Understanding Pleuroparenchymal Fibroelastosis (PPFE) Lung Disease

1. PPFE: frequency and diagnosis Pleuroparenchymalfibroelastosis (PPFE) is arare form of lung disease associated with scarring (fibrosis) of the lungs PPFE is a rare condition,with only around 100 cases identified up until 2017 It is, however, being increasingly recognised, as physicians become more aware of it as an entity PPFE has only been recognised as a separate condition (from other types of lung fibrosis) within the last few years

2. Characteristics PPFE tends to affect the upper lobes of the lungs The scarring tends to involve the regions just beneath the lining of the lungs (the pleura) and the parenchyma (the lung itself)

3. Symptoms COMMON SYMPTOMS OF PPFE INCLUDE: Dry cough Shortness of breath, particularly when exercising Some people report a dull pain around the lungs Some people can have episodes of pneumothorax (caused by air entering into the chest cavity and collapsing the lung)

4. Risk factors • It is not completely clear why PPFE happens,but it is strongly associated with: • Previous lung, bone marrow or haematopoietic cell transplant • Radiotherapy • Treatment with some (but not all) chemotherapies • It also appears to be associated with • recurrent lung infections • an overactive immune system in some patients

5. Risk factors • PPFE can occur at any age (current range of ages from 13–87 years), the average is in the early 50s • Cigarette smoking does not appearto be a risk factor • Often sufferers are of lean build, andthe disease is associated with furtherweight loss over time

6. Prognosis PPFE is a serious disease. However the disease is highly variable and it is very difficult to predict what will happen in a particular person The prognosis can range from poor to relatively stable over a long period of time

7. Monitoring X-RAY Disease monitoring is carried out by lung function tests at regular intervals, usually every 6–12 months. If the condition worsens, an X-ray or computed tomography (CT) scan of the chest are among the tests that can be done, to check for any changes such as a pneumothorax or a chest infection. Walking tests to check for oxygen saturation, can be performed to assess whether oxygen during exercisemay be helpful. O2 An echocardiogram (which images the heart)is a test that can be carried to check that the condition is not indirectly affecting the heart.

8. Pharmacological treatments Currently there is no direct treatment shown to alter the course of the disease, but there are treatments available with the potential to help • Low dose steroids and/or immunosuppressive agentsmay be prescribed if immune overactivity is thoughtto play a role • Antibiotics may be prescribed as a prophylacticto prevent lung infections, which seem tobe a frequent feature Physicians now think that PPFE is not asrare as originally thought • This means there are likely to be trials in the near future to determine the best treatments to use

9. NON-Pharmacological treatments Additional treatments include pulmonary rehabilitation,an exercise programme to improve breathlessness O2 Oxygen during activities can help with breathlessness if oxygen levels drop below a certain level during a walking test

10. Links for more information • http://www.rbht.nhs.uk/patients/condition/pleuroparenchymal-fibroelastosis/ • https://www.blf.org.uk/ • https://radiopaedia.org/articles/pleuroparenchymal-fibroelastosis • https://www.actionpulmonaryfibrosis.org/(PF charity) • https://www.breathingmatters.co.uk/about/ (Fundraising charity for PF and associated diseases) OTHER RESOURCES: • There are active patient chat rooms here:https://patient.info/forums/discuss/ppfe-558239?order=latest&page=10#topic-replies • https://healthunlocked.com/blf/posts/136953591/ppfe • Face book support page:https://www.facebook.com/PleuroParenchymalFibroelastosisSupportGroup/

11. United States Links • http://www.lung.org/ • http://www.pulmonaryfibrosistrust.org/ • https://www.actionpulmonaryfibrosis.org/ • http://www.pulmonaryfibrosis.org/

12. Clinical publications • There is an increasing body of scientific literature on PPFE. Many have described series of cases from particular hospitals. • Various scientific reviews of the disease with a lot of technical detail for physicians can be found here: • Bonifazi et al. Idiopathic Pleuroparenchymalfibroelastosis. CurrPulmonol Rep. 2017;6(1):9-15.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5346597/ • English J et al. Pleuroparenchymalfibroelastosis: a rare interstitial lung disease. Respirol Case Rep. 2015 Jun; 3(2): 82–84https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4469148/ • von der Thüsen J et al. PleuroparenchymalFibroelastosis: Its Pathological Characteristics. CurrRespir Med Rev. 2013 Aug; 9(4): 238–247.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3949414/ • Watanabe K. PleuroparenchymalFibroelastosis: Its Clinical Characteristics. KentaroCurrRespir Med Rev. 2013 Aug; 9(4): 229–237.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3933942/ • A full list of the scientific literature can be found here:https://www.ncbi.nlm.nih.gov/pmc/?term=Pleuroparenchymal+fibroelastosis%5Btitle%5D