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Lung diseases

Idiopathic pulmonary fibrosis (IPF)

A disease in which scar tissue forms in the lungs for an unknown reason. Breathing becomes difficult. It usually begins in older age and progresses slowly.

Not part of the panel of Türkiye's National Newborn Screening Programme (heel-prick test). Source

Orphanet has a guideline for emergency doctors for this disease (language: French). See on Orphanet

Prepare an emergency card

This page is translated from the Turkish original.

This disease is followed through the “Lung diseases” group.

Understanding the disease

This section has not yet been reviewed by a physician. It summarises patient information from sources such as MedlinePlus, Orphanet and the NHS in plain language. The disease can differ from person to person; talk to your doctor about diagnosis and treatment.

What happens in this disease?

Over time, scar tissue builds up in the lung tissue. This is called fibrosis. The lungs become stiff and cannot pass enough oxygen into the blood. The cause is unknown; this is why it is called idiopathic. How fast it progresses varies greatly from person to person.

Which symptoms can occur?

Symptoms begin slowly and increase over time.

  • Shortness of breath, at first on exertion (common)
  • A dry cough that does not go away (common)
  • Tiredness
  • Loss of appetite and weight
  • Rounding of the fingertips, called clubbing

How is it diagnosed?

When listening, the doctor may hear a crackling sound in the lungs. Breathing tests, a chest X-ray and a high-resolution CT scan are done. If needed, a bronchoscopy, which means looking into the lungs with a camera, or a biopsy is done. Other diseases that cause similar symptoms are also looked for.

Treatment and care

The disease does not go away completely. But there are approved medicines that slow the build-up of scar tissue. Oxygen support can ease shortness of breath. Pulmonary rehabilitation, which is a breathing and exercise program, is helpful. Stopping smoking and getting vaccinated are important. In some people, a lung transplant may be considered. The doctor who follows the disease decides which treatment is suitable.

For the family: inheritance and genetic counselling

Most people have no other affected family members. In a small number of families, several people become ill. This is called familial pulmonary fibrosis. In these families, inheritance appears to be dominant. But not everyone who carries the gene becomes ill. If there are other affected family members, genetic counselling is recommended.

Source: MedlinePlus Genetics: Idiopathic pulmonary fibrosis · NHS: Idiopathic pulmonary fibrosis · MedlinePlus: Idiopathic pulmonary fibrosis

Key facts

How common is it?
Prevalence: 1-5 per 10 000 (Worldwide) · New cases per year: 1-9 per 100 000 (Worldwide)
How is it inherited?
Multigenic / multifactorial
When does it usually start?
Adulthood
Gene(s) involved
SFTPA1, SFTPC, DSP, TERT, TERC, MUC5B

These facts are taken from Orphanet and MedlinePlus records (last checked: October 7, 2026). Frequency values are approximate ranges.

You are not alone: patient associations in Türkiye

Non-profit patient and family organizations in Türkiye working on this disease or disease group. Links are for information; Doktorclub is not affiliated with these organizations and does not endorse them.

For all rare diseases: Nadir Hastalıklar Federasyonu

Links checked on October 8, 2026.

Support and help

Doktorclub does not diagnose, recommend treatments or refer you to hospitals or doctors. These pages are meant to help you talk with your doctor.

News about IPF

Clinical trials (ClinicalTrials.gov)

A clinical trial is a regulated study that tests whether a new treatment is safe and whether it works, in patients who volunteer. Most trials look for a certain age, genetic change and health condition. Taking part is voluntary.

This list is not a treatment recommendation. Treatments tested in phase 1–3 trials are investigational (experimental): their efficacy and safety have not yet been proven and they are not approved treatments. Taking part depends on each trial’s criteria and a physician’s assessment; the first step is to talk to the doctor who manages your care.

Questions you can ask your doctor

  • Could this trial be suitable for us?
  • If we take part, how would current treatment and check-ups be affected?
  • Where does the trial take place, and how are travel and accommodation handled?
  • What happens if we want to leave the trial?

ClinicalTrials.gov lists 80 active interventional studies for this disease. 45 are recruiting now; 5 have sites in Türkiye. Last checked: October 7, 2026.

Selected records (studies with a site in Türkiye first):

See all studies on ClinicalTrials.gov · Joining clinical trials

Lung diseases: other news

Medical codes (for talking with your doctor and finding sources)

These codes identify the disease in international classifications and databases. They can help when you talk with your doctor or look for reliable sources.

Orphanet code: ORPHA:2032 · ICD-11 CB03.4

Codes checked against WHO ICD-11 and Orphanet on October 7, 2026.

This page provides general information and does not replace diagnosis or treatment. Talk to your doctor about symptoms, diagnosis and treatment.

The information on these pages is for general information only and does not replace medical advice. Talk to your doctor about diagnosis and treatment decisions. In an emergency in Türkiye, call 112.

Every update is based on an open source and labeled with its level of evidence. No advertising and no drug brand names. Our scientific principles

This page was translated from Turkish by the Doktorclub editorial team.