Idiopathic pulmonary fibrosis
| Idiopathic pulmonary fibrosis | |
|---|---|
| Specialty | Pulmonology  |
Idiopathic Pulmonary Fibrosis (IPF), also known as Cryptogenic fibrosing alveolitis, is a chronic progressive interstitial lung disease of 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 etiology associated with a histological pattern of UIP.[2]
Clinical Features
Idiopathic Pulmonary Fibrosis 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. [3]
Symptoms are gradual in onset. The most common are dyspnea, but also include nonproductive cough, clubbing, and crackles. [3]
Histology
The histological pattern of fibrosis associated with IPF is referred to as usual interstitial pneumonia (UIP). Although UIP is required for the diagnosis of IPF, it can be seen in other disease's as well.[4]
Classification
Idiopathic pulmonary fibrosis is a type of idiopathic interstitial pneumonia (IIP), which in turn is a type (or group) of interstitial lung diseases. [3]
Idiopathic interstitial pneumonia's include:
- idiopathic pulmonary fibrosis (IPF) (the most common)
- nonspecific interstitial pneumonia
- cryptogenic organizing pneumonia
- acute interstitial pneumonia
- respiratory bronchiolitis-associated interstitial lung disease
- desquamative interstitial pneumonia
- lymphoid interstitial pneumonia
References
- ^ The Washington Manial of Medical Therapeutics, 32nd Ed., p. 276.
- ^ Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment, Am. J. Respir. Crit. Care Med., Volume 161, Number 2, February 2000, 646-664.
- ^ a b c American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias , Am. J. Respir. Crit. Care Med., Volume 165, Number 2, January 2002, 277-304.
- ^ Robbins and Cotran's Pathological Basis of Disease, 7th edition (2005), p.729.
External links
- American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias , Am. J. Respir. Crit. Care Med., Volume 165, Number 2, January 2002, 277-304.
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