In pulmonary fibrosis (thesaurus synonyms: acute interstitial pneumonia; acute interstitial pneumonia [AIP]; alveolar microlithiasis of the lung; alveolar disorder n.d. ; alveolar and parietoalveolar disease states; alveolar pneumopathy; alveolar proteinosis; alveolar damage; alveolar wall damage; alveolitis with fibrosis; alveolocapillary blockage; atrophic pulmonary fibrosis; chronic fibroid lung induration; chronic fibroid pneumonia; chronic fibrous lung induration; Chronic fibrous lung disease; chronic fibrous pneumonia; chronic pulmonary fibrosis; chronic pulmonary cirrhosis; chronic cirrhotic pneumonia; desquamative interstitial pneumonia; diffuse pulmonary fibrosis; diffuse pulmonary fibrosis; endogenous lipid bronchopneumonia; endogenous lipid pneumonia; Fibrosis of pulmonary capillaries; fibrosing alveolitis; fibrosing cryptogenic alveolitis; fibrosis pulmonum; common interstitial pneumonia; Hamman-Rich syndrome; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; idiopathic pulmonary fibrosis (IPF); induration pneumonia; interstitial idiopathic pulmonary fibrosis; interstitial pneumonia; interstitial lung disease; interstitial lung disease; lung cirrhosis; microlithiasis alveolaris pulmonum; nonspecific interstitial pneumonia; parenchymatous pneumonia; parietoalveolar pneumopathy; parietoalveolar disorder a. n.k. ; peribronchial fibrosis; pneumonosis; postinflammatory pulmonary fibrosis; postinflammatory pulmonary fibrosis; postpneumonic pulmonary fibrosis; pulmonary fibrosis; pulmonary sclerosis; pulmonary cirrhosis; other interstitial lung disease; other interstitial lung disease with fibrosis; ICD-10-GM J84.-: Other interstitial lung diseases) is a group of chronic diseases associated with remodeling of the lung skeleton (interstitial lung diseases). There is increased formation of connective tissue between the alveoli (air sacs) and the blood vessels surrounding them. Consequently, both the volume and distensibility of the lungs decrease. The group of interstitial lung diseases (ILDs) includes:
- ICD-10-GM J84.-: Other interstitial lung disease.
- ICD-10-GM J84.0: Alveolar (“affecting the alveoli”) and parietoalveolar disease states.
- Alveolar proteinosis
- Microlithiasis alveolaris pulmonum
- ICD-10-GM J84.1: Other interstitial lung disease with fibrosis.
- Acute interstitial pneumonia [AIP].
- Diffuse pulmonary fibrosis
- Fibrosing alveolitis (cryptogenic)
- Common interstitial pneumonia
- Hamman-Rich syndrome
- Idiopathic pulmonary fibrosis (idiopathic pulmonary fibrosis, idiopathic (without apparent cause) pulmonary fibrosis (IPF)/idiopathic pulmonary fibrosis): chronic, progressive, fibrosing interstitial pneumonia (pneumonia) of unknown cause); excl. : pulmonary fibrosis (chronic): occurs not primarily in smokers but due to inhalation of chemical substances, gases, smoke, and fumes (ICD-10-GM J68.4: Chronic diseases of the respiratory system due to chemical substances, gases, smoke, and fumes); after exposure to radiation (ICD-10-GM J70.1: Chronic and other pulmonary involvement due to exposure to radiation).
- ICD-10-GM J84.8: Other specified interstitial lung disease.
- ICD-10-GM J84.9: Interstitial lung disease, unspecified
- Interstitial pneumonia o.n.a.
As a result of the above diseases, gas exchange disorders occur. Sex ratio: both sexes are equally affected by idiopathic pulmonary fibrosis (IPF). Frequency peak: idiopathic pulmonary fibrosis (IPF) occurs predominantly in the elderly (average age peak: 65 years). The prevalence (disease incidence) of idiopathic pulmonary fibrosis (IPF) is 2-29 per 100,000 persons. Prevalence is up to 10-fold higher in populations greater than 70 years of age. The incidence (frequency of new cases) of idiopathic pulmonary fibrosis (IPF) is approximately 10 cases per 100,000 population per year (in Germany). Course and prognosis: It takes about 1-2 years until the diagnosis of idiopathic pulmonary fibrosis (IPF) is made.The differentiation of IPF from other chronic progressive pulmonary fibroses is essential in view of the different therapeutic approaches [S2k guideline].The inflammation of the alveoli (pulmonary alveoli) is a progressive process that can lead to death. The course of IPF is much more aggressive than exogenous-allergic pulmonary fibrosis, and accordingly the life expectancy of IPF is much shorter (after diagnosis: 3-4 years). Overall, however, the disease courses are very different and depend on the risk factors. Acute exacerbations are particularly important for the prognosis of idiopathic pulmonary fibrosis. The only curative treatment option for idiopathic pulmonary fibrosis is lung transplantation (LUTX).
