Type:
Educational Exhibit
Keywords:
Lung, Respiratory system, Thorax, CT, CT-High Resolution, Diagnostic procedure, Education and training
Authors:
�. Vetešková, K. Zelenak, M. Stevik; Martin/SK
DOI:
10.1594/ecr2018/C-0981
Background
IPF is the most common entity of idiopathic interstitial pneumonias.
It is defined as a chronic progressive fibrotising lung disease of unknown cause and it occurs in older patients.
With the median survival from 2 to 3 years it has a poorer prognosis than non-specific interstitial pneumonia (NSIP) and other interstitial pneumonias.
Although the primary cause of the condition stays unknown,
the current theory suggests that IPF is caused by tiny foci of acute lung injury which occur and reoccur in the interstitium over many years.
Inflammation is present only secondarily.
The recurrent injury leads to abnormal healing of the tissue – permanent fibrosis.
The diagnosis is built up on clinical,
radiological and pathological findings.Typical clinical symptoms are progresive dyspnoe,
cough,
bibasilar inspiratory crackles,
and finger clubbing.
Radiological features typical for IPF are discussed below.
Unfortunately,
no treatment has been proven to be effective so far,
except for lung transplantation,
which currently offers the only cure.