are used clinically in some centres despite limited RCT evidence.

Pulmonary hypertension: Arrange transthoracic echocardiogram if symptoms of right heart strain are present.

Lung cancer: IPF carries a 4-fold increased lung cancer risk. Low-dose CT surveillance is reasonable where life expectancy exceeds 5 years; discuss with the ILD team.

Oxygen therapy: Long-term oxygen therapy is provided for resting hypoxia (SpO₂ under 88% on air) via state-based domiciliary oxygen schemes. Ambulatory oxygen supports exercise tolerance with exertional desaturation.

Pulmonary rehabilitation: Structured 8–12 week programmes improve 6-minute walk distance, dyspnoea, and quality of life. Multiple RCTs support pulmonary rehabilitation as standard care.

Vaccinations: Annual influenza; pneumococcal (PPV23 and PCV13); COVID-19 per ATAGI; RSV when adult vaccine available. Respiratory infections precipitate acute exacerbations.

D. Australian operations

MBS items relevant to GP: Standard consultations 23, 36, and 44. HRCT chest 56301 and 56307. Spirometry and DLCO items 11500, 11506, and 11512 (specialist). 6-minute walk test item 12203. GPCCMP (post-July 2025) items 965 and 967. Mental Health Care Plan items 2715 and 2717 — anxiety and depression are common in IPF. Telehealth items 91790 and 91891 (12-month existing-relationship rule).

PBS Section 100 (Highly Specialised Drugs):

Pirfenidone (Esbriet) — Authority Required for IPF with FVC 50–80% predicted; specialist initiation
Nintedanib (Ofev) — Authority Required for IPF, SSc-ILD, and non-IPF PPF; specialist initiation
Long-term oxygen therapy is funded via state-based domiciliary oxygen schemes (not directly PBS) for PaO₂ under 55 mmHg or 56–59 mmHg with cor pulmonale

AIPFR: The Australian Idiopathic Pulmonary Fibrosis Registry coordinates national epidemiology, natural history, and treatment outcome data through Lung Foundation Australia and TSANZ. Enrol patients through the treating ILD specialist.

Occupational compensation: Asbestosis, silicosis, and coal worker’s pneumoconiosis — all of which can phenocopy IPF — are notifiable to state work-health-and-safety bodies and attract specific compensation pathways. Silicosis from engineered stone is under active regulatory action in New South Wales and Queensland.

Lung transplant referral: Early referral to the Alfred (Melbourne), RPA (Sydney), Prince Charles Hospital (Brisbane), or Sir Charles Gairdner (Perth) is recommended for eligible patients — do not wait for end-stage disease.

E. Special populations

Familial pulmonary fibrosis (telomeropathy): Patients under 50 years, or with a family history of pulmonary fibrosis, should have a telomere gene panel (TERT, TERC, SFTPA2, SFTPC) and MUC5B testing. Telomeropathy may be associated with bone marrow failure (cytopenias), liver disease, and earlier-onset skin changes. Specialist genetics input is warranted.

Aboriginal and Torres Strait Islander patients: Limited IPF-specific epidemiological data exist in Indigenous Australian populations. ATSI health assessment (item 715) provides a structured entry. Telehealth and outreach services reduce access barriers in rural and remote settings. Cultural sensitivity is particularly important in lung transplant evaluation and advance care planning discussions.

Occupational exposure groups: Workers with silica, asbestos, or metal dust exposures who develop ILD warrant careful differentiation from IPF — imaging and BAL profiles often differ. Notify and refer to occupational medicine where applicable.

When to escalate

Refer to ILD specialist: any new presentation with suspected IPF (progressive dyspnoea plus dry cough plus Velcro crackles in an older adult); to ILD-MDT for formal diagnostic review; for antifibrotic initiation; and for acute exacerbation (urgent).

Refer to lung transplant centre: early in the disease course for eligible patients — declining FVC or DLCO, impaired exercise tolerance, or resting hypoxaemia.

Refer to palliative care: alongside disease-directed therapy, not reserved for end-of-life — dyspnoea, cough, anxiety, and advance care planning are all addressable through early palliative care integration.

Refer to mental health: anxiety and depression are common; a Mental Health Care Plan (item 2715) is routinely justified.

