Pulmonary Fibrosis

There is no cure for PF, but there are various treatment options and management strategies to help stop or slow the progression of the condition and manage symptoms. If you also have other conditions, it is important to work with your healthcare team to build a plan for treating those conditions alongside your PF.

Your Multidisciplinary Team

People with PF may have a Multidisciplinary Team (MDT) caring for them as they navigate life with PF. Some of the team you will get to know well, like your specialist doctor, GP, lung function testing team, respiratory nurses and physiotherapists.  

Your pulmonary rehabilitation team will usually be supervised by a physiotherapist and have other allied health specialists like dietitians, occupational therapists, rheumatologists and others involved to help you live the best life possible.  

There will also be radiologists and pathologists working behind the scenes that will be actively involved in your initial diagnosis and monitoring your progress. 

  • Current treatment options

    PF can result from several different causes. Understanding the cause of your PF will help your specialist doctor to decide which treatment is best. The following information is a general overview of some of the treatments your specialist doctor can offer for PF. Please note, this information is not medical advice. Some treatments may be right for some people, but no particular treatment is right for everyone. To ensure you are receiving the treatment that best suits your condition, speak with your specialist doctor and always consult with them before starting, changing or stopping any medical treatment. 


    Idiopathic Pulmonary Fibrosis (IPF) is a specific type of PF for which two anti-fibrotic medications are currently available in Australia. In patients with mild to moderate IPF (as judged by lung function tests), pirfenidone and nintedanib have been shown to slow down the progression of fibrosis within the lungs. Both are subsidised by the Australian government for patients with IPF who meet certain eligibility criteria.   


    Some forms of PF are caused by inflammation in the lung tissue, often related to autoimmune diseases such as rheumatoid arthritis, scleroderma, Sjögren’s syndrome, dermato/polymyositis and antisynthetase syndrome. These innflammatory conditions are often treated with immunosuppressive medications such as prednisolone, mycophenolate mofetil and azathioprine 

    Based on your type of inflammatory related PF, your specialist doctor will tailor their selection of the different medications to ensure you receive the most benefit.  

    Oxygen therapy

    You may be prescribed oxygen if you develop low levels of oxygen in your blood. You may only need to use oxygen at night when sleeping or when doing exercise. Alternatively, it may need to be prescribed all the time. 

    Pulmonary rehabilitation

    Pulmonary rehabilitation is an exercise and education program provided by specially trained health professionals that teaches you the skills needed to manage your breathlessness and to stay well and out of hospital.  

    Ask your healthcare team about the possibility of a referral to your nearest pulmonary rehabilitation program. If a pulmonary rehabilitation program is not available in your area, a physiotherapist or exercise physiologist can help with an exercise program that is right for you. Speak with your GP about your eligibility for a management plan to access subsidised visits to a physiotherapist or exercise physiologist. 

    Lung transplantation 

    Lung transplantation may be a treatment option for some patients with PF, but not suitable for others. Successful outcomes following transplantation vary according to age, other disease burden and severity of PF. Your specialist doctor will provide guidance on whether transplantation is an option and whether and when you should be referred to your local lung transplant unit. 

    Clinical Trials in Pulmonary Fibrosis 

    Clinical trials are particularly important when treatment options are limited, as they are with PF. A clinical trial is a research study that tests whether a treatment works or not. If you take part in a clinical trial, you may get access to a new treatment whilst it is still in the testing phase, however, there are no guarantees that it will be successful or make you feel better. Taking part in a clinical trial may result in more frequent monitoring and contact with your healthcare team. 

    Palliative care 

    Many people believe that we only need to access palliative care at the end stage of our lives, however, this is incorrect. Palliative care is about symptom management and helping you to live well. An expert palliative care team may be helpful at all stages of your PF . Research shows that people who are linked with a palliative care team early on in their condition have better symptom management throughout their journey. Often you will be introduced to palliative care in the earlier stages of your diagnosis and it continues alongside other treatments. 


  • Other management options

    There are many opportunities for you to participate in caring for yourself.  Some of the things you can do include:  

    Maintaining a healthy mind is also important during such a challenging time dealing with diagnosis, as well as understanding and making treatment and management decisions. Anxiety and depression is not uncommon in people with PF and it is important that you talk to your doctor about this. 

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