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IPF · KNOWLEDGE & UPDATES

The full spectrum of IPF care, in one place.

For patients, caregivers and doctors: approved medicines, established care, diagnosis and research developments, with sources and limitations.

Sources checked: 3 October 2026 · Selected verified updates, not an exhaustive or automatic news feed.

Understanding IPF in greater depth

From biology to clinical practice. Open each topic to see what it explains and how it informs care.

1. Fibrosis is a disorder of repair

The biological model links repeated alveolar epithelial injury to abnormal repair. Activated fibroblasts and myofibroblasts deposit extracellular matrix. Increasing stiffness can reinforce their activation: a feedback loop, rather than inflammation alone.

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2. ‘Idiopathic’ does not mean without biology

It means no specific cause explaining the disease has been identified. Age and genetic susceptibility contribute to risk without determining one person’s future. A family history of pulmonary fibrosis deserves discussion of genetic counselling.

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3. UIP: a pattern, not an IPF diagnosis by itself

Usual interstitial pneumonia (UIP) is an imaging or histological pattern. It can occur in other diseases. IPF diagnosis requires integration of imaging, history and exclusion of known causes by a multidisciplinary team.

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4. Tests measure different things

FVC measures forcibly exhaled volume; DLCO assesses gas transfer. HRCT shows structural changes. Breathlessness and exercise capacity also depend on oxygenation, cardiac and muscle function and other conditions. Trends must be interpreted together.

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5. Slowing progression is not reversal

Treatment aims to reduce future loss of function. Function may still decline despite a treatment benefit. Oxygen and rehabilitation serve different purposes from medicines that slow fibrosis.

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6. Other ILD treatments do not automatically apply to IPF

In PANTHER-IPF, prednisone, azathioprine and NAC increased deaths and hospitalisations. This concerns a specific IPF regimen; it does not remove the role of immunosuppression in appropriate autoimmune ILDs. Do not change medication without diagnostic review.

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7. Telomeres & cellular senescence

Telomere-maintenance genes such as TERT/TERC are linked to fibrosis susceptibility. Cellular senescence involves altered cell function and signalling, not simply the patient’s age. Research reveals fibroblast heterogeneity; a biological target is not yet a proven treatment strategy.

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8. Sudden deterioration needs investigation

The chronic course varies between people. Sudden change should not automatically be attributed to usual progression: it requires urgent assessment for acute exacerbation and other causes. Pulmonary hypertension and respiratory failure are possible complications that change care needs.

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Care map

Nintedanib

EU-approved for IPF. Review tolerability, diarrhoea, liver monitoring and interactions before changing treatment.

EMA · Ofev

Pirfenidone

EU-approved for IPF. The care plan includes tolerability, liver monitoring and sun protection.

EMA · Esbriet

nerandomilast · Jascayd

Nerandomilast (brand name: Jascayd) has been approved in the EU for adults with IPF or PPF since 15 July 2026. Selection, combinations and dose adjustments require the current product information.

EMA · Jascayd

Diagnosis & second opinion

HRCT, exposure and medication history, exclusion of other causes and multidisciplinary discussion. Biopsy is not automatically needed for everyone.

ATS/ERS/JRS/ALAT · 2022

Monitoring the disease

Discuss FVC/DLCO trends, symptoms and exercise capacity with your team. One measurement alone is insufficient for a treatment decision.

NICE · CG163

Pulmonary rehabilitation

Recommended in ILD: structured exercise, education and self-management, adapted to ability and oxygen needs.

ATS · 2023

Oxygen at home & on the move

Assess low oxygen levels at rest and on exertion, choose suitable equipment and receive safety training. Flow rates require an individual prescription.

ATS · 2020

Lung transplantation

Discuss referral early when appropriate. A transplant centre assesses individual eligibility.

NICE · CG163

Cough, breathlessness & supportive care

Ask for symptom relief and psychological support alongside disease treatment. Palliative care can begin early.

NICE · CG163

Daily living & caregivers

Discuss fatigue, nutrition, smoking cessation, work and travel. Bring practical difficulties to your appointment and request an adaptation plan.

NICE · CG163

Vaccines & infection prevention

Annual vaccine review with a separate schedule for each vaccine: flu, RSV, pneumococcal disease, shingles and routine boosters.

Pneumogenesis · 03/10/2026

Clinical trials

Check the population, eligibility criteria, country and actual recruitment. Trial registration is not approval or proof of benefit.

ClinicalTrials.gov

News & scientific developments

2026 · MECHANISTIC RESEARCH

Cellular senescence: fibroblasts are not one uniform population

New research examines senescent fibroblast heterogeneity and DNA damage responses in IPF. It identifies biological targets, not demonstrated treatment efficacy in patients.

npj Aging · πρωτότυπη έρευνα / original research

2026-07-15 · EU APPROVAL

Nerandomilast is now EU-approved

EMA records marketing authorisation for IPF and PPF. This adds a treatment option; approval does not establish Greek availability or reimbursement.

EMA

Find the latest evidence

These searches open current source records. Each new finding needs assessment before it changes care.

All IPF literature · PubMedMedicines & combinations · PubMedRehabilitation, oxygen & transplantation · PubMedSymptoms & quality of life · PubMed

New or suddenly worsening breathlessness, chest pain, confusion or blue lips need urgent medical assessment; do not wait for a website update. In an emergency call 112.

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