Idiopathic Pulmonary Fibrosis

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Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis

Video: Idiopathic Pulmonary Fibrosis

Video: Idiopathic Pulmonary Fibrosis
Video: Idiopathic Pulmonary Fibrosis | Restrictive Lung Disease | Pulmonology 2023, September

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a mostly chronically progressive disease. It occurs primarily in the elderly. Tissues in the lungs are remodeled and there is an increasing loss of lung function. In pulmonary fibrosis, more connective tissue is formed in the lungs, which hardens and scars. Complaints often only become noticeable gradually…


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  • more on the subject
  • Advice, downloads & tools
  • Causes & Symptoms
  • diagnosis
  • therapy
  • Acute deterioration
  • Whom can I ask?
  • How are the costs going to be covered?

Causes & Symptoms

Pulmonary fibrosis affects the so-called pulmonary framework (interstitium), which is located between the bronchi and bronchioles. The lungs "stiffen". Breathing becomes difficult and the amount of oxygen in the blood drops. As the disease progresses, vital processes such as oxygen intake and exchange are increasingly restricted.

The causes are not yet known. The mechanisms of origin are also not yet fully understood. For example, tobacco smoke, gastroesophageal reflux, environmental influences, viral infections and genetic factors are discussed.

Symptoms are usually not noticeable immediately, but creeping up:

  • One sign is a dry cough.
  • Those affected also suffer from difficult breathing or rapid exhaustion during physical exertion.
  • As the disease progresses, there is an increasing decline in performance and weight loss. Pulmonary hypertension and stress or damage to the heart from pulmonary hypertension (right heart stress) are also long-term consequences.
  • The reduced oxygen content in the blood leads, among other things, to the blue coloration of the skin and mucous membranes. Affected people may have drumstick fingers and watch glass nails.


The doctor gets an idea of the patient's symptoms. In addition, certain information is essential, e.g. with regard to lifestyle (smoking), occupation, taking medication, etc. In order to diagnose the disease and rule out other diseases, various tests are carried out, including

  • Physical examination (auscultation, percussion. In those affected, the doctor can detect a crackling rattle while listening to the inhalation, which is also known as sclerophonia),
  • Pulmonary function test (spirometry),
  • Laboratory tests, e.g. ESR, CRP, blood gas analysis,
  • Biopsy,
  • X-ray of the chest area as well
  • high resolution CT (HRCT).

In those affected, a so-called honeycomb pattern can often be seen in the lung x-ray or CT as the tissue changes progress.


With drug therapy, attempts are made to delay the development of fibrosis (pirfenidone, nintedanib). In the event of acute deterioration (acute exacerbations with shortness of breath and coughing), steroids (“cortisone”) or other drugs that suppress or weaken the immune system (eg cyclophosphamide) may be used. So far, it has not been possible to cure the disease with drugs.

A pulmonary rehabilitation can be placed. Further information can be found under Rehabilitation & Cure and COPD: Rehabilitation.

Long-term oxygen therapy may be necessary, for example as the disease progresses. Some sufferers need constant oxygen therapy. As part of this, oxygen-enriched air is inhaled through nasal cannula. Portable devices support mobility in everyday life.

A lung transplant may be done in severe cases. More information on transplantation.

Check-ups will be arranged to monitor the progress. The recognition and treatment of comorbidities (e.g. pulmonary hypertension, gastroesophageal reflux and CHD) also play an important role.

Acute deterioration

Acute rapid deterioration (acute exacerbation) can occur in IPF. For example, there is increased dyspnea and deterioration in the oxygen supply to the body. Those affected should inform a doctor at the first signs of deterioration.

Rapid emergency medical help (emergency number 144) is necessary for:

  • severe shortness of breath
  • (increasingly) accelerated breathing,
  • severely deteriorated general condition,
  • increased heart rate,
  • Impaired consciousness or comatose states,
  • blue-red discoloration of the skin that is new or worsening. (These indicate a decrease in oxygen in the blood) etc.

Whom can I ask?

The contact person is a pulmonologist. Specialists from various disciplines are involved in the diagnosis and therapy process, e.g. for radiology, pathology, internal medicine. Family doctors can initiate referrals. Diagnosis and therapy are carried out in corresponding specialized departments of a clinic / hospital.

In addition, respiratory physiotherapists and other professional groups (health professions) are usually involved in the treatment.

Note In the event of a severe deterioration in the general condition, shortness of breath, impaired consciousness, blue-red discoloration of the skin that appears or increases, etc., the emergency doctor must be called immediately (144)!

How are the costs going to be covered?

The costs for diagnosis and therapy are usually covered by the responsible health insurance company.

Your doctor will generally settle accounts directly with your health insurance provider. However, you may have to pay a deductible with certain health insurance providers. You can also use a doctor of your choice (ie doctor without a health insurance contract) or a private outpatient clinic. For more information, see Visit a Doctor: Costs and Deductibles.

For more information on costs for a hospital stay, see What does a hospital stay cost?

The costs of certain examinations (e.g. biopsy, X-ray, CT) are covered by your health insurance provider if you suspect that you are ill. Referral or referral is required for hospital examinations; this is usually valid for one month from the date of issue. For a CT to be reimbursed by the health insurance company, a senior physician's approval is required within this period. Certain forms of oxygen therapy (oxygen concentrator and liquid oxygen) also require a permit.

For information on the respective provisions, please contact your health insurance provider, which you can find on the social security website.