Friday, 23 December 2016

Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall



In 2004, Frankel et al. reported five cases with upper-lobe-dominant pulmonary fibrosis that progressed gradually to respiratory insufficienc. As the histology comprised subpleural elastosis, intraalveolar collagenous fibrosis with septal elastosis and collagenous thickening of the visceral pleura, they named this unique disorder pleuroparenchymal fibroelastosis (PPFE). In 2013, PPFE of unknown etiology (idiopathic PPFE, IPPFE) appeared as one of the forms of idiopathic interstitial pneumonias (IIPs) in the international classification of these diseases. 
Pleuroparenchymal Fibroelastosis

Since then, there have been increasing numbers of papers on PPFE. Although IPPFE is categorized as a rare form of IIP, it might not be as rare as reported previously. IPPFE, as a form of chronic fibrosing interstitial pneumonia, has a similar clinical course to that of idiopathic pulmonary fibrosis (IPF), and could be confused with IPF in clinical practice. Read more>>>>>>>>>>>>>>>

1 comment:

  1. On September 28, 2016, my partner Allen’s pulmonologist told him to go home and get his affairs in order, because he only had 30 days to live. At that time, his biopsy showed severe idiopathic pulmonary fibrosis (IPF). Allen’s IPF caused him to struggle with many common daily activities. For instance, walking just 10 feet would leave him feeling dizzy and ready to fall. He had to use a walker to get from his living room chair to the bathroom. Even on supplemental oxygen, he had no energy.Allen began doing all the research he could on his condition and treatment options. At first, he was discouraged by the lack of information and support groups for IPF — the support groups he found were for people with COPD. Then, Allen found the multivitamincare .org website, and everything started to change for him.After his treatment, he doesn’t need his walker to get around anymore, and he’s been able to walk short distances and exercise his legs. He still needs supplemental oxygen, but the amount he uses hasn’t increased. He’s been able to go on an Alaskan cruise with his friends without feeling like a burden. He’s also been able to travel to beaches along the Oregon coast and watch whales in the bay. He says he likes being able to go places, get out of the car and take in the sights. Before multivitamin herbal formula, he wouldn’t have been able to travel or sustain the energy for sightseeing. Now, Allen says he feels so lucky to be alive after his doctor told him he only had 30 days left. Because of the results from his treatment, Allen has a short, sweet piece of advice for other chronic lung disease patients considering getting help from multivitamin herbal remedy.

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