This content, which is intended for UK healthcare professionals only, has been developed and funded by Boehringer Ingelheim. Dr Steve Holmes, GP, received payment for involvement in the development and accuracy review of this content. Mr Ron Flewett also received payment for involvement in the development of this content as a trustee and spokesperson of the Pulmonary Fibrosis Trust.
The GP’s role in diagnosis
Idiopathic pulmonary fibrosis (IPF) is a challenging disease to diagnose as it requires ruling out several other potential diseases and even high-resolution computed tomography (HRCT) features may not be typical in around half of cases.1,2,3,4
According to National Institute for Health and Care Excellence (NICE) guidelines, a diagnosis of IPF can only be made through the consensus of a multi-disciplinary team (MDT) with expertise in interstitial lung diseases (ILDs). At a minimum, this should include a consultant respiratory physician, a consultant radiologist, an ILD specialist nurse, and an MDT co-ordinator.1
“It took me three years to be diagnosed with IPF. In 2011, I was feeling breathless and I had a dry cough. My GP requested a chest X-ray, but it came back clear. The same happened 7-8 months later. The cough got gradually worse and worse and my wife convinced me to see my GP again, who referred me to a respiratory consultant. In July 2012, a CT scan revealed some fibrosing in my lungs. That’s when my IPF journey started.
I was referred to a specialist centre in November 2012; I had various tests done, including a lung biopsy, and the following year I was eventually diagnosed with IPF. I was lucky because I was referred to an MDT specialist team relatively quickly and I was not treated for lung conditions I didn’t have, but during those three years I was in the darkness.”
Ron Flewett, trustee and spokesperson of the Pulmonary Fibrosis Trust, who is living with IPF
To help inform the specialist at the point of referral, you can share the findings of any preliminary tests you have undertaken, as well as the answers to key questions on history and lifestyle.
The following steps can be taken to assess patients with suspected IPF:1
Take a detailed history, carry out a clinical examination and perform blood tests to help exclude alternative diagnoses, including lung diseases associated with environmental and occupational exposures, with connective tissue diseases and with a careful evaluation of drugs taken
Request lung function testing, both spirometry and gas transfer
Request a chest X-ray (remembering this might be normal) and, if you have access, a computer tomography (CT) scan of the thorax, including high-resolution images
“In your assessment, you might consider lung function testing, although it is useful to remember that, following referral, specialist colleagues will undertake more complex testing gas transfer, which is important as inhaled air is often impaired due to the ongoing fibrosis. Lung function findings do not always show restriction until late in the disease and can be complicated by COPD. If the clinical features are suggestive, do not be distracted by spirometry.
In your referral letter, I recommend writing that you suspect an ILD or IPF and that the patient should be seen soon. Additionally, provide them with a good patient history based on presenting symptoms, any history of smoking, exposure to allergens, pre-existing illnesses such as rheumatoid conditions or any related medication. This information will help the specialist team rule out, and rule in conditions, as well as decide on any tests they will run.
Also include information on how debilitated the patient is by their condition, what they’d like to achieve – for example are they keen to rule something out, or do they have a physical goal they are working towards – and what their commitment is to specialist care. Treatment at a specialist centre can require a lot of travel and regular follow-up. It is useful for the specialist team to know what the patient is hoping to achieve so they can make the most appropriate recommendations when a diagnosis is settled upon.”
Dr Steve Holmes, GP
What happens once the patient is referred?
The MDT may order further tests to help them make a diagnosis including bronchoalveolar lavage, transbronchial biopsy and/or surgical lung biopsy (if required).1
Once all the results are available, an MDT discussion will follow, and a decision will be made on diagnosis and appropriate next steps for the patient.
Importance of early referral and diagnosis
There are treatments that can help manage the symptoms and potentially slow down the progression of the disease.5 Prompt referral to a specialist should result in an early diagnosis and the opportunity to commence treatment and receive support and ongoing management, which may have the greatest impact on reducing disease progression and improving prognosis.6
“I can’t stress enough the importance of three things; first of all, looking for the key symptoms of IPF – breathlessness; persistent, dry cough; distinctive bilateral inspiratory ‘Velcro-like’ crackles; clubbed fingers. Secondly, carefully compiling an accurate medical history of the patient that goes beyond their respiratory symptoms. Thirdly, appropriate tests but referring onwards if there is doubt.
While it can be hard to make sense of an IPF picture, these steps are essential to reduce the risk of misdiagnosis, saving time and expediting access to appropriate specialist care.”
Dr Steve Holmes, GP
National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis in adults: diagnosis and management. CG163. Available at: https://www.nice.org.uk/guidance/cg163/resources/idiopathic-pulmonary-fibrosis-in-adults-diagnosis-and-management-pdf-35109690087877. Last accessed November 2019
Hayen A, Herigstad M, Pattinson KTS. Understanding dyspnea as a complex individual experience. Maturitas 2013;76(1)45–50
National Institute for Health and Care Excellence (NICE). Chronic obstructive pulmonary disease in over 16s: diagnosis and management [NG115]. Last accessed November 2019
Tomassetti S et al. The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review. Eur Respir Rev 2015;24(135):69-77
NHS. Overview – Idiopathic pulmonary fibrosis. Available at: www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis. Last accessed November 2019
du Bois RM. An earlier and more confident diagnosis of idiopathic pulmonary fibrosis. Eur Respir Rev 2012;21(124):141-146