IDIOPATHIC PULMONARY FIBROSIS

' 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 cause associated with a histological pattern of UIP.^[2]
It is believed that pulmonary fibrosis is a autoimmune disease.

Contents

Clinical Features

Histology

Classification

References

External links

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 diseases as well.^[5]

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

1. The Washington Manial of Medical Therapeutics, 32nd Ed., p. 276.
2. Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment, Am. J. Respir. Crit. Care Med., Volume 161, Number 2, February 2000, 646-664.
3. 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.
4. 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.
5. ''Robbins and Cotran's Pathological Basis of Disease, 7th edition'' (2005), p.729.
6. 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.

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.