PPFE: frequency and diagnosis Pleuroparenchymal fibroelastosis (PPFE) is a rare 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

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)

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)

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

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 appear to be a risk factor Often sufferers are of lean build, and the disease is associated with further weight loss over time

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

Monitoring Disease monitoring is carried out by lung function tests at regular intervals, usually every 6–12 months. X-RAY 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. O2 Walking tests to check for oxygen saturation, can be performed to assess whether oxygen during exercise may be helpful. An echocardiogram (which images the heart) is a test that can be carried to check that the condition is not indirectly affecting the heart.

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 agents may be prescribed if immune overactivity is thought to play a role Antibiotics may be prescribed as a prophylactic to prevent lung infections, which seem to be a frequent feature Physicians now think that PPFE is not as rare as originally thought This means there are likely to be trials in the near future to determine the best treatments to use

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

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/

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

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 Pleuroparenchymal fibroelastosis. Curr Pulmonol Rep. 2017;6(1):9-15. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5346597/ English J et al. Pleuroparenchymal fibroelastosis: a rare interstitial lung disease. Respirol Case Rep. 2015 Jun; 3(2): 82–84 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4469148/ von der Thüsen J et al. Pleuroparenchymal Fibroelastosis: Its Pathological Characteristics. Curr Respir Med Rev. 2013 Aug; 9(4): 238–247. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3949414/ Watanabe K. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Kentaro Curr Respir 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