Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a chronic, progressive interstitial lung disease with an unknown cause. It is one of the two classic interstitial lung diseases, the other being sarcoidosis.[1]
More specifically, IPF is defined as a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with a histological pattern of usual interstitial pneumonia (UIP).[2]
Pulmonary fibrosis has often been called an autoimmune disease. However, it is perhaps better characterized as an abnormal and excessive deposition of fibrotic tissue in the pulmonary interstitium with minimal associated inflammation.[3] Autoantibodies, a specific sign of autoimmune diseases, are found in a minority of patients with truly idiopathic pulmonary fibrosis. Moreover, many autoimmune diseases associated with “pulmonary fibrosis”, such as scleroderma, are more frequently associated with a related but more inflammatory disease, nonspecific interstitial pneumonitis.[4] It is sometimes associated with smoking[5] and exhibits some dependency on the amount of smoking.[6]
Idiopathic pulmonary fibrosis is a type of idiopathic interstitial pneumonia (IIP), which in turn is a type (or group) of interstitial lung diseases.[7]
Idiopathic interstitial pneumonias include:
IPF is slightly more common in males and usually presents in patients greater than 50 years of age. Average survival from time of diagnosis varies between 2.5 and 3.5 years, depending on severity, although some patients live greater than 10 years.[7]
Symptoms are gradual in onset. The most common are progressive dyspnea (difficulty breathing), but also include nonproductive cough, clubbing (a disfigurement of the fingers), and crackles (crackling sound in lungs during inhalation).[7] It should be noted that these features are non-specific and can occur in a spectrum of other pulmonary disorders.
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