PNEUMOGENESIS / IPF PRIMER
IPF,
from the beginning.
An introductory guide for patients, families and anyone beginning to study idiopathic pulmonary fibrosis. Six steps, with plain explanations and routes to deeper learning.
Start with the basics ↓Read in order or choose the chapter you need. This is educational content and does not replace individual assessment.
01 / 06
What does IPF mean?
Idiopathic pulmonary fibrosis is a chronic disease involving scarring of lung tissue. It is one interstitial lung disease, not a synonym for all pulmonary fibrosis.
- “Idiopathic”: no specific cause has been identified after appropriate investigation.
- Progression varies between people; the diagnosis alone does not predict an individual’s course.
For deeper understanding
IPF is a clinical diagnosis. UIP is a morphological pattern that can also occur in other conditions.
A question for your consultationWhat supports classifying my fibrosis as idiopathic?
02 / 06
From alveolus to fibrosis
Oxygen enters the blood through the alveoli. In IPF, abnormal repair after injury promotes scar formation.
- Thickening and stiffness make lung expansion and oxygen transfer more difficult.
- Age, smoking and genetic predisposition are associated with risk; they do not explain every case.
For deeper understanding
Biology is more complex than a linear “injury → scar” sequence. Family history may warrant specialist genetic assessment.
A question for your consultationDoes family history matter for my evaluation?
03 / 06
How is the diagnosis established?
Diagnosis combines history, examination, HRCT and exclusion of other causes. No single finding is sufficient in every case.
- Review exposures, medication, autoimmune disease and family history.
- Multidisciplinary discussion integrates the available information.
For deeper understanding
Biopsy is not required for everyone. In an appropriate clinical setting, UIP or probable UIP can support diagnosis without biopsy; decisions are individualised.
A question for your consultationHow confident is the diagnosis, and what remains uncertain?
04 / 06
What does each test measure?
Imaging shows structure; physiological tests assess different aspects of function. Interpret results alongside symptoms and test quality.
- FVC: volume exhaled forcibly. DLCO: gas transfer capacity.
- 6MWT: functional exercise capacity. Oximetry: an estimate of oxygen saturation.
For deeper understanding
“FVC 70% predicted” does not mean “70% of the lung remains”. A single value is not an individual prognosis.
A question for your consultationAre serial tests comparable, and what do they show together?
05 / 06
What does treatment aim to achieve?
Treatment aims to slow functional decline and support life with the disease. It does not remove established scarring.
- Nintedanib and pirfenidone are established antifibrotic options; selection and monitoring are individualised.
- Nerandomilast, a preferential PDE4B inhibitor, reduced FVC decline versus placebo over 52 weeks in FIBRONEER-IPF.
For deeper understanding
FVC is a functional endpoint, not by itself proof of survival benefit. For nerandomilast, check the current SmPC for dosing, co-administration and interactions. EU authorisation does not establish Greek reimbursement.
A question for your consultationWhat is the plan for monitoring effectiveness and adverse effects?
06 / 06
Care beyond medication
Rehabilitation, oxygen assessment, symptom support and psychological care belong in the overall plan.
- ATS recommends pulmonary rehabilitation for adults with interstitial lung disease. Programmes are adapted to your abilities.
- Discuss transplantation when appropriate and arrange regular follow-up.
For deeper understanding
Supportive/palliative care can accompany active treatment. Oxygen prescription requires assessment; follow the flow agreed with your team.
A question for your consultationWhich intervention could help my daily life most right now?
A — Z
The words you encounter.
- IPF / ΙΠΙ
- Idiopathic pulmonary fibrosis.
- ILD
- Interstitial lung diseases: a group that includes IPF.
- UIP
- Usual interstitial pneumonia: a morphological pattern.
- HRCT
- High-resolution computed tomography of the chest.
- FVC
- Forced vital capacity.
- DLCO / TLCO
- Lung diffusing capacity for carbon monoxide.
- 6MWT
- Six-minute walk test.
- MDD
- Multidisciplinary diagnostic discussion.
When something changes suddenly.
New or markedly worsening breathlessness, chest pain, fainting or confusion require immediate medical assessment. In an emergency, call 112. Do not wait to finish this guide.
Your next step.
Educational synthesis prepared and cited sources checked: 19 October 2026. This does not signify an exhaustive systematic review or personal review by the Professor. Sources have different publication dates; guidelines and current product information take precedence over older patient-information pages.