Pulmonary fibrosis
| Classification according to ICD-10 | |
|---|---|
| J62 | Pneumoconiosis from quartz dust |
| J63 | Pneumoconiosis due to other inorganic dusts |
| J68.4 | Chronic diseases of the respiratory organs due to chemical substances, gases, smoke and vapors Pulmonary fibrosis (chronic) |
| J70.1 | Chronic and other lung involvement after exposure to radiation |
| J84.1 | Other interstitial lung diseases with fibrosis |
| ICD-10 online (WHO version 2019) | |
Pulmonary fibrosis (formerly part synonym "lungs with cirrhose " and "pulmonary sclerosis") is a change in the lung tissue , wherein the amplified connective tissue between the air sacs ( alveoli ) and the surrounding blood vessels hardened is formed, whereby the lungs and scarred ( fibrotic ). Due to the reduced elasticity, the lung volume is reduced and breathing is impeded.
There are numerous clinical pictures that can lead to pulmonary fibrosis. They belong to the group of interstitial lung diseases . Pneumoconioses (dust lungs) such as silicosis (quartz dust lung) are represented. If the cause is unknown, the form is called idiopathic interstitial pneumonia , the most common representative of which is idiopathic pulmonary fibrosis (IPF, idiopathic pulmonary fibrosis). Idiopathic pulmonary fibrosis is much more aggressive as it progresses; H. life expectancy is lower than with exogenous allergic pulmonary fibrosis.
The exogenous allergic pulmonary fibrosis is caused by allergens . Inflammation of the alveoli is a progressive process that can lead to death. In pulmonary fibrosis, the lungs stiffen , their compliance decreases, and more force is needed to ventilate the lungs sufficiently to allow gas exchange to take place. This is disturbed in any case by tissue scarring and the oxygen content in the blood decreases ( hypoxemia ).
causes
- Fibrosis with a known cause (drugs, collagenoses). So stand bleomycin , busulfan and amiodarone suspected to cause the side effect of pulmonary fibrosis. Other known causes (around 50% of cases):
- Granulomatous diffuse parenchymal lung disease
- Pneumonia : Idiopathic interstitial pneumonia (infectious or pre-existing conditions of the lungs)
- Inhaled or non-inhaled noxious substances
- Systemic diseases such as B. Sarcoidosis, Rheumatoid Arthritis, Collagenoses
- Circulatory lung damage, etc. a. Chronic congestive lung in left heart failure
- other forms (e.g., histiocytosis)
The trigger can be the inhalation of certain fibers such as asbestos , dust particles such as fine quartz dust, etc. or an exogenous allergic reaction to certain protein components (for example from hay silos or pigeon droppings). Radiation therapy for lung cancer can also lead to pulmonary fibrosis.
Symptoms
Symptoms are often shortness of breath, low physical endurance, constant coughing, an increase in breathing rate and weakness.
Therapy status 2019
Treatment is sometimes carried out with preparations containing cortisol and the administration of oxygen (for example as long-term therapy ) if the respiratory function is inadequate. It is also possible to treat with immunosuppressants - in a lower dose than with chemotherapy . These include B. Cyclophosphamide and azathioprine .
Nintedanib has a positive effect on lung function in patients with systemic sclerosis. For 52 weeks, taking it twice a day significantly delayed the decline in lung function compared to comparison patients.
Treatment with pirfenidone should also be considered. The previously used substance acetylcysteine is used less often. In severe cases, a lung transplant is considered. After a lung transplant for an ILD , the median survival time is about five years.
literature
- Jürgen Behr: pulmonary fibrosis. Current aspects in diagnostics and therapy. Uni-Med Verlag, 2003.
- Joseph P. Lynch III: Idiopathic Pulmonary Fibrosis. CRC Press, 2003.
Web links
- Pulmonary fibrosis e. V. - Help for those affected and their relatives
- Lungeninformationsdienst.de - pulmonary fibrosis
- Website of the patient organization Lungenemphysema-COPD Germany - symptoms, causes, treatment, therapy of COPD, emphysema and pulmonary fibrosis
- Behr, J .: Diagnostics and therapeutic options for idiopathic pulmonary fibrosis . In: Dtsch Arztebl Int . No. 110 (51-52) , 2013, pp. 875–881 ( review in Deutsches Ärzteblatt ).
- COPD - Germany e. V .: Idiopathic pulmonary fibrosis (PDF; 1.4 MB)
Individual evidence
- ↑ Joachim Frey : Diseases of the respiratory organs. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 599-746, here: pp. 690-693 ( pulmonary cirrhosis or fibrosis ) and 699-708 ( pulmonary fibroses (scleroses) ).
- ↑ a b c d e Gerd Herold and colleagues: Internal medicine . Ed .: Dr. med. Gerd Herold. 2016th edition. Doccheck, Cologne, ISBN 978-3-9814660-5-8 , pp. 396 .
- ↑ Oliver Distler, Susanne Stowasser, et al .: Nintedanib for Systemic Sclerosis – Associated Interstitial Lung Disease. The New England Journal of Medicine. doi : 10.1056 / NEJMoa1903076
- ^ Andreas Günther; Daniel von der Beck; Philipp Markart: IPF - a rare disease with a poor prognosis. In: Andreas Günther; Daniel von der Beck; Philipp Markart: Certified advanced training in idiopathic pulmonary fibrosis, CME, diagnostics and therapy. Internet training (PDF file) Springer Verlag
- ↑ EMA accepts marketing authorization application for nintedanib in IPF. Press release Boehringer Ingelheim from June 5, 2014
- ↑ Roche Annual Report 2014
- ↑ Martinez FJ: Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. In: N Engl J Med. 2014 May 29; 370 (22): 2093-2101. doi : 10.1056 / NEJMoa1401739 .
- ↑ Brown AW, Lung Transplantation in IIP, Review, in Respirology (2016) 21, 1173–1184