What this article is and is not

This is general health information based on current international guidelines — ATS/ERS/JRS/ALAT 2022 diagnosis and 2018 treatment guidelines — and Australian resources including Lung Foundation Australia, TSANZ, eTG, and the AIPFR, alongside pivotal trials (CAPACITY, ASCEND, INPULSIS, INBUILD, PANTHER-IPF). It is not personal medical advice and does not create a doctor–patient relationship. Antifibrotic initiation and specialist ILD diagnosis require specialist input.

Consumer resources: Lung Foundation Australia — Pulmonary Fibrosis; HealthDirect — Pulmonary fibrosis; Better Health Channel.

Sources cited

Frequently asked questions

What symptoms and signs should prompt a GP to consider IPF?

The classic combination is: progressive exertional dyspnoea (often initially dismissed as deconditioning); a dry, persistent, debilitating cough; bibasal fine end-inspiratory crackles described as a Velcro-pulling sound on auscultation; and digital clubbing in 25–50% of cases. The key insight is that IPF is frequently missed in its early stages because each symptom in isolation is common in older adults. Think IPF in any patient over 60 with chronic dyspnoea plus dry cough not fully explained by COPD or heart failure — especially with a history of smoking, occupational dust exposure, or family history of pulmonary fibrosis.
What initial investigations should a GP request?

Arrange spirometry and DLCO — IPF produces a restrictive pattern (reduced FVC and FEV1 with preserved or increased FEV1/FVC ratio) plus reduced DLCO. A chest X-ray may show basal reticular markings but is insensitive early. High-resolution CT (HRCT) chest is the key investigation — a definite UIP pattern (subpleural basal-predominant honeycombing, reticular change, traction bronchiectasis) allows diagnosis without biopsy in the right clinical context. Blood workup to exclude alternative causes: ANA, ENA, RF, anti-CCP, ANCA, and antigen-specific hypersensitivity pneumonitis serology. Refer to a respiratory ILD multidisciplinary team for formal diagnostic review.
What are the antifibrotic medications available in Australia and what do they do?

Two antifibrotics are PBS-listed (Authority) for IPF in Australia. Pirfenidone (Esbriet) — a TGF-β-related antifibrotic, available since 2017, dosed at 801 mg three times daily with food. The CAPACITY and ASCEND trials demonstrated slowing of FVC decline by approximately 50% and reduced all-cause mortality. Nintedanib (Ofev) — a multi-targeted tyrosine kinase inhibitor, available since 2018, dosed at 150 mg twice daily (reduce to 100 mg if not tolerated). The INPULSIS trials showed equivalent FVC-decline slowing and reduced exacerbations. Both require specialist initiation; FVC 50–80% predicted is the typical PBS eligibility criterion. They do not reverse established fibrosis.
What complications of IPF does the GP need to monitor for?

Several complications require active GP surveillance. Pulmonary hypertension — echocardiogram if symptoms of right heart strain (exertional pre-syncope, ankle oedema, loud P2). Lung cancer — IPF carries a 4-fold increased risk; low-dose CT lung surveillance is reasonable in patients with life expectancy over 5 years. GORD — common; treat with PPI as subclinical micro-aspiration may drive fibrosis progression. Obstructive sleep apnoea — arrange sleep study if features present. Acute exacerbations — sudden severe worsening of dyspnoea over days to weeks carries approximately 50% in-hospital mortality; refer urgently. Anxiety, depression, and end-of-life planning should be addressed proactively.
When should a GP refer a patient with IPF for lung transplant assessment?

Referral to a lung transplant centre should happen early — lung transplant waiting lists in Australia are long and eligibility assessment takes time. Refer when: FVC or DLCO is declining despite antifibrotic therapy; exercise capacity is significantly impaired on 6-minute walk; or resting hypoxaemia is present. Ideal criteria include age under 70, BMI under 30, and absence of major comorbidity, though individual assessment varies. Australian lung transplant centres for IPF include the Alfred (Melbourne), RPA (Sydney), Prince Charles Hospital (Brisbane), and Sir Charles Gairdner (Perth). Bilateral lung transplant is preferred. Five-year post-transplant survival is approximately 50–60%.

Source quality

Sources grouped by evidence tier. AU primary tier first; international where AU is silent or lagging; named-author reconstruction where guidelines have not yet caught up. How tiers work.