Idiopathic fibrosing alveolitis, chronic form

Common Name(s)

Idiopathic fibrosing alveolitis, chronic form

Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Although older studies included several forms of interstitial pneumonia under the term 'idiopathic pulmonary fibrosis,' the clinical label of 'pulmonary pulmonary fibrosis' should be reserved for patients with a specific form of fibrosing interstitial pneumonia referred to as usual interstitial pneumonia ({15:Gross and Hunninghake, 2001}). It is estimated that 0.5 to 2.2% of cases of idiopathic pulmonary fibrosis are familial ({26:Marshall et al., 2000}). Pulmonary fibrosis can also be a feature in patients with mutations in the TERT ({187270}) or the TERC ({602322}) gene; see PFBMFT1 ({614742}) and PFBMFT2 ({614743}). Some patients with surfactant protein C deficiency ({610913}) who survive to adulthood manifest features of pulmonary fibrosis.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Idiopathic fibrosing alveolitis, chronic form" for support, advocacy or research.

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Scientific Literature

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Azathioprine and Prednisone in the Treatment of Idiopathic Pulmonary Fibrosis
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 16 Aug 2007

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Pennsylvania Idiopathic Pulmonary Fibrosis Research Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 22 Jun 2012

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Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry
 

Status: Recruiting

Condition Summary: Idiopathic Pulmonary Fibrosis

 

Last Updated: 14 Nov 2014

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